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ABSTRACT Tumor lysis syndrome is a well-characterized and potentially deadly complication of spontaneous or treatment-related tumor destruction, and it is most commonly associated with hematologic malignancies. Our case illustrates a rare example of fatal tumor lysis syndrome in the setting of metastatic gastric adenocarcinoma treated with radiation therapy. This case highlights the critical importance of identifying patients with solid organ malignancies at risk for tumor lysis syndrome and of early recognition and treatment of this syndrome.

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ABSTRACT Cardiac lymphoma is a rare entity. In this setting, the secondary involvement of the heart is far more frequent than the primary cardiac lymphoma. Herein, we present an autopsy case of a disseminated anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma with a dominant mediastinal involvement. Extensive cardiac infiltration with the near replacement of the myocardial wall by the neoplastic cells was observed. A total of nine isolated case reports of anaplastic large cell lymphoma with cardiac involvement were found in the English-language literature, and a widespread cardiac and thymic infiltration by the systemic ALK-positive anaplastic large cell lymphoma has not been documented. An incidental regenerative nodule was also identified in the liver. The patient died of pulmonary thromboembolism and cardiac arrest.

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ABSTRACT Metastatic gallbladder carcinoma to the ovaries is occasional but a recognized entity. It can mimic, clinical and morphologically, a primary ovarian tumor, challenging the diagnosis. We present the case of a patient with a lump in the hypogastrium extending into the right iliac fossa and was found to have abdominopelvic cystic lesion with enhancing solid components and multiple sub-centimetric and ill-defined abdominal lymph nodes. Also, subpleural and parenchymal nodules in the lungs were present. She subsequently underwent a laparotomy. Cholecystectomy was also done due to pre-existing symptomatic biliary lithiasis. The histologic report described the ovarian involvement as metastases from a gallbladder carcinoma. The presentation of ovarian metastases can challenge the diagnosis. Hence, careful evaluation of the digestive tract and judicious use of immunohistochemistry should be considered in patients presenting with ovarian masses.

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ABSTRACT Aortopulmonary window (APW) is a rare congenital heart defect with abnormal communication between the ascending aorta and the pulmonary trunk with two separate semilunar valves. We present an autopsy case report wherein a young primigravida woman presented with progressive breathlessness and central cyanosis at 21 weeks of gestation. Echocardiography performed in the emergency room revealed elevated right-sided cardiac pressures suggestive of severe pulmonary hypertension; however, no structural cardiac defect was discernible. The patient succumbed to congestive cardiac failure and progressive hypoxia within 5 days of hospitalization. The autopsy revealed a Type I aortopulmonary window (2 cm) with patent ductus arteriosus. The lungs showed changes of severe pulmonary hypertension with superadded bronchopneumonia. This report underscores a rare presentation of APW, undiagnosed until pregnancy, leading to the Eisenmenger syndrome and death.

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ABSTRACT First described by Rokitansky in 1842, and further characterized by Virchow in 1854, amyloidosis is a disorder caused by amyloid deposition, a fibrillary insoluble protein. The clinical spectrum of amyloidosis is broad, as the amyloid deposition may virtually occur in all tissues. Herein, we report the case of a 66-year-old man with a long-lasting emaciating disease, diagnosed, at autopsy, with primary systemic amyloidosis. Amyloid protein deposition was found in many tissues and organs. The involvement of the vessels’ wall rendered ischemic injury most prominent in the intestinal loops causing mesenteric ischemia. Despite the thorough organic involvement, the immediate cause of death was aspiration bronchopneumonia. Massive amyloid deposition was found in virtually all major organs, such as the heart, liver, kidneys, spleen, pancreas, adrenals, prostate, skin, and thyroid: the latter, a complication of the amyloidosis known as amyloid goiter. Post-mortem review of the deceased’s laboratory workup showed a slightly abnormal kappa:lambda ratio in the blood; however, no clonal lymphoplasmacytic disorder was confirmed in the bone marrow and other lymphoreticular system organs either by the microscopic examination and immunohistochemical staining. Laser-capture microdissection and tandem mass spectrometry of the splenic tissue detected a peptide profile consistent with an immunoglobulin Kappa light chain. The presence of amyloid purpura favors the diagnosis of primary systemic amyloidosis.

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ABSTRACT Blunt chest trauma (BCT) is one of the rarest causes of acute myocardial infarction (AMI). This paper reports the case of a young married man who suffered from AMI due to BCT sustained in a fight with his wife. The histopathology examination revealed a rupture of atherosclerotic plaque with superimposed thrombus in the proximal left anterior descending artery. This report also reviews previously reported BCT-induced AMI cases in the literature.

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ABSTRACT Epignathus, is a rare oropharyngeal teratoma arising from the head and neck region. Sporadic cases have been described with associated intracerebral teratoma. Even more infrequent and extraordinary is the circumstance of a teratoma with oropharynx destruction. We describe the case of a fetus with pharyngeal mass that completely destroyed the oral cavity. The histological examination revealed an immature teratoma (G3); only one other G3 case has been described.

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ABSTRACT A case of probable coronary arteritis in a young girl who died suddenly and unexpectedly is presented. The histologic presentation of the disorder is discussed, especially the differential diagnosis of arteritis of the coronary arteries with an emphasis on tuberculosis (TB). TB myocarditis with or without concomitant lung involvement is rare, and tubercular coronary arteritis without underlying pulmonary Koch’s disease is all the rarer. We herein describe a case where the cause of death was ascertained on post-mortem examination.

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ABSTRACT Uterine rupture during pregnancy is a known complication of placenta accreta. This paper presents a case of sudden maternal death in the 27th week of gestation due to a ruptured uterine scar at the site of placenta accreta with a short inter-pregnancy period of 6 months with previous two C-sections. Autopsy findings revealed a massive hemoperitoneum and a thinned out anterolateral uterine wall. Internal examination revealed clotted and fluid blood in the peritoneal cavity with rupture of the anterior uterine wall at the site of the placenta accreta in a healed cesarean section scar. Placenta accreta is a rare complication of pregnancy. However, it is becoming more frequent and a significant risk factor with the increasing rate of C-section.

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ABSTRACT Whipple’s Disease, a rare diagnosis caused by the slow-growing bacterium Tropheryma whipplei, most often presents with the classically described signs of malabsorption due to gastrointestinal colonization. However, it can also have signs and symptoms that clinically overlap with rheumatic diseases, potentially resulting in misdiagnosis. Furthermore, treatment with modern potent biologic immunosuppressive agents and classic disease modifying anti-rheumatic drugs (DMARDs) can lead to serious exacerbation of undiagnosed infections. We present the case of a middle-aged woman with long term complaints of arthalgias, who was diagnosed with seronegative rheumatoid arthritis and subsequently treated for almost 7 years with such immunosuppressive therapies. The patient’s disease course included chronic diarrhea that abruptly intensified and culminated in fatal hypovolemic shock/sepsis. A diagnosis of WD was made by autopsy examination, wherein several organ systems were found to be heavily involved by Tropheryma whipplei organisms, and their identification was confirmed with histochemical and molecular evaluation. Notably, most bacterial organisms were located deeply in the submucosa/muscularis of affected organs, a practical reminder to practicing pathologists that challenges the classic histopathologic description of Whipple disease as an infiltration of predominantly lamina propria, and the potential for sampling bias in typically superficial endoscopic biopsies during routine procedures.

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ABSTRACT The gluteal region contains important neurovascular and muscular structures with diverse clinical and surgical implications. This paper aims to describe and discuss the clinical importance of a unique variation involving not only the piriformis, gluteus medius, gluteus minimus, obturator internus, and superior gemellus muscles, but also the superior gluteal neurovascular bundle, and sciatic nerve. A routine dissection of a right hemipelvis and its gluteal region of a male cadaver fixed in 10% formalin was performed. During dissection, it was observed a rare presentation of the absence of the piriformis muscle, associated with a tendon fusion between gluteus and obturator internus, and a fusion between gluteus minimus and superior gemellus muscles, along with an unusual topography with the sciatic nerve, which passed through these group of fused muscles. This rare variation stands out with clinical manifestations that are not fully established. Knowing this anatomy is essential to avoid surgical iatrogeny.

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ABSTRACT Extranodal NK/T-cell lymphoma, nasal type (ENKTL-NT) is a rare type of Non-Hodgkin’s lymphoma, which usually presents with extranodal involvement and affects the nasal/upper aerodigestive tract in the classical presentation. Herein, we report the case of a 31-year-old, previously healthy, male patient diagnosed with ENKTL-NT with the involvement of the lung parenchyma and heart. Unfortunately, due to the rapid disease progression, the diagnosis was performed only at the autopsy. The authors highlight the rare clinical presentation of this type of lymphoma, as well as the challenging anatomopathological diagnosis in necrotic samples.

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ABSTRACT Hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal syndrome resulting from a hyperactivated immune system. Diverse patient profiles and clinical presentations often result in misdiagnosis. This article describes the varied clinical presentations and autopsy findings in three patients with this entity. The etiopathogenesis of HLH, its disparate and confounding clinical features, the diagnostic criteria, and management principles are also briefly reviewed.

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ABSTRACT Peutz-Jeghers syndrome (PJS) is a rare autosomal dominant polyposis entity that often remains undiagnosed. The major problems associated with PJS are acute complications due to (i) polyp-related intestinal obstruction, (ii) intussusception, and (iii) the risk of cancer in the long-term. We report the case of a 32-year-old female who presented at the emergency room with signs of acute abdomen and died during the clinical workup. She had a one-month history of nausea, vomiting, and diarrhea and was pregnant at about 30 weeks. There was no contributing past history except for undergoing small bowel resection in infancy. The postmortem examination revealed multiple arborizing polyps throughout the gastrointestinal tract, chiefly in the small bowel. Intestinal obstruction was found at the proximal jejunum with necrosis, perforation, and peritonitis. Histologically, the polyps were composed of tree branch-like bundles of smooth muscle covered by normal-appearing glandular epithelium, confirming the diagnosis of hamartomatous polyps. No malignant or premalignant lesions were detected in the gastrointestinal tract or other organs. This case was an opportunity to analyze the natural history and the pathological features of the Peutz-Jeghers syndrome in an adult and to investigate the presence of neoplastic lesions associated with this condition.

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ABSTRACT We describe an unusual case of lymphomatosis cerebri in a middle-aged lady presenting with rapid-onset dementia. The lymphomatous infiltrate, instead of forming mass lesions, percolated throughout the brain parenchyma, which is often missed on a stereotactic biopsy and hence warrants caution and awareness about this entity. The nonspecific symptoms at presentation and a variable picture at imaging make this entity diagnostically challenging.

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ABSTRACT Invasive aspergillosis is an uncommon infection, which is mainly seen among immunocompromised patients. In recent years, cases of aspergillosis involving immunocompetent hosts are increasingly being reported. Herein, we report the case of a 27-year-old man with fever, productive cough, shortness of breath, and left hemiparesis. He had suffered trauma to his head 25 days prior. Imaging of the chest showed bilateral cavitary lesions in the lungs, and neuroimaging revealed a space-occupying lesion in the right frontoparietal cerebrum. He was suspected of having an abscess or metastasis. He died on day 3 of hospitalization, and an autopsy was performed. The autopsy revealed the cause of death to be invasive pulmonary aspergillosis, with brain dissemination. Invasive aspergillosis is uncommon in apparently immunocompetent individuals, and we discuss the autopsy findings in detail.

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ABSTRACT We performed autopsies on two cases of COVID-19. The microcirculations of all organs were the site of the pathological findings. Thrombotic microangiopathy was found in the brain and also the kidneys. Vasculitis was also a feature of the autopsy findings, together with portal triaditis of the liver. The major pathological findings in both cases were fibrin deposits. Within the lung, the fibrin deposits were observed in the alveolar microcirculation in sub-endothelial locations of capillaries, arterioles, post capillary venules, and the adventitia of larger vessels. These fibrin deposits in the lungs occurred at the sites where pericytes are located in these vessels. The pericyte with its high concentration of ACE-2 receptors and its procoagulant state may represent one of the primary sites of action of SARS-CoV-2. A review of pericytes in health and disease is undertaken. COVID-19 is a disease of the microcirculation.

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ABSTRACT Background Neonatal acute liver failure (NALF) is a rare and life-threatening condition. It causes bilirubin to accumulate to a dangerous level in the body, causing permanent damage to vital organs such as the brain and lungs. In many cases, the etiology of NALF remains unknown. Case presentation We described a case of an 8-day-old baby girl who presented with poor oral intake, lethargy, and jaundice. Her clinical condition rapidly deteriorated with progression to multi-organ failure, and despite intensive resuscitation efforts, she expired. At autopsy, the most significant findings were liver necrosis, yellow hyaline membrane deposition in the lungs, and bilirubin deposition in the brain (kernicterus). Conclusions NALF is a rare and potentially fatal condition necessitating prompt recognition and disease-specific treatment approaches. Toxic accumulation of bilirubin in the lungs can lead to hypoxia and precipitate further ischemic injury to the liver.

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ABSTRACT Rheumatic heart disease is still common in developing countries and requires prompt intervention to prevent chronic complications. Vegetations in rheumatic heart disease might be due to acute episodes of rheumatic fever itself or due to either infective endocarditis (IE) or Non-infectious thrombotic endocarditis (NITE). Each form of vegetations has specific pathological characteristics on gross and microscopic examination. However, clinically IE and NITE may have overlapping signs and symptoms. A chance of misdiagnosis of NITE as culture-negative infective endocarditis is higher if the former present with infective symptoms like fever. NITE of valves can be due to underlying associated malignant neoplasm, particularly mucinous adenocarcinoma, pneumonia, cirrhosis, autoimmune disorders, and hypercoagulable state. The coexistence of tuberculosis, non-infectious thrombotic endocarditis and rheumatic valvular heart disease was rarely documented in medical literature. We describe a case of chronic rheumatic heart disease with vegetations in the posterior mitral valve leaflet, treated as culture-negative infective endocarditis, which, at autopsy, reveals the presence of Nonbacterial thrombotic endocarditis vegetation over calcified, fibrosed mitral valve leaflets and associated disseminated tuberculosis along with classic pathological sequela findings of chronic rheumatic mitral valvular heart disease in lungs and liver.

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ABSTRACT Extremely low birth weight (ELBW) infants are at particularly high risk for infection due to an immature immune system, invasive procedures such as endotracheal intubation, intravascular catheterization, and other factors. Neonatal infections in this population are associated with a high mortality, poor growth, and neurodevelopmental outcomes. Pseudomonas aeruginosa (P. aeruginosa) infection is an uncommon but potentially devastating cause of pneumonia and sepsis in the ELBW population. P. aeruginosa is an important cause of healthcare-associated infections (HAI) or nosocomial infections. P. aeruginosa can perceive unfavorable environmental changes and orchestrate adaptations by developing plasmid-mediated and adaptive resistance to antibiotics. We describe an ELBW infant born at 26 weeks’ gestation who succumbed at 13 days of life to P. aeruginosa infection. Some of the factors related to the pathogenesis and multidrug resistance are described.

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ABSTRACT Peritoneal lymphomatosis (PL) is a rare presentation of extranodal precursor leukemia/lymphoma. The presentation is often non-specific, leading to delayed diagnosis and treatment. In this case, though the preliminary diagnosis was established on ascitic fluid cytology, the disease progressed rapidly, leading to demise before initiating chemotherapy. Immunophenotyping and molecular studies, performed later, established a diagnosis of de novo B-cell precursor leukemia/lymphoma with MYC, BCL2 rearrangements (Double-hit lymphoma). MYC, BCL2 rearrangements are rarely reported in precursor B-lymphoma/leukemia which carry dismal prognosis. In this report, we illustrate autopsy findings of PL in an elderly gentleman who presented with ascites for evaluation.

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ABSTRACT Sickle cell trait (SCT), a heterozygous state characterized by hemoglobin AS, occurs commonly in sub-Saharan Africa, South America, Central America, India, and the Mediterranean countries. SCT is compatible with a normal lifespan and is not commonly regarded as a cause of morbid illness or death compared to its homozygous counterpart. We describe a case of fatal sickling-associated microvascular crisis, identified on post mortem evaluation in a previously undiagnosed 21-year-old military recruit with sickle cell trait. The individual presented with repeated syncope episodes during his training and was autopsied in the pursuit of cardiac anomalies and heat syncope. During the terminal episode, he collapsed and died of severe metabolic complications as he struggled to complete an organized run during routine training activities. To our knowledge, this is the first report of fatal sickling-associated crisis in a military recruit with sickle cell trait from India. This case serves to remind all armed forces and sports physicians of the importance of screening a recruit who is unable to complete exertional physical training for the presence of sickle cell trait.

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ABSTRACT Aortoduodenal fistula (ADF) is the most common type of aortoenteric fistula (AEF). This is a rare entity, which produces communication between an abdominal aortic aneurysm (AAA) and the gastrointestinal tract (GIT), resulting in massive gastrointestinal bleeding. AEF/ADF is difficult to recognize clinically, with the classical triad of symptoms including a pulsating, palpable mass, abdominal pain, and GIT bleeding. AEF/ADF can be classified into primary when a communication between an AAA and the GIT develops with no history of prior aortic reconstructive surgery, and secondary, where the communication is on the background of previous aortic reconstructive surgery. Herein we present a case report of a 75-year-old Caucasian male patient with a clinical history of AAA, who presented with massive GIT bleeding and expired shortly after. An autopsy revealed communication between an atherosclerotic AAA and the lower third of the duodenum.

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ABSTRACT Blood transfusion for chronic anemia can lead to acute or decompensated heart failure in patients who have fluid overload as part of their compensatory response and/or have intrinsic heart disease, and then it could be fatal in such clinical scenarios. This is the report of a case of profound chronic anemia in a young male patient, who was not transfused and then developed confusion followed by terminal cardiopulmonary arrest. Autopsy revealed severe trichuriasis to be the cause of the anemia, along with severe ascariasis, but minimal intrinsic brain disease. This supports the conclusion that anemia was the cause of the confusion, and the lesson that confusion may be a sign that the benefit of blood transfusion outweighs the risk in a patient with severe chronic anemia.

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ABSTRACT Fetal akinesia deformation sequence (FADS), or Pena-Shokeir phenotype is a constellation of deformational changes resulting from decreased or absent fetal movement, and include arthrogryposis, and craniofacial and central nervous system anomalies. We report an autopsy case of a 36-6/7week female neonate with a normal female karyotype and chromosome microarray demonstrating findings consistent with FADS. We provide a detailed examination of the severe and complex central nervous system abnormalities, including marked pontocerebellar hypoplasia and cortical and cerebellar migration and gyration defects. This case represents a rare detailed examination of the central nervous system of a patient with FADS.

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ABSTRACT Varix of the lower extremities is a common entity that eventually presents fatal outcome. Fatal massive bleeding due to rupture of a peripheral varicose vein is rare. The estimated incidence of these cases is 1/1000 autopsies. The case we present is unique among 26,054 autopsies performed in Milan from 1993 to 2020. It describes the investigations carried out in the suspicion of a non-natural event in an elderly woman. She was found dead at home with a large volume of blood near her feet that drained from the right leg. Pathological examination disclosed that the hemorrhage occurred by the rupture of a venous varix of the lower limb. Cases of fatal hemorrhage from peripheral variceal rupture are insidious and require proper characterization. The bloodstain pattern analysis, careful autopsy dissection by layers to demonstrate the rupture, and histologic examination of the lesion are the essential elements to find out the actual cause of death.

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ABSTRACT Primary hepatic gas gangrene is a form of primary abdominal gas gangrene. The condition is caused by Clostridium perfringens, other clostridia, and non-clostridia bacterial species producing gas. Unlike classical gas gangrene or myonecrosis, the disease develops without a wound or a port of entry. Instead, gas-producing bacteria in the gastrointestinal tract colonize an underlying pathological process with foci of necrosis, producing excessive gas and spreading hematogenously to other organs. Herein we present two autopsy cases of primary hepatic gas gangrene diagnosed on autopsy, with the gross and histological changes that can be considered specific for this rare condition. Both patients had severe underlying liver disease-prone for this entity development. The gross changes in the cases are postmortem subcutaneous emphysema, skin bullae with pooled blood, pneumothorax, pneumoabdomen, abundant gas in the circulatory system, porous structure of the internal organs (tissue gas bubbles), and advanced tissue lysis, not corresponding to the post mortem time. Histology showed optically empty areas of varying size in the internal organs, which weave the structure of the organs and rod-shaped bacteria with scarcity or complete absence of inflammatory reaction.

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ABSTRACT Leigh syndrome is an inherited neurodegenerative disorder of infancy that typically manifests between 3 and 12 months of age. The common neurological manifestations are developmental delay or regression, progressive cognitive decline, dystonia, ataxia, brainstem dysfunction, epileptic seizures, and respiratory dysfunction. Although the disorder is clinically and genetically heterogeneous, the histopathological and radiological features characteristically show focal and bilaterally symmetrical, necrotic lesions in the basal ganglia and brainstem. The syndrome has a characteristic histopathological signature that helps in clinching the diagnosis. We discuss these unique findings on autopsy and radiology in a young infant who succumbed to a subacute, progressive neurological illness suggestive of Leigh syndrome. Our case highlights that Leigh syndrome should be considered in the differential diagnosis of infantile-onset, subacute neuroregression with dystonia and seizures, a high anion gap metabolic acidosis, normal ketones, elevated lactates in blood, brain, and urine, and bilateral basal ganglia involvement.

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ABSTRACT Spindle cell squamous cell carcinoma (SpSCC) is a rare biphasic malignant neoplasm, uncommonly affecting the oral cavity. The SpSCC diagnosis is difficult, especially when it exhibits inconspicuous morphology, inadequate tissue sampling, or association with an exuberant inflammatory reaction. Post-radiotherapy recurrent SpSCC occurring at the same site of conventional SCC is a rare phenomenon. A 59-year-old man was complained of “painful injury on the tongue” with 20 days of duration. He reported smoking and alcohol consumption. Medical history revealed conventional SCC on the tongue treated with surgery and radiotherapy 10 years ago. Intraoral examination showed a polypoid lesion with ulcerated areas, measuring 3 cm in diameter, on the tongue and floor of the mouth, at the same site of previous conventional SCC. The microscopical analysis showed small foci of carcinomatous component admixed with an exuberant inflammatory reaction. Immunohistochemistry highlighted the sarcomatoid component. Both malignant components were positive for EMA, CD138, p40 (deltaNp63), p63, and p53. Moreover, CK AE1/AE3 evidenced the carcinomatous component, whereas vimentin stained the sarcomatoid component. The Ki-67 was >10%. The current case emphasizes the importance of immunohistochemistry in the differential diagnosis of SpSCC from mimics and documents a rare complication of Ionizing Radiation.

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ABSTRACT Undifferentiated carcinoma of the pancreas with osteoclast-like giant cells (UCOGCs) is an extremely rare morphologically and clinically distinct variant of pancreatic ductal adenocarcinoma (PDAC), exhibiting a characteristic component of reactive osteoclast-like giant cells admixed with neoplastic mononuclear cells. Sommers and Meissner first described it in 1954 as an “unusual carcinoma of the pancreas”. Later it acquired many different names. In 2010, the WHO classified these tumors as a variant of PDAC under the heading of “undifferentiated carcinoma with osteoclast-like giant cells”. Here we describe the first case of pancreatic mixed neuroendocrine-non-neuroendocrine neoplasms (MiNEN) composed of UCOGC and pancreatic neuroendocrine tumor (NET), which occurred in a 78-year-old man with biliary colic and pancreatitis. The mass did not respond to the chemotherapy, and he soon developed liver metastasis from the NET component, and unfortunately, the patient passed away 10 months later. Since UCOGC is extremely rare, and its association with NET has not been reported yet, our case expands the knowledge regarding its unusual presentation and poor prognosis.

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ABSTRACT Ossifying fibromyxoid tumor (OFMT) is a rare mesenchymal soft tissue benign neoplasm with an uncertain line of differentiation, which arises most frequently in extremities. The head and neck region involvement is uncommon, with only ten intraoral cases published in the English-language literature. One additional case of OFMT is reported here, including a literature review of intraoral reported cases. A 45-year-old female patient presented a painless nodule involving the buccal mucosa of approximately two years duration, measuring nearly 1.3 cm in maximum diameter. The main histopathological features include ovoid to round cells embedded in a fibromyxoid matrix with a perpheral shell of lamellar bone. Immunohistochemically, the tumor showed immunoreactivity for vimentin and S100. No recurrence has been detected after 7 years of follow-up.

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ABSTRACT Merkel cell carcinoma is an aggressive malignancy that frequently recurs/disseminates, but metastases to the genitourinary tract are rare. Only eight cases of Merkel cell carcinoma metastatic to the testis are reported. We describe the ninth case of this event and provide a review of the literature. A 58-year-old man diagnosed with Merkel cell carcinoma of the wrist, presented, 37 months later, a recurrence in the form of a testicular metastasis. The tumor consisted of a monotonous proliferation of small, blue, round cells, with immunoexpression of neuroendocrine markers and the typical dot-like paranuclear immunostaining for cytokeratin 20, in the absence of immunostaining for cytokeratin 7. The patient is alive with no evidence of disease. Clinicians should be aware of the possibility of metastatic dissemination to the testis since genital examination/imaging is not part of routine follow-up for these patients, but timely orchiectomy may be curative.

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ABSTRACT Tuberculosis is an infectious disease that involves any organ. However, the primary pituitary tuberculosis is an extremely rare disease. Intracranial tuberculomas account for 0.15-5% of intracranial space-occupying lesions, of which, pituitary as the primary site is unusual, and easily misdiagnosed as pituitary adenoma. In this setting, the late diagnosis can result in permanent endocrine dysfunction. We report the case of a 50-year-old woman who presented to the neurosurgery outpatient department with complaints of progressively increasing headache and diminished vision over the last year. On the clinical examination, the patient was conscious and oriented. The routine hematological and biochemical workup showed an increased erythrocyte sedimentation rate (ESR) and increased prolactin levels. The radiological working diagnosis was consistent with pituitary macroadenoma. No other radiological and/or clinical clue that could elicit the suspicion of pulmonary or extrapulmonary lesions of tuberculosis was found. The transsphenoidal endonasal tumor excision was done. The histopathology showed numerous epithelioid cell granulomas, Langhans giant cells along with scant necrosis. Ziehl Neelsen staining demonstrated acid-fast bacilli, and the final diagnosis of pituitary tuberculoma was made. We report this rare case of pituitary lesion that may be included in the differential diagnosis of sellar lesions to avoid unnecessary surgical interventions, especially in regions where the disease is endemic.

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ABSTRACT Appendiceal tumors comprise a variety of histologic types, including appendiceal mucinous neoplasms, which can be grouped as premalignant lesions, tumors of uncertain malignant potential, and malignant lesions. The appendiceal mucinous neoplasms are characterized by mucinous epithelial proliferation with extracellular mucin and pushing tumor margins, commonly an incidental finding during operative exploration. We report the case of a low-grade appendiceal mucinous neoplasm presenting as a subepithelial lesion in Crohn´s Disease patient. The diagnosis was not straightforward, and only surgical resection allowed an accurate diagnosis. Although Inflammatory Bowel Disease is a risk factor for the development of colorectal neoplasms, the absolute risk for appendiceal tumors is uncertain. The frequency of progression to malignancy remains to be determined.

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ABSTRACT Bowel obstructions can have a variety of causes, including impacted feces, adhesions, volvulus, non-internal hernias, and in rare cases internal hernias. We report a 63-year-old woman who presented to the emergency department with severe abdominal pain, nausea, vomiting, and obstructive symptoms that had started 12 hours earlier. A computed tomographic scan of the abdomen and pelvis showed a right internal hernia with a cecal bascule traversing through the foramen of Winslow, concerning for a closed-loop obstruction. The patient underwent an exploratory laparotomy with cecal bascule reduction and cecopexy. Given the increased mortality risk if undiagnosed, it is important to remain aware of internal hernias. Patient outcomes are markedly improved with early diagnosis and surgical intervention.

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ABSTRACT Rhinosporidiosis is a chronic infection of the mucous membrane caused by the Rhinosporiduim seeberi, which infects through transepithelial penetration. Although described worldwide, this entity is mostly found in the western hemisphere, afflicting young people, predominantly males, associated in many cases with recreational or professional contact with bath in ponds, rivers, or stagnant waters. The clinical features are varied depending on the affected membrane, in some cases mimicking other diseases postponing the correct diagnosis. Although nasal obstruction and epistaxis are the common clinical presentations in sinonasal rhinosporidiosis, patients with epiphora without a nasal mass often challenge the diagnosis. In the present case, we have documented a case of isolated lacrimal sac rhinosporidiosis masquerading as chronic dacryocystitis, which was successfully managed by endoscopic excision, accompanied by a literature review.

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ABSTRACT Inflammatory Myofibroblastic Tumor (IMT) is a rare pathologic entity that was first described in 1973. This lesion is most commonly found in the lungs, but other organs’ involvement has also been reported. Intracranial location of Inflammatory Myofibroblastic Tumor is rare, and the first case was reported in 1980. An intriguing fact about the intracranial IMT is its resemblance with meningioma on clinical presentation and neuroimaging. We came across a case of intracranial Inflammatory Myofibroblastic Tumor (IIMT) in a 27-year-old male who presented with recurrent episodes of seizures and was diagnosed as meningioma on neuroimaging. The lesion did not subside with medical management and kept on progressing in size. The patient had to undergo surgery, and diagnosis of Inflammatory Myofibroblastic Tumor was ascertained on histopathology. This ‘surprise’ diagnosis prompted us to review the literature on all cases of IIMTs reported to date to better understand the entity and its implications. In this review article, we present our observations regarding various studied parameters, including patient profile, clinical presentation, site of involvement, focality of the lesion, special associations, and lines of management of the 49 published cases of IIMTs.

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ABSTRACT Immune-mediated encephalitis as an adverse event due to checkpoint inhibitors is very rare. We describe herein the case of a 38-year-old woman with metastatic triple-negative breast cancer who developed seizures and somnolence twelve days after receiving the first dose of Atezolizumab. Work up ruled out all infectious etiologies, and the patient was eventually diagnosed with immune-mediated meningoencephalitis. Symptoms recovered with a high-dose of steroids, and she was found to have an excellent response on follow-up imaging, which raised the question of whether a relationship exists between the occurrence, and severity of the adverse event and the response to treatment. Only a few other cases of atezolizumab–related encephalitis have been published. Early recognition and treatment are crucial; the reason why we are describing this case along with a review of the literature and a review on all the neurological immune-related adverse events due to the different checkpoint inhibitors.

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ABSTRACT Androgen secreting adrenocortical carcinoma (ACC) is a very rare disease with a poor prognosis. Approximately 80% of tumors are functional, most commonly secreting glucocorticoids. We herewith report a case of a huge functional ACC of the right adrenal gland in a 33-year-old female who presented with complaints of hirsutism, amenorrhea and an abdominal lump. On abdominal examination a large lump was palpable in the right hypochondrium reaching up to the umbilicus. Contrast-enhance computed tomography (CECT) revealed a mass in the right suprarenal region. The tumor measured 29 cm × 20 cm × 12 cm and weighed 7.8 kg, the largest reported case of ACC in the world to the best of our knowledge.

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ABSTRACT Villous adenoma is uncommonly seen in the urogenital tract and is even more rarely seen in the upper urinary tract and renal pelvis. Like colorectal adenomas, these neoplasms can transform into adenocarcinoma. The preoperative diagnosis is challenging due to their frequent association with hydronephrosis. Herein, we present the case of a villous adenoma of the renal pelvis in a 62-year-old man presenting with recurrent urinary tract infection. The computed tomography scan showed marked hydronephrosis but no suspicious mass in the right kidney. A laparoscopic right nephrectomy was performed. Gross examination revealed a dilated renal pelvis with an irregular exophytic lesion in the renal pelvis’s upper surface. The histopathological examination showed slender, elongated villi with thin fibrovascular cores, consistent with villous adenoma morphology. Isolated villous adenomas have a favorable prognosis. However, the pathologist should undertake a search for an invasive component.

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ABSTRACT Paragangliomas are rare, encapsulated, benign neuroendocrine tumors that can arise from the adrenal medulla or extra-adrenal paraganglia. Extra-adrenal paragangliomas may develop a gangliocytic component with ganglion cells (Gangliocytic paragangliomas). Nearly 25%of cauda equina paragangliomas are gangliocytic paragangliomas. Here, we describe the case of a 35-year-old male who presented with weakness of both lower limbs over the last two months. Radiological findings were suggestive of myxopapillary ependymoma. However, the histopathological examination revealed a tumor with cells arranged in sheets, papillae, lobules, and around vessels forming pseudo rosettes. Ganglion cells were seen in small groups and, also singly. Tumor cells were immunopositive for chromogranin, synaptophysin, and S-100. Ganglion cells were immunopositive for synaptophysin, NSE, and NFP. A final histological diagnosis of Gangliocytic paraganglioma (WHO grade I) was made. To date, only nine gangliocytic paraganglioma cases have been previously reported, and to the best of our knowledge, this is the largest gangliocytic paraganglioma.

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ABSTRACT Primary malignant giant cell tumor (PMGCT) is a diagnosis based on the presence of a high-grade sarcomatous component along with a typical benign giant cell tumor (GCT). We report the first case of PMGCT of the sternum in a 28-year-old male with painless swelling over the manubrium sterni. The differential diagnoses of PMGCT and giant cell-rich osteosarcoma were considered. Surgical resection was performed, and the reconstruction was done with a neosternum using polymethyl methacrylate and prolene mesh. At 30 months follow-up, the patient is disease-free.

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ABSTRACT Background Hemangiomas are benign neoplasms of capillary proliferation that arise from a developmental anomaly where angioblastic mesenchyme fails to form canals. Most hemangiomas arise in the head and neck region, either superficially in the skin or deeper within endocrine organs such as the parotid gland. Parathyroid hemangiomas, however, are extremely rare, with only five cases previously reported in the literature. Case presentation Herein, we present a case of a 68-year-old man with a hemangioma almost completely replacing the right upper parathyroid gland, grossly measuring 1.3 × 1.3 × 1.2 cm and weighing 700 mg, associated with primary hyperparathyroidism. Conclusions Parathyroid gland enlargement due to vascular neoplasms such as hemangiomas can mimic, both clinically and radiographically, hyperplasias and/or adenomas. Surgeons need to be aware of the presence of this entity and should consider it in the differential diagnosis of hyperparathyroidism or parathyroid gland enlargement.

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ABSTRACT The sacrococcygeal region is the most common site for the extragonadal germ cell tumors comprising seminomatous and non-seminomatous tumors. Seminomatous tumors are seminomas, and non-seminomatous tumors comprise mainly teratoma (mature and immature), yolk sac tumor (YST), embryonal carcinoma (EC), and choriocarcinoma. These tumors occur in newborns, infants, and adolescents. Other common sites for extragonadal germ cell tumors are the brain and mediastinum, although they may occur anywhere in the body. These tumors may occur in mixed as well as pure form. So, sectioning from different areas should be done before labeling them as pure germ cell tumors. YST, in its pure form, is rare and therefore should not be missed as it is chemosensitive. The patient should be thoroughly assessed clinically. Imaging also becomes necessary while evaluating swelling in the sacrococcygeal region and can aid in differentials. When the clinical and imaging suspicion of either Sacrococcygeal teratoma or other germ cell tumor is high, serum biomarkers as alfa-fetoprotein should be requested. The serum levels are necessary and should be done preoperatively, postoperatively, and during the course of chemotherapy as follow-up. However, the final diagnosis rests on the histopathological diagnosis. We report one such case of pure YST in the sacrococcygeal region in a 9-month-old female child. The imaging suggested sacrococcygeal teratoma type 4, and high alfa-fetoprotein levels were determined postoperatively.

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ABSTRACT Adamantinoma of the long bones is an exceedingly rare and slow-growing tumor that affects the diaphysis of long bones, particularly the tibia. Based on the pattern of the epithelial cell component and the presence or absence of the osteofibrous dysplasia-like element, several histological variants have been described, such as (i) tubular (the most frequent), (ii) basaloid, (iii) squamous, (iv) spindle variant, (v) osteofibrous dysplasia –like variant, and (vi) Ewing’s sarcoma – like adamantinoma (the least frequent). The diagnosis may be challenging since this tumor may be mistakenly interpreted as carcinoma, myoepithelial tumor, osteofibrous dysplasia, and vascular tumor. We report the case of a 41-year-old male who presented with swelling over the right leg associated with pain. The X-ray showed a lytic lesion of the right-sided tibia. The diagnosis of adamantinoma was made based on the clinico-radiological, histomorphology, and immunohistochemical findings. Histologically, classic adamantinoma is a biphasic tumor characterized by epithelial and osteofibrous components in varying proportions and differentiating patterns. The diagnosis can be confirmed by immunohistochemistry for demonstrating sparse epithelial cell nests when the radiological features are strongly consistent with adamantinoma. This case is highlighted because the epithelial component can lead to a misdiagnosis, particularly when the clinico-radiological features are overlooked. Adamantinoma of long bones has the potential for local recurrence and may metastasize to the lungs, lymph nodes, or other bones. The prognosis is good if early intervention is taken.

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ABSTRACT Intraconal dermoid cysts are very unusual in routine clinical practice. Clinical symptoms depend upon the site and extension of the lesion. Though rare, proptosis, diplopia, and orbital pain are the presenting symptoms encountered in patients with an intraorbital dermoid cyst. Although radiology can be diagnostic, a complete correlation with the final histopathology is always mandatory for its confirmation. Endoscopic excision of the cyst ensures a complete cure for the disease without any intraoperative/postoperative complications.

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ABSTRACT Squamous odontogenic tumor (SOT) is a rare benign neoplasm of the jaw that likely arises from remnants of the dental lamina. It is a slow-growing lesion, with a radiolucent appearance in the central variant. Microscopically, SOT shows islands of squamous epithelium supported by fibrous stroma. In rare cases, squamous odontogenic tumor-like proliferation (SOT-LP) can be observed arising from odontogenic cysts (SOT-LPOC). Herein, we describe the case of a 42-year-old man who presented with discreet bleeding in the maxillary gingiva. Imaging revealed a well-defined, ovoid-shaped lesion with sclerotic margins involving tooth #18 in the intraosseous location. Fine needle aspiration supported the cystic nature of the lesion. After surgery, microscopy revealed a dentigerous cyst showing SOT-LP features. There was no recurrence after a 3-year follow-up. To the best of our knowledge, this is the first report of a dentigerous cyst showing SOT-LP features in the maxilla. Such cysts should be identified to avoid misdiagnosis, with the finding having therapeutic and prognostic implications.

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ABSTRACT Oro-facial-digital syndrome is a group of rare heterogeneous hereditary disorders characterized by abnormalities of the oral cavity, face and digits, along with varying degrees of mental retardation. Currently, Oro-facial-digital syndrome has been classified into 14 types and two additional unclassified variants have been proposed. Amongst the various variants described, Oro-facial-digital syndrome type I is the most common. We report an interesting subclinical sporadic case of Oro-facial-digital syndrome type I in a 21-year-old female patient. Interestingly, our patient presented with a few novel hitherto unreported clinical findings like midline pits in the philtrum area and a hamartomatous proliferation of tissue in the anterior maxillary alveolar gingival region. This case report highlights the importance of prudent histopathological-clinical correlation, which can direct the flow of clinical investigations leading to the detection and diagnosis of unsuspected conditions as learned in this case. We would also like to emphasize that comprehensive examination of new born for structural abnormalities of the orofacial region is crucial to early diagnosis of syndromes and subsequent referral for further evaluation and management.

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ABSTRACT Myositis ossificans (MO) is a benign, ossifying lesion that usually affects the skeletal muscle. The rare non-traumatic form of MO can cause diagnostic dilemma and management issues. These lesions, however, have similar radiology and histopathological characteristics described in the more frequently encountered traumatic forms. Depending on the stage of the lesion, the inherent feature of myositis ossificans varies, and so does the management of the lesion. We describe a non-traumatic MO occurring in latissimus dorsi of a young girl and discuss the review of literature on this rare subtype.

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ABSTRACT Epidermal inclusion cyst (EIC) of the thyroid is extremely rare in the clinical practice. A handful of cases have been documented in the past in the world literature. A giant EIC of the thyroid is hitherto unreported. This lesion may arise from the squamous metaplasia of the thyroid follicular cells. Though non-neoplastic, giant forms can cause compression of the vital structures of the neck. In the present case, we have described a giant epidermal inclusion cyst successfully managed with surgical management.

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ABSTRACT Angiomyolipoma with epithelial cysts (AMLEC) is a recently described entity and is an uncommon subtype of kidney angiomyolipomas. AMLEC is a benign entity but usually masquerades a renal cell carcinoma on imaging examination. AMLEC has a distinct histological and immunohistochemical staining pattern, which helps in the pathological diagnosis. We present a rare case of AMLEC in a 26-year-old female, which was provisionally diagnosed as renal cell carcinoma on radiology. We also summarize the differential diagnosis of this rare variant, its characteristic features, and a review of the literature.

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ABSTRACT Thymomas are a heterogeneous group of tumors arising from the epithelium of the thymus. They are categorized by the proportion of neoplastic epithelia to lymphocytes and by the degree of cytologic atypia. Thymomas constitute 0.2-1.5% of all malignancies and nearly all occur in patients over 20 years. We reviewed the available literature and found less than 50 cases of thymoma reported in children (<18 years of age), the youngest being 4 years old, and no cases in newborns. They represent less than 1% of all mediastinal tumors in children. Due to the limited number of cases in the pediatric population, the diagnosis and treatment in this population is extremely challenging. Thymomas in all age groups may be associated with paraneoplastic syndromes, being myasthenia gravis the most common, which is associated with a worse prognosis in the pediatric population. We present the first case of a newborn infant with congenital thymoma. This case demonstrates a rare tumor in an unusual age group and emphasizes the importance of multidisciplinary teamwork in the decision-making and management of this condition.

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ABSTRACT Mucosal Schwann cell hamartoma (MSCH) is a rare benign neurogenic tumor characterized by pure S100p positive spindle cell proliferation. Most cases occur in the distal colon. Involvement of the gall bladder is exceedingly rare. There have been no reports of recurrence or a syndromic association with MSCH. Herein, we describe a case of MSCH of the gallbladder in a 55-year-old female patient with prior history of gastrointestinal neurofibromas who presented with abdominal pain. MR imaging revealed choledocholithiasis, gallbladder thickening, and marked biliary and pancreatic ductal dilation. The patient subsequently underwent cholecystectomy with choledochoduodenostomy. Histologic evaluation of the gallbladder showed diffuse expansion of the mucosa with S100p positive cells with spindly nuclei and indistinct cytoplasmic borders and diagnosis of MSCH of the gallbladder was rendered.

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ABSTRACT Myeloid sarcoma (MS) is a rare extramedullary neoplasm of myeloid cells, which can arise before, concurrently with, or following hematolymphoid malignancies. We report 04 such cases of MS, diagnosed in this institute over a period of 6 years, during various phases of their respective myeloid neoplasms/leukemias. These cases include MS occurring as a relapse of AML (Case 1), MS occurring as an initial presentation of CML (Case 2), MS occurring during ongoing chemotherapy in APML (Case 3), and MS presenting as a progression of MDS to AML (Case 4). In the absence of relevant clinical history and unemployment of appropriate immunohistochemical (IHC) studies, these cases have a high risk of being frequently misdiagnosed either as Non-Hodgkin’s Lymphoma (NHL) or small round cell tumors or undifferentiated carcinomas, which may further delay their management, making an already bad prognosis worse. This case series has been designed to throw light on the varied presentation of MS and the lineage differentiation of its neoplastic cells through the application of relevant IHC markers along with their clinical correlation.

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ABSTRACT Lymphangiomas are rare and correspond to 0.7% to 4.0% of mediastinal tumors, and isolated mediastinal location occurs in 1% of cases. They are benign tumors that originate from a congenital malformation of the lymphatic vessels and are diagnosed more frequently in children less than 2 years of age. Chylous ascites is a clinical manifestation of thoracic duct lymphangioma and is composed of lymph accumulation caused by dilation of this lymphatic channel. It appears milky in the peritoneal cavity, containing triglyceride levels higher than 200 mg/dl. We report the case of a young patient with chylous ascites and lymphangioma of the thoracic duct, who was conservatively treated with octreotide and a low-fat diet with medium-chain triglycerides.

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ABSTRACT Although uncommon in patients under oral therapy, bisphosphonate-related osteonecrosis of the jaw (BRONJ) can be a very severe issue. Early intervention with surgical resection should be the preferable method of treating any stage of the disease, resulting in better outcomes and decreasing the morbidity of this condition. A 77-year-old female patient attended the Special Care Dentistry Centre of the University of São Paulo Faculty of Dentistry (CAPE FOUSP) complaining mainly of “an exposed bone that appeared after tooth extraction performed six months earlier”. The patient was diagnosed with osteonecrosis associated with bisphosphonate (sodium ibandronate) and surgically treated with removal of bone sequestration and antibiotic therapy. The patient was followed up for six years (a total of 6 appointments), presenting good general health and no sign of bone exposure. Imaging findings showed no changes related to BRONJ either.

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ABSTRACT Scleroderma is a rare autoimmune disease characterized by excessive collagen production. The oral manifestations of the patient with scleroderma can include microstomia, xerostomia, and changes in the resorption teeth. We report the case of a 7-year-old female patient diagnosed with systemic scleroderma where photobiomodulation therapy was used to treat xerostomia associated with hyposalivation. She attended a pediatric clinic and presented with dry and rigid facial skin, trismus, xerostomia, malocclusion, and difficulty swallowing. Stimulated salivary flow was assessed before, during, and after treatment. Photobiomodulation therapy was conducted at four points at the sublingual glands with 660 nm, 100 mW, and 0.8 J/cm2 to each point; eight points at the parotid glands; and six points at the submandibular glands with 808 nm, 100 mW, and 0.8 J/cm2 for 8 seconds at each point. After this therapy, an increase in salivary flow, remission of the xerostomia, and an improvement in mastication and swallowing were observed. Photobiomodulation therapy was effective in controlling xerostomia in this pediatric patient, resulting in increased salivary flow and an improvement in her quality of life.

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ABSTRACT The Numb Chin Syndrome (NCS) is defined as facial and oral numbness restricted to the mental nerve’s distribution involving the lower lip, skin of the chin, or gingiva of the lower anterior teeth. Hypoesthesia can occur unilaterally or bilaterally. Although this syndrome is rare, its importance is related to the fact that it represents the clinical manifestations of malignant diseases. Breast cancer and non-Hodgkin lymphoma are the most common cause of NCS. The patient, a 58-year-old woman, treated for a Burkitt Lymphoma (BL) nine years ago, described a two-week history of change in sensitivity and pain in the chin region, without relief with the use of analgesics. She had no headache, speech disturbance, dysphagia, visual disturbance, or other neurological symptoms. No surgical intervention has been performed recently. The intraoral examination revealed a healthy oral mucosa and a small area adjacent to the right mental nerve region that was uncomfortable to palpation. No changes were found in the bone trabeculae at cone-beam computed tomography. The contrasted magnetic resonance features made it possible to identify a change in the mandibular body extending to the entire right side, coinciding with the patient’s complaint, indicating a probable mandibular medullary invasion. The patient was submitted to a biopsy to rule out a possible recurrence of BL. The microscopic findings were consistent with the diagnosis of BL. The present report described a very unusual presentation of late recurrent BL nine years after the first treatment, which manifested as an NCS.

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ABSTRACT Renal cell carcinoma (RCC) is a malignant disease that is often diagnosed at a metastatic stage. The head and neck represent up to 3% of the metastatic RCC, and the paranasal sinus area is one of the least involved sites. Here, we introduce the case of a 74-year-old female patient who presented with a history of traumatic nasal bleed. A cranial computed tomography scan and magnetic resonance imaging showed a fronto-ethmoidal mass with pachymeningeal involvement. A nasal biopsy from the paranasal sinuses was taken. On histopathological examination, metastatic clear cell carcinoma was the main hypothesis, which later was confirmed to be RCC on immunohistochemistry. On further radiological examination, an exophytic mass was depicted in the kidney’s upper and middle pole. The patient had no renal complaints and was asymptomatic. Fronto-ethmoidal sinus is a rare site for metastatic RCC, especially in cases where the patient is asymptomatic. Early detection by keeping RCC metastasis as the differential diagnosis in such cases can lead to early treatment and improve the overall survival of the patient.

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ABSTRACT Xanthogranulomatous pyelonephritis (XGP) is a rare variant of chronic pyelonephritis. It is characterized by progressive parenchymal destruction caused by chronic renal obstruction due to calculus, stricture, or rarely tumor, resulting in kidney function loss. Herein, we describe the case of a 36-year-old female who presented with left loin pain, left lower limb pain, and dysuria. On contrast-enhanced computed tomography (CECT), multiple abscesses and an obstructive staghorn calculus were depicted in the left kidney with the classical appearance of “Bear Paw Sign.” An abscess with calculi was also present within the left psoas muscle. Though psoas muscle abscess in association with XGP was described, a ureteric fistula and calculi within the psoas muscle have not yet been reported in the literature. Left nephrostomy was performed, which came out to be positive for E. coli on culture. The patient underwent left nephrectomy, and the histopathological report of the surgical specimen confirmed XGP.

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ABSTRACT The infestation of the human body by maggots has been reported worldwide and occurs most commonly in people of lower socioeconomic status and poor personal hygiene. Urogenital is the rarest site of myiasis presentations. Here we report the case of a 20-year-old, sexually inactive female student who presented with a necrotic growth in the paraurethral region infested with numerous maggots. The lesion involved the urethra and the bladder base. She was treated with debridement and bladder irrigation. The cystoscopy and local examination performed 2 weeks after admission, confirmed the complete healing of the urogenital lesion. Managing this patient’s unique challenge was to assess the extent of the involvement and removal of all maggots from the deepest wound portion. The female internal and external urogenital myiasis is a very occasional and under-reported health hazard. Reporting such cases increases the public and physician awareness about the mode of presentation, right diagnosis, and available treatment options.

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ABSTRACT Venous aneurysm of the head and neck is a rare clinical entity due to its asymptomatic nature and tendency of clinicians to report only surgical results. Whereas the primary aneurysm of internal jugular vein (IJV) in children is being increasingly recognized, secondary aneurysms of veins of the head and neck in adults, notably the external jugular vein (EJV) aneurysm remains only in anecdotal case reports. We present the case of a 63-year-old previously healthy woman who presented with a gradually progressive right lateral neck swelling over the last 18 months. Following the evaluation, she was diagnosed as a case of isolated spontaneous right-sided EJV aneurysm and was managed by surgical excision of the aneurysm.

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ABSTRACT Angiosarcoma is a rare neoplasm, constituting only 2% of all the soft tissue tumors and most frequently involves the skin of the head and neck region in elderly males. They are extremely aggressive tumors with high rates of metastasis and poor outcomes. We report a unique case of angiosarcoma involving an unusual site – upper alveolus and maxilla in a young patient highlighting the diagnostic challenges in such a scenario. A 29 years old female presented with a non-healing wound of the oral cavity, which had progressed to the current maximum size of 6.4 cm within one month. Magnetic resonance imaging (MRI) scan revealed the involvement of maxilla up to the floor of the orbit and adjacent soft tissue. However, no distant metastasis was detected on Positron Emission Tomography (PET) scan. Biopsy of the lesion showed an irregular, highly pleomorphic, and mitotically active epithelioid soft tissue tumor conclusively diagnosed as angiosarcoma.

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ABSTRACT Retroperitoneal liposarcomas are rare tumors arising from the soft tissue of the retroperitoneum and are of mesenchymal cell origin. They can reach a large size prior to causing symptoms and generally have a poor prognosis. We present the case of a 93-year-old lady presenting with a large retroperitoneal liposarcoma at the site of a previous colonic anastomosis for the adenocarcinoma treatment. It caused minimal symptoms initially, but surgical resection was undertaken when the tumor was found to be growing significantly in size. However, due to the tumor’s location and its invasion into surrounding structures, the resection was not feasible and subsequently abandoned. A retroperitoneal liposarcoma arising from the site of a previous colonic resection has not been previously described. A review of the diagnosis and current management of these lesions is also given.

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ABSTRACT B-cell prolymphocytic leukemia (B-PLL) is an extremely rare disease, accounting for approximately 1% of the lymphocytic leukemias. B-PLL generally occurs in older people. It is characterized by the presence of more than 55% prolymphocytes in the peripheral blood (PB), no or minimal lymphadenopathy, massive splenomegaly, and very high white blood cell counts. The prognosis of B-PLL patients is generally poor, with a median survival of 3 years, although a subset of patients may show a prolonged survival. Herein, we report a case of a 70-year-old male with weakness, generalized lymphadenopathy, and moderate splenomegaly at the initial presentation. Hematologic examination revealed lymphocytic leukocytosis, favoring a chronic lymphoproliferative disorder (CLPD). The key to decoding the precise CLPD was a combination of the clinical profile, morphologic findings on the peripheral blood and the bone marrow, immunophenotypic analysis, and cytogenetic study. The best diagnosis proffered was a de novo chronic lymphocytic leukemia/prolymphocytic leukemia. There was no prior history of lymphoproliferative disorder or lymphocytic leukocytosis. Discriminating this entity from other lymphoproliferative disorders is crucial as the treatment and prognosis are varied compared to the other lymphoproliferative disorders. The diagnostic conundrum encountered and the incredible utility of ancillary studies in such a scenario are highlighted in this study.

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ABSTRACT Secretory carcinoma of the breast (SBC) is a rare breast neoplasm. Most of the patients present at an early stage with a relatively indolent clinical course. Lymph node and distant metastasis are also very infrequent. The histomorphological features of the secretory breast carcinoma are quite characteristic. Predominantly three histological patterns, solid, microcystic, and tubular, have been noted with copious amounts of intra and extracellular secretory material. Most commonly, no positivity for estrogen receptor (ER), progesterone receptor (PR) and ERBB2(HER2/neu) is observed in SBCs. As SBC can occasionally be hormone receptor-positive, they should not be categorized in the triple-negative breast carcinoma (TNBC) group in general. A very characteristic genetic translocation t (12;15) has been noted in this rare tumor, resulting in a fusion between ETV6 and NTRK3 proteins. We present a case of a 60-year-old lady who presented with right breast lump of 1-month duration and was managed by lumpectomy and sentinel lymph node dissection. Axillary dissection was not performed because the sentinel lymph node biopsy was negative. Postoperative radiotherapy was given to the right breast with a boost to the tumor bed. No adjuvant chemotherapy was given No recurrence has been noted even after a year of the completion of treatment

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ABSTRACT Oncocytoma of the eyelid is a rare neoplasm. Oncocytoma associated with an ocular surface squamous neoplasm, namely conjunctival intraepithelial neoplasia, is very hard to find in the literature. Herein we report a case of a 53-year-old male who presented with a swelling in the right lower lid over the last 6 years, along with a growth in the conjunctiva of the same eye for the last 2 years and encroaching upon the cornea for the last 4 months. Excision biopsy of the lower lid mass showed histopathological features consistent with oncocytoma. The conjunctival tissue revealed conjunctival intraepithelial neoplasia 3 (severe dysplasia). This case documents a rare synchronous dual ocular neoplasia, a very unlikely coexistence of oncocytoma with conjunctival intraepithelial neoplasia.

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ABSTRACT Mucormycosis is an opportunistic fungal disease that commonly presents as cutaneous or rhinocerebral infections associated with immunocompromised states. It may exceptionally present as isolated involvement of the brain with a varied clinical presentation, which may be difficult to diagnose early, leading to increased mortality. Herein, we report the case of a 42-year-old immunocompetent female with left-sided limb weakness and a history of recurrent vomiting and headache for the last two years. Clinically, glioma was suspected, but histopathological examination revealed a few broad aseptate fungal hyphae. As no other organ was involved, the diagnosis of isolated cerebral mucormycosis was rendered. Reporting this case, we show an unusual presentation of a central nervous system mucormycosis masquerading a tumor in an immunocompetent patient. The case also highlights the importance of a careful histopathological examination to avoid missing the presence of occasional fungal hyphae. Ideally, recognition of fungal hyphae in the brain, during intraoperative consultation, can prompt brain tissue culture for definitive diagnosis and early empirical antifungal therapy, which may prove life-saving.

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ABSTRACT Acute hemorrhagic cholecystitis is a rare, life-threatening condition that can be further complicated by perforation of the gallbladder. We describe a patient with clinical and radiologic findings of acute cholecystitis with a gallbladder rupture and massive intra-abdominal bleeding. Our patient is a 67-year-old male who presented with an ischemic stroke and was treated with early tissue plasminogen activator. His hospital course was complicated by a fall requiring posterior spinal fusion surgery. He recovered well, but several days later developed subxiphoid and right upper quadrant pain and an episode of hemobilia and melena. A computed tomography scan revealed an inflamed, distended gallbladder with indistinct margins and a large hematoma in the gallbladder fossa extending to the right paracolic gutter. The patient also developed hemodynamic instability concerning for hemorrhagic shock. He underwent an emergent laparoscopic converted to open subtotal fenestrating cholecystectomy with abdominal washout for management of his acute hemorrhagic cholecystitis with massive intra-abdominal hemorrhage. Prompt recognition of this lethal condition in high-risk patients is crucial for optimizing patient care.

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ABSTRACT Collision tumors have been reported in various organs like the gastrointestinal tract, lung, skin, adrenals, central nervous system, lymph nodes, uterus, but are rarely seen in the ovary. Collision tumors are two histologically distinct neoplasms in the same organ without any intermixture between them. Here we present a case of a collision tumor of the ovary represented by a mucinous cystadenoma and teratoma. It is imperative for a surgical pathologist to correctly diagnose the collision tumor components and differentiate them from mixed tumors as it will dictate the appropriate treatment based on the individual biological aggressiveness of each component.

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ABSTRACT Introduction Acute liver failure (ALF) due to diffuse infiltrating solid malignancy without any focal lesions on radiographic imaging is rare. Case report A 70-year-old man was admitted due to mental confusion, abdominal pain, and ALF. Three years before, he had undergone a left nephrectomy for urothelial carcinoma followed by adjuvant chemotherapy. The abdominal computed tomography (CT) showed hepatomegaly and ascites. Ascitic fluid had transudate characteristics, with no malignant cells. Percutaneous liver biopsy (LB) showed diffuse liver infiltration of metastatic urothelial carcinoma. The patient rapidly deteriorated and died in a week due to ALF. Discussion History of solid cancer and hepatomegaly and/or liver failure without other obvious explanation should encourage to perform LB. Conclusion LB is warranted to avoid misdiagnosis, prolonged hospital stays, and delay in palliative care.

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ABSTRACT Lactating adenoma is a rare benign breast lesion that most often presents as a small (up to 3 cm), solid, well-circumscribed, solitary, painless, mobile, lobulated mass. The highest incidence occurs in primiparous women (20 to 40 years old) during the third trimester of pregnancy. However, in the rare case presented herein, in addition to its giant size (more than 10 centimeters on palpation), this lactating adenoma is distinctive due to the presence of multiple nodules, poorly defined ultrasonographic margins, worrisome radiologic features, growth since early pregnancy, presence of infarction and association with chronic mastitis. From the clinical-radiologic perspective, the differential diagnoses included abscess associated with puerperal mastitis, phyllodes tumor, and galactocele. Biopsy was performed, and pathologic examination revealed the classic characteristics of lactating adenoma with multiple infarcted areas, leading to an unexpected confirmed case of giant lactating adenoma.

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ABSTRACT Necrotizing sialometaplasia (NS) is a benign, self-limiting inflammatory entity that mainly affects the minor salivary glands located in the hard palate. Classically, NS is characterized as a nodule that evolves to a central ulcer. The most widely recognized triggering factor is an ischemic event. The diagnosis becomes a challenge in non-ulcerated NS cases which is essential to rule out the possibility of salivary gland tumors, especially the malignant ones. Here, we presented a case of a 32-year-old male patient with a 1-month complaint of a painful, slightly elevated erythematous area on the hard palate. Incisional biopsy was performed, and NS was diagnosed based on histopathological and immunohistochemical analyses. Clinicians should be aware of and consider NS as a differential diagnosis of minor salivary gland tumors, particularly when it presents as a non-ulcerated clinical aspect.

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ABSTRACT Hydrogen chloride is available commercially as an anhydrous gas or an aqueous solution, hydrochloric acid. Exposure to this gas has been associated with the development of reactive airways dysfunction syndrome. However, there are few published reports. A 37-year-old woman developed progressive bronchospasm and acute respiratory failure after cleaning an enclosed space with an unknown concentration of hydrochloric acid gas from a cleaning substance. She had no prior history of asthma or atopy. Severe bronchospasm developed, leading to hypoxemia and diffuse interstitial infiltrates, necessitating orotracheal intubation and admission to the intensive care unit. Asthma-like symptoms such as cough, wheezing, and dyspnea; requiring bronchodilators, and repeated hospitalizations are persistent a year after the accident. Pulmonary function testing showed mild airflow obstruction.

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ABSTRACT Background Myelodysplastic syndromes (MDS) mainly occur in the elderly but can rarely affect younger individuals too. The correct diagnosis relies on careful morphologic evaluation, cytogenetic/molecular results, and excluding reactive conditions mimicking MDS. We present the clinical, pathologic, cytogenetic, and molecular features of a case of MDS with excess blasts-2 (MDS-EB-2) in a 30-year-old male who was found to have pancytopenia during his hospitalization for coronavirus disease 2019 (COVID-19) and discuss the diagnostic challenges of MDS in patients with COVID-19. Case presentation A 30-year-old man presented to an outside hospital with fever, chills, weakness, coughing spells, dizziness and shortness of breath and was diagnosed with bilateral pneumonia due to COVID-19. At the outside hospital, he was found to be pancytopenic, and a subsequent bone marrow aspiration and biopsy raised concern for a COVID-19 induced hemophagocytic lymphohistiocytosis. In addition, MDS could not be ruled out. The patient was thus referred to our institute for further management. The patient’s peripheral blood showed pancytopenia with occasional dysplastic neutrophils and a few teardrop cells. Given the diagnostic uncertainty, a bone marrow aspiration and a biopsy were repeated revealing a hypercellular bone marrow with erythroid hyperplasia, megakaryocytic hyperplasia, trilineage dysplasia, increased blasts (13%), many ring sideroblasts, and mild to moderate myelofibrosis, consistent with MDS-EB-2. Chromosomal analysis revealed isochromosome 14. Next generation sequencing demonstrated SF3B1 K700E mutation. Discussion and conclusion The diagnosis of MDS can be challenging, particularly in young patients. Cytopenia and myelodysplastic features have been reported in COVID-19 patients, making the diagnosis of MDS more elusive. A careful pathologic examination of the bone marrow with ancillary studies including flow cytometry, immunohistochemistry, and cytogenetic and molecular studies in combination with a thorough clinical evaluation, leads to the accurate diagnosis.

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ABSTRACT Primary signet-ring cell carcinoma of the urinary bladder is a rare tumor. The overall incidence is approximately 0.12-0.6% of all urinary bladder malignancies. The majority of the patients present in an advanced stage with a uniformly grim prognosis. As signet-ring cell carcinomas are more common in the gastrointestinal tract, a possibility of metastasis needs to be considered. Here we report, a 42-year-old patient who presented with hematuria and was diagnosed with a urinary bladder tumor. The patient was managed with partial cystectomy and pelvic lymph node dissection. The histopathological examination confirmed primary signet-ring cell carcinoma of the urinary bladder.

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ABSTRACT Congenital medulloblastoma is a rare brain tumor that appears in less than 1% of pediatric patients. Congenital medulloblastoma has a poor prognosis and should be suspected in patients with clinical manifestations of hyporeactivity, slow suction reflexes, and the presence of hydrocephalus. Herein we present the case of a 12-day-old female newborn who developed non-communicative hydrocephalus, hyporeactivity, and hyporeflexia. Magnetic resonance imaging of her brain showed a heterogeneous and cystic mass on the posterior cranial fossa. A suboccipital craniotomy was performed. The histopathologic analysis reported a congenital medulloblastoma. She remained in hospital until her death at 112 days old. This is one of the first case reports with clinical-radiological and pathological documentation. Awareness of this diagnosis can allow prenatal intervention, rendering a better prognosis. This case report exemplifies the importance of good prenatal follow-up.

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ABSTRACT Encapsulating peritoneal sclerosis occurs due to chronic irritation of the peritoneal surface resulting in inflammation and fibrosis. Encapsulating peritoneal sclerosis usually occurs in patients requiring peritoneal dialysis (PD); however, it may also occur in liver transplant patients. The fibrosis in encapsulating peritoneal sclerosis could be severe enough to cause small bowel obstruction (SBO). Herein, we report a case of encapsulating peritoneal sclerosis secondary to liver transplantation that presented with SBO. The patient was started on Tamoxifen for encapsulating peritoneal sclerosis and evaluated at follow-up without any other intestinal obstruction episodes. This case demonstrates that encapsulating peritoneal sclerosis can occur as a liver transplant complication and present with small bowel obstruction.

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ABSTRACT Acute esophageal necrosis (AEN), also known as “black esophagus,” is an entity characterized by the circumferential black appearance of esophageal mucosa, usually associated with hypoperfusion and gastric outlet obstruction. This entity has a reported prevalence of up to 0.2%, affecting predominantly elderly men with multiple comorbidities. Most cases resolve with conservative treatment with no need of surgical intervention. However, the overall prognosis is poor, with mortality reaching one-third of cases due to the patient’s underlying illness. In this article we present three cases of patients with AEN.

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ABSTRACT Pemphigus Vulgaris (PV) is an uncommon autoimmune and blistering mucocutaneous disease. Childhood Pemphigus Vulgaris (CPV) is a pediatric variant of PV, which affects children below 12 years, being very rare among children under 10 years of age. CPV has similar clinical, histological, and immunological features as seen in PV in adults. The mucocutaneous clinical presentation is the most common in both age groups. Vesicles and erosions arising from the disease usually cause pain. A few CPV cases have been reported in the literature. This study reports a case of an 8-year-old male patient with oral lesions since the age of 3 years, and the diagnosis of pemphigus was achieved only 2 years after the appearance of the initial lesions. CPV remains a rare disease, making the diagnosis of this clinical case a challenge due to its age of onset and clinical features presented by the patient. Therefore, dentists and physicians should know how to differentiate CPV from other bullous autoimmune diseases more common in childhood.

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ABSTRACT Background Intestinal duplications are rare congenital developmental anomalies with an incidence of 0.005-0.025% of births. They are usually identified before 2 years of age and commonly affect the foregut or mid-/hindgut. However, it is very uncommon for these anomalies, to arise in the colon or present during adulthood. Case presentation Herein, we present a case of a 28-year-old woman with a long-standing history of constipation, tenesmus, and rectal prolapse. Colonoscopy results were normal. An abdominal computed tomography (CT) revealed a diffusely mildly dilated redundant colon, which was prominently stool-filled. The gastrografin enema showed ahaustral mucosal appearance of the sigmoid and descending colon with findings suggestive of tricompartmental pelvic floor prolapse, moderate-size anterior rectocele, and grade 2 sigmoidocele. A laparoscopic exploration was performed, revealing a tubular duplicated colon at the sigmoid level. A sigmoid resection rectopexy was performed. Pathologic examination supported the diagnosis. At 1-month follow-up, the patient was doing well without constipation or rectal prolapse. Conclusions Tubular colonic duplications are very rare in adults but should be considered in the differential diagnosis of chronic constipation refractory to medical therapy. Due to the non-specific manifestations of this entity, it is rather challenging to make an accurate diagnosis pre-operatively. Surgery remains the mainstay of treatment. Some reports suggest that carcinomas are more prone to develop in colonic/rectal duplications than in other GI tract duplications.

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ABSTRACT Acquired Hemophagocytic Lymphohistiocytosis is a rare and deadly syndrome resulting from an overactive immune system, with uncontrolled activation of macrophages and lymphocytes, hypercytokinemia, and systemic inflammatory response. A 75-year-old male presented with typical anginal pain and was diagnosed with the acute coronary syndrome, which required a percutaneous transluminal coronary angioplasty. Instead of resolving the symptoms, the patient began to exhibit pyrexia and worsening altered sensorium with progressing renal failure, anemia, thrombocytopenia and respiratory failure. This constellation of symptoms caused the patient to require mechanical ventilation and hemodialysis. Upon laboratory analysis, hyperferritinemia provided an indication to the diagnosis of acquired hemophagocytic lymphohistiocytosis. After the initiation of dexamethasone, the patient made a significant recovery and was discharged from the hospital.

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ABSTRACT Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare variant of the inflammatory myofibroblastic tumor. It has an aggressive clinical course and a high rate of recurrence. EIMS primarily affects children and young adults. Hereby, we report this entity in a 4-month-old infant who presented with an abdominal mass. Imaging studies revealed a large hypodense mesentery-based lesion involving the right half and mid-region of the abdomen. The mass with an attached segment of the small bowel was excised in toto. Grossly, a large encapsulated tumor was identified arising from the mesentery of the small bowel. The histological examination showed a tumor consisting of epithelioid to spindle cells loosely arranged in a myxoid background with numerous blood vessels and lymphoplasmacytic inflammatory infiltrate. On immunohistochemistry, the tumor cells showed positivity for ALK1 (nuclear), desmin, SMA, CD68, and focal positivity for CD30. A final diagnosis of EIMS of the small intestine was rendered. To the best of our knowledge, this case is the youngest reported case in literature.

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ABSTRACT Stratified mucin-producing intraepithelial lesion (SMILE) is an intraepithelial lesion with overlapping features of the high-grade squamous intraepithelial lesion (HSIL) and adenocarcinoma in situ (AIS). Currently, it is well described in the cervix. We present a case showing similar SMILE-like lesions in the polypectomy specimen from the anal canal along with invasive adenocarcinoma components. This lesion showed an immuno-profile characteristic of a SMILE lesion described in the cervix, such as p63 negativity, high ki67 index, and nuclear positivity for p16. It might be arising from the Human papillomavirus prone transitional region of the anal canal as described in the cervix. However, we could not assure this association and etiological link due to insufficient material in the formalin-fixed paraffin-embedded block. Notwithstanding, we strongly suggest that the HPV is the main driver for this SMILE-like lesion similar to what is described in the cervix. To our knowledge, this is the first case report of a SMILE lesion in the anal canal. Further studies will be required to elucidate the underlying pathogenetic mechanism of SMILE-like lesions described in the anal canal.

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ABSTRACT Necrotizing sarcoid granulomatosis (NSG) is a rare and under-recognized cause of granulomatous disease, described as a variant of typical nodular sarcoidosis. It can be asymptomatic when the patient has a single pulmonary nodule or may be accompanied by cough, fever, and dyspnea, or even symptoms due to the involvement of other organs such as the eyes, liver, and central nervous system. The histopathological analysis is essential for the differential diagnosis of other infectious and non-infectious causes of granuloma and to determine the appropriate treatment. NSG is characterized by the presence of a granuloma with extensive coagulative necrosis associated with the occurrence of vasculitis. We present the case of a patient diagnosed with NSG who had an unusual outcome with recurrent pulmonary thromboembolisms followed by hemodynamic instability and death.

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ABSTRACT Plasmacytoma is a neoplastic proliferation of monoclonal plasma cells, which can present clinically as solitary bone neoplasm, extramedullary plasmacytoma, and multiple myeloma. The biological behavior of these tumors is variable from periods of clinical latency to rapid growth and progression from localized forms to more disseminated multiple myeloma. We present the case of solitary plasmacytoma of the mandible with rare bilateral involvement in a 65-year-old female patient. This paper highlights the importance of understanding the maxillofacial manifestations of the disease by the dentist for early diagnosis and thus better prognosis.

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ABSTRACT Primary malignant melanoma of the oral cavity is a rare tumor in clinical practice. Extensive malignant melanomas are still very rare in the literature. Patients with malignant melanoma of oral cavity are often diagnosed at the advanced stage of the disease due to their painless and nonspecific radiological findings. Histopathology is the gold standard for the final diagnosis and staging of the tumor. Surgery followed by radiotherapy is the standard treatment offered to patients with malignant melanoma. Here we present a rare case of extensive malignant melanoma of oral cavity which was successfully managed by surgical excision followed by adjuvant radiotherapy.

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ABSTRACT Adult T- lymphocyte leukemia/ lymphoma (ATLL), described by Uchiyama et al. in 1977, is a distinct neoplasia of peripheral T-lymphocytes caused by human T-cell lymphotropic virus type 1 (HTLV-1). The authors describe the case of a 75-year-old female patient who presented with fever, chills, and altered mental status. The peripheral blood morphology showed large atypical lymphocytes with multilobed nuclei and flow cytometry consistent with ATLL. The authors discuss the pathophysiology, differential diagnosis, and subtypes of ATLL in addition to the diagnostic approach using flow cytometry when bone marrow biopsy is not available and modalities of treatment.

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ABSTRACT Anaplastic lymphoma kinase (ALK) positive, anaplastic large cell lymphoma involving the non-mammary implant is an extremely rare presentation. Irrespective of the type or site, the implant-associated primary ALCL is morphologically and immunophenotypically similar to ALK-negative ALCLs. Herein, we present the case of a 42-year-old male who developed a lytic lesion after an implant for a right femur fracture. The lytic lesion biopsy revealed anaplastic large cell lymphoma with ALK protein expression. Imaging findings showed the widespread dissemination of disease all over the body, entrapping the implant too. ALCL involving the bone implant is a very unusual and rare presentation that needs to be documented.

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ABSTRACT Malignant tumors of the penis are rare, most of them being squamous cell carcinomas (SCCs). We report the case of a 75-year-old man with a large penile mass submitted to partial penectomy. The specimen showed an exophytic mass involving the glans, coronal sulcus, and prepuce. Microscopic examination showed a carcinoma with two distinct areas: a mixed SCC and a sarcomatoid carcinoma. The SCC component had areas of verrucous carcinoma and areas of classical invasive SCC. The tumor cells expressed p63 with the absence of p16 expression. Vimentin and p53 were positive in the sarcomatous component. The morphology and immunohistochemistry were compatible with mixed SCC (verrucous hybrid-sarcomatoid carcinoma). Additionally, the tumor cells also expressed 3 different clones of PDL1 (22C3, SP263, and SP142). Two months later, the patient presented local recurrence with multiple lymph nodes and lung metastases, dying 7 weeks later. Mixed tumors represent diagnostic challenges. The correct identification of adverse prognostic factors can be the first step to implement the treatment with a higher probability of success.

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ABSTRACT Primary paraganglioma and small cell neuroendocrine carcinoma of the urinary bladder are rare tumors, comprising 0.05% of all bladder tumors and <1% of all malignant bladder tumors, respectively. These tumors can be the cause of a diagnostic dilemma or misdiagnosis on morphology. Paraganglioma is often mistaken for urothelial carcinoma and small cell carcinoma for poorly differentiated carcinoma or lymphoma. Herein, we report a case of primary paraganglioma and another of a small cell carcinoma of the urinary bladder and discuss their closest differential diagnoses. The diagnostic pitfalls should be kept in mind so that correct, timely diagnosis of these entities can be made due to implications in the management and prognosis.

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ABSTRACT Cyclin-dependent kinase 4/6 inhibitors represent a major advance in breast cancer treatment, emerging as the standard of care of the initial treatment of hormone receptor-positive and HER2-negative metastatic breast cancer. Their activity in this subset of patients leads to interest in their use in the adjuvant and neoadjuvant settings. This case report presents a real-life case of cyclin-dependent kinase 4/6 inhibitors use in a patient initially considered to have stage IV luminal HER2-negative breast cancer with liver metastasis. The discrepancy of treatment response between the breast tumor and liver node led to a repetition of the liver biopsy, which revealed metastasis of a neuroendocrine tumor of unknown primary. The breast tumor showed a partial response, and the initial therapeutic strategy was then redefined for curative intent. While cyclin-dependent kinase 4/6 inhibitors are not yet approved for clinical practice in the neo / adjuvant treatment of hormone receptor-positive breast cancer, this case report portrays a successful example of its application in a neoadjuvant setting.

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ABSTRACT Immunoglobulin IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition with a characteristic histopathological appearance that can affect almost any organ. The clinical features result from a focal or diffuse appearance of a tumor-like swelling of the affected organs, identified by physical and/or imaging examination. Herein, we report the case of a 38-year-old male complaining of a worsening chronic right lumbar pain associated with legs and scrotum edema. He also had itchy and erythematous cutaneous lesions on the abdominal wall over the last 8 months, and complained of a diffuse and mild to moderate abdominal discomfort. On examination, the liver was firmly enlarged and tender. His legs had 2+ symmetrical pitting edema extending from his feet to just above the knees. An abdominal computed tomography scan showed a large mass (10 x 8 x 4cm) involving the abdominal infrarenal aorta and the iliac arteries, and compressing the inferior vena cava, with dilated iliac veins, raising the possibility of lymphoproliferative disease. During the initial investigation, the laboratory workup revealed anemia, without other marked changes. A laparoscopic-guided biopsy of the peri-aortic mass was undertaken. The histological report associated with IgG4 immunoglobulin measurement rendered the diagnosis of IgG4-RD. The patient had a favorable outcome after the use of glucocorticoids with the abdominal mass remission.

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ABSTRACT A hepatic abscess caused by a swallowed foreign body is a rare and challenging diagnosis. Most patients have nonspecific symptoms, and more than 90% of patients do not remember having swallowed it, which occurred accidentally. In this setting, fish bones, chicken bones, and toothpicks are the most found foreign bodies. We reported the case of a 54-year-old male patient admitted with abdominal pain and intermittent fever. He was diagnosed with liver abscess and treated successfully with antibiotics and a laparoscopic procedure; a rosemary twig was found during the abscess drainage procedure. Furthermore, a literature review of 22 cases of laparoscopic treated liver abscesses associated with a foreign body was made.

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ABSTRACT Papillary thyroid carcinoma with desmoid-type fibromatosis (PTC-DTF) or nodular fasciitis-like stroma (PTC-NFS) is a rare morphological variant of PTC with a favorable prognosis. There is a paucity of molecular data regarding this entity. We present the case of a 20-year-old female who presented with a palpable mass over the anterior aspect of the neck for the past 3–4 months, which was diagnosed as PTC-NFS. Ultrasonogram of the neck revealed a bulky left lobe of thyroid that contained a well-defined heterogenous lesion measuring around 24 × 26 × 36 mm with involvement of the adjacent isthmus. She underwent a total thyroidectomy with central compartment lymph node dissection. Histological examination revealed a biphasic tumor with epithelial and stromal components resembling nodular fasciitis. Two dissected lymph nodes showed metastasis of the epithelial component only. On immunohistochemistry, BRAF mutant protein expression was evident in the epithelial component only, while β-catenin was negative in both the components. The histopathological diagnosis of papillary thyroid carcinoma with nodular fasciitis-like stroma was offered. Sanger sequencing revealed a BRAFV600E (c.1799T>A, Val600Glu) mutation. Post-operatively, no residual tumor was detected on ultrasound and radioiodine scans. The patient was doing well at follow-up of 9 months. PTC-NFS/DTF is a histological variant of PTC with a favorable prognosis. Our index case was associated with the BRAF mutation, which was restricted to the epithelial component. Thorough sampling of the excised specimen is essential in order not to miss the epithelial component, which, in most reported cases (including ours) appears to be small.

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ABSTRACT Only 14 cases of extraskeletal myxoid chondrosarcoma (EMC) of the vulva have been documented in the literature. We report a case of a 63-year-old woman with EMC of the vulva confirmed by both EWSR1 and NR4A3 fluorescence in situ hybridization, the latter of which is a more specific probe for this entity. The unusual location of this tumor of prominent myxoid morphology gave rise to a wide differential diagnosis, which necessitated thorough histologic evaluation and confirmatory ancillary testing in the form of immunohistochemistry and cytogenetic studies. This article aims to review extraskeletal myxoid chondrosarcoma of the vulva and various diagnostic clues to help differentiate it from its histologic mimics. This is the fifth case of vulvar EMC in the literature with confirmation of a NR4A3 gene rearrangement.

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ABSTRACT Background The bowel is the most common site of extragenital endometriosis, with involvement of the locoregional sigmoid colon and anterior rectum seen most often. The clinical presentation varies depending on how soon patients seek medical care, thus requiring changes in management strategies. Endometriosis can cause a life-threatening surgical emergency with progressive obliteration of the bowel lumen leading to obstruction and late complications including toxic megacolon and transmural necrosis. Case presentation We report the case of a 41-year-old woman presenting with an acute abdomen and complete large bowel obstruction complicated by sepsis and toxic megacolon. The patient underwent emergency total colectomy with ileostomy. Medical history was significant for chronic, vague, and episodic lower abdominal pain self-medicated with herbal tea and laxatives. Pathologic examination demonstrated colonic endometriosis within the bowel wall as the cause of obstruction, ischemia, and transmural necrosis. Conclusions Although a rare clinical entity, this case highlights two important points. First, it demonstrates the value of performing proper and complete clinical work up to rule out or in all possible causes of colonic obstruction, including intestinal endometriosis. Second, it suggests a potential benefit of a formalized multidisciplinary approach, including surgery, in the management of medically unresponsive endometriosis. In conclusion, this case shows that endometriosis can cause life-threatening colonic obstruction in women of childbearing age. Prompt early intervention is warranted, particularly when obstruction is only partial and ischemia has not supervened, to conceivably prevent the development of a toxic megacolon requiring colectomy and avoid late complications.

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ABSTRACT Erdheim-Chester disease is a rare non-Langerhans cell histiocytosis (LCH) that affects different body systems. It was recently recognized as a neoplastic disorder after identifying an activating mutation of the MAPK pathway. Neurological presentations of ECD are rare. We present a case of a 35-year-old male who presented to the emergency department with neck pain, headache and vomiting for 2 months; MRI showed multiple heterogeneous intracranial masses. Neurosurgery performed a suboccipital craniotomy, partially resected the cerebellar mass, and placed a parietal to frontal shunt catheter. Biopsy results from the cerebellar mass demonstrated cerebellar tissue involved by a diffuse proliferation of foamy histiocytes and spindle cells admixed with prominent lymphoplasmacytic infiltrate and positive for CD68, CD163, Factor XIIIa and Fascin. PET scan showed hypermetabolic uptake within the medullary portions of the diffuse abnormal lesions of the distal femurs, tibias, and fibulas, and cardiac MRI was nonsignificant. The patient was started on vemurafenib and continued to show improvement in a 3-month outpatient follow-up.

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ABSTRACT Intravascular large B-cell lymphoma (IVLBCL) is a rare form of diffuse large B-cell lymphoma, characterized by malignant B-cells primarily localized to the lumina of small- and medium-sized blood vessels without lymphadenopathy. Two patients initially presented with fever of unknown origin and persistent lactic acidosis without evidence of tissue hypoxia. Neither patient had an identifiable source of infection and both underwent peripheral blood smear demonstrating leukocytosis with a neutrophilic predominance and thrombocytopenia without evidence of hematologic malignancy. One had previously had a bone marrow biopsy which was unremarkable. Both patients’ condition deteriorated rapidly, progressing to multiorgan failure requiring pressors and mechanical ventilation, which ultimately resulted in cardiopulmonary arrest. At autopsy, each patient demonstrated malignant lymphocytoid cells, staining positive for CD20, localized to the lumina of small- and medium-sized vessels in multiple organs, including the lungs, liver, spleen, and kidneys, among others, allowing for the diagnosis of IVLBCL. IVLBCL is exceedingly rare, which in combination with significant variability in presentation, can make identification and diagnosis challenging. Diagnosis requires biopsy, therefore a high index of suspicion is needed to obtain an adequate tissue sample, whether pre- or postmortem. In the presented cases, both patients exhibited type B lactic acidosis with an unknown etiology that was ultimately determined at autopsy.

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ABSTRACT Background Acute tubulointerstitial nephritis (ATIN) is a very rare paraneoplastic manifestation in patients with multiple myeloma (MM). It is an uncommon pattern of renal disease in such patients. Case presentation We report a case of an 82-year-old male who was admitted with acute kidney injury. Renal biopsy showed typical findings of light chain-associated ATIN with scattered inflammatory cells in the interstitium and associated active tubulitis. No other common manifestations of MM were present at the time of presentation, including hypercalcemia, hyperuricemia, proteinuria, bone pain or lytic bone lesions. Subsequent immunoassays revealed significant serum lambda light chain burden and Bence Jones protein in urine. Immunofluorescence demonstrated linear tubular basement membranes with positive staining for lambda light chain (3+). Electron microscopy (EM) further showed interstitial edema and inflammation. All the aforementioned findings are consistent with ATIN and supported the diagnosis of MM. Conclusions In conclusion, light chain-associated ATIN should be considered in the differential diagnosis of acute interstitial nephritis. Henceforth, serum free light chains as well as serum and urine protein electrophoresis should be included in the workup of such patients.

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ABSTRACT Metaplastic breast carcinoma is a rare subtype of invasive breast carcinoma. Metaplastic carcinoma with osseous differentiation is exceptionally uncommon. Because of the heterogenous microscopy of the lesion, various clinical and radiological features are observed, leading to diagnostic difficulty. Herein, we present a case of a 43-year-old female with a recurrent breast lump, who was clinically diagnosed as a phyllodes tumor. However, histopathological examination revealed metaplastic carcinoma with extensive osseous differentiation.

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ABSTRACT Currently, there is growing evidence in the literature warning of misdiagnosis involving amyloidosis and chronic inflammatory demyelinating polyneuropathy (CIDP). Although inducing clinical manifestations outside the peripheral nervous system, light chain and transthyretin amyloidosis may initially present with peripheral neuropathy, which can be indistinguishable from CIDP, leading to a delay in the correct diagnosis. Besides, the precise identification of the amyloid subtype is often challenging. This case report exemplifies clinical and laboratory pitfalls in diagnosing amyloidosis and subtyping amyloid, exposing the patient to potentially harmful procedures.

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Abstract Mucinous adenocarcinoma of the appendix is a rare neoplasm with a low propensity for lymph node metastasis. The present case refers to an appendicular mucinous adenocarcinoma with inguinal lymph node metastasis. A 71-year-old woman underwent an appendectomy due to a clinical presentation of acute appendicitis. However, the histological examination of the surgical specimen revealed a mucinous adenocarcinoma of the appendix. After staging, the patient underwent a right hemicolectomy and was proposed for adjuvant chemotherapy. At the 3rd year of follow-up, inguinal lymphadenopathy was diagnosed, which biopsy confirmed inguinal node metastases from primary colorectal cancer, with areas of extracellular mucin. Restaging revealed liver and peritoneal metastasis, and the patient was proposed for palliative chemotherapy. Appendicular neoplasms, due to their rarity, represent a diagnostic and therapeutic challenge. This clinical case depicts an unusual metastasis pathway for an unusual neoplasm.

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ABSTRACT Background Predominantly intraosseous meningiomas are rare entities that include true primary intraosseous meningiomas (PIM), as well as meningiomas that may show extensive bone involvement, such as en plaque meningiomas. Different hypotheses have been proposed to decipher the origin of PIMs, such as ectopic arachnoid cap cell entrapment during birth or after trauma. Surgical resection is the treatment of choice of such lesions. Case presentation We present a case of a 65-year-old man with an enlarging mass in the parieto-occipital region that grew slowly and progressively over 13 years, following head trauma during a motor vehicle accident. One year prior to presentation, he started experiencing daily holocranial headaches and blurry vision. CT and MRI studies revealed a permeative midline calvarial lesion measuring 14 cm in greatest dimension with extensive periosteal reaction, extension into the subcutaneous soft tissues, subjacent dural thickening and intracranial extension with invasion of the superior sagittal sinus. The favored pre-operative clinical diagnosis was osteosarcoma. The abnormal calvarium was excised and histopathological examination confirmed the diagnosis of a predominantly intraosseous calvarial meningioma, WHO grade I. Conclusions The present case highlights the importance of histopathologic diagnosis in guiding therapeutic decisions and reiterates the necessity of considering PIM or meningiomas with extensive intraosseous component in the differential diagnosis of calvarial masses, even when imaging suggests a neoplasm with aggressive behavior, such as osteosarcoma.

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ABSTRACT Mantle cell lymphoma is characterized by t(11;14) with CCND1-IGH fusion and manifests with a spectrum of disease ranging from relatively indolent to aggressive. Here, we present a case of pleomorphic mantle cell lymphoma with three fusion signals that presented with lethal atraumatic splenic rupture. We discuss on the implications of variant CCND1 signal patterns as well as the epidemiology and pathophysiology of atraumatic splenic rupture.

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ABSTRACT Karyomegalic interstitial nephritis (KIN), first described in 1974, is a rare form of chronic tubulointerstitial nephritis. It is defined by the presence of markedly enlarged, hyperchromatic nuclei with prominent nucleoli, mainly involving tubular epithelial cells of the kidney, accompanied by marked interstitial fibrosis. The disease presents as asymptomatic proteinuria, gradually progresses to chronic kidney disease and eventually leads to end-stage renal disease by 30-40 years. The etiology of the disease remains unclear; however, genetic risk factors and possible association with HLA (B27/35) is proposed by some. It has also been linked to FAN1 (FANCD2/FANC1- associated nuclease 1) mutation. Case Report We present two cases of KIN with associated focal segmental glomerulosclerosis. Both patients presented with nephrotic range proteinuria. The biopsies demonstrated marked enlargement of tubular nuclei (3-5x larger than the uninvolved tubular nuclei, a metric used by some authors in previous studies) in some tubules, meeting the diagnostic criteria of KIN.. Interestingly, case one had a prior biopsy that showed minimal change disease. In the biopsies done at our institution, H&E sections showed patchy tubular attenuation with readily recognizable tubular cell mitotic figures, indicating concurrent acute tubular injury. Electron microscopy showed diffuse podocyte foot process effacement, along with microvillous transformation, podocyte hypertrophy, and cytoplasmic vacuoles, suggesting podocyte injury. This cytoplasmic vacuolization was also observed in the tubular epithelial cells. In both cases, the injury factor appeared to target both podocytes and tubular cells.

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ABSTRACT Langerhans cells, found in the supra-basal region of the mucous membranes in the epidermis of the skin, in lymph nodes and thymus, function as antigen-presenting cells within the histiocyte system. Tumors derived from Langerhans cells (LC) can be divided according to the degree of cytological atypia and clinical behavior into Langerhans cell histiocytosis (LCH) and Langerhans cell sarcoma (LCS). LCS is rare, and the nodal presentation is even rarer with challenging histological characteristics. LCS has a dismal overcome despite intensive chemotherapy. Herein, we report a case of a 29-year-old male who presented with generalized lymphadenopathy initially considered as a lymphoma. An outright definitive diagnosis could not be attained in the initial histomorphological and immunohistochemical evaluation, fraught with differential diagnoses. The key to decoding the precise neoplasm was a combination of the cytopathologic features, review of the histomorphology, and extensive immunohistochemical assessment in conjunction with the clinical and positron emission tomography (PET) scan findings. The best diagnosis proffered was a Langerhans cell histiocytosis progressing to Langerhans cell sarcoma. This case highlights the grey zone areas in LC neoplasms, the diagnostic conundrums encountered, the indispensable role of meticulous pathological analysis, and the importance of ancillary studies in hammering out the final diagnosis.

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ABSTRACT Forensic odontology is a specialty of dental sciences that deals with dental evidence in the interest of the justice system. The science of autopsy has been developing from the ancient times even before the popularization of general medicine. The objective of a medico-legal autopsy is to identify significant clues for an ongoing forensic investigation. However, in certain circumstances, it is difficult to conduct an oral examination owing to the anatomic location of the oral cavity. The onset of rigor mortis after death poses further complications. Thus, skillful and sequential dissections of the oral and para-oral structures are required to expose the dentition. Dental autopsy includes incisions and resection of the jaw for the detailed examination of the oral cavity. The procedure involves various modes of examination, including visual and radiographic, which help in human identification in forensic investigation. The present paper provides an overview of the various methods of dental autopsy.

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ABSTRACT Sarcina ventriculi is a gram-positive bacterium, able to survive in extreme low pH environment. It’s first description dates from 1842, by John Goodsir. Since then, just a few cases have been reported. In veterinary medicine, especially in ruminants, it causes bloating, vomiting, gastric perforation and death of the animal. It is commonly associated with delayed gastric emptying or obstruction to gastric outlet, although it’s pathogenicity in humans is not fully understood. We report two cases with identification of the bacteria in gastric specimens stained with hematoxylin-eosin staining, in different clinical settings. The first patient is a young female patient, presenting cardiac arrest and death after gastric perforation and the second patient an adult male presenting with gastric adenocarcinoma, treated with partial gastrectomy followed by adjuvant chemoradiation. In our literature review, we identified forty-five cases reporting Sarcina ventriculi appearance, with a sudden increase since 2010.

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ABSTRACT Introduction Squamous carcinoma is the commonest malignancy of the head and neck region. It is associated with high morbidity and mortality. Epidermal growth factor receptor (EGFR) regulates downstream signaling pathways through its tyrosine kinase (TK) domains that play a role in cell proliferation and survival. EGFR mutations have been found to occur between exons 18 to 21 on chromosome 7. Limited studies are available on EGFR-TK mutations in the head and neck squamous cell carcinoma (HNSCC) globally. This study explores EGFR mutations in 30 HNSCC cases presenting to a tertiary care hospital over a period of two years. Material and Methods Fresh tumor tissue was collected from the resection specimens of cases of primary HNSCC. Cases with pre-operative therapy were not included. Parameters in the form of patients’ age, gender, smoking/tobacco intake, site of the lesion were recorded. Tumor parameters after histopathological examination were recorded in the form of TNM stage, tumor grade. DNA was extracted from fresh tissue of all the cases. EGFR Mutation Analysis Kit assay was used to detect mutations of the EGFR gene. PCR was run and results were analyzed. Results EGFR Mutations were found in 6.7%of the patients. There was no significant association of the EGFR Mutation with the studied parameters. Conclusion EGFR mutations are present in a subset of patients of HNSCC. Patients having these mutations may benefit from targeted therapy with tyrosine kinase inhibitors.

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ABSTRACT Introduction Cervical erector spinae plane block (ESPB) provides postoperative pain relief when administered at the level of first thoracic costotransverse junction (CTJ) for surgeries on the proximal shoulder and cervical spine. We propose to describe the spread of 20 ml radiocontrast - dye solution administered at this level from caudad to cephalad direction in a fresh frozen cadaveric model through imaging and cross-sections. Methods An observational study with four thoracic to cervical ESP blocks at the level of first thoracic CTJ level on two fresh cadavers (total 4 specimens) was conducted using 20 ml of radiocontrast- methylene blue combination (10 ml through the needle and 10 ml through the catheter). Both cadavers were subjected to computed tomography (CT) scan. An anatomist and radiologist, respectively, analyzed cross-sections of cadavers and CT contrasted images. Results The spread was assessed in axial, sagittal, and coronal at the levels of C4, C5, C6, C7and T1. The medial limit was articular processes in both cadavers. The lateral limits were the outer border of the middle scalene muscle in cadaver 1 and posterior to the sternocleidomastoid muscle in cadaver 2. Contrast spread was visualized on the superior and anterior aspect of anterior scalene muscle in cadaver 2. An epidural spread was observed at the level of C5-6 and C6-7 in axial and coronal planes in cadaver 1. Conclusions The cervical ESPB administered at the first thoracic CTJ with injections directed cephalad has a consistent action on the dorsal spinal nerves of thoracic and cervical area, and spreads in the paravertebral space dorsal to the ventral cervical roots.

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ABSTRACT Gaucher disease (GD) is an autosomal recessive lysosomal disorder caused by a disturbance in the metabolism of glucocerebroside in the macrophages. Most of its manifestations – hepatosplenomegaly, anemia, thrombocytopenia, and bone pain – are amenable to a macrophage-target therapy such as enzyme replacement. However, there is increasing evidence that abnormalities of the liver persist despite the specific GD treatment. In this work, we adapted histomorphometry techniques to the study of hepatocytes in GD using liver tissue of treated patients, developing the first morphometrical method for canalicular quantification in immunohistochemistry-stained liver biopsies, and exploring histomorphometric characteristics of GD. This is the first histomorphometric technique developed for canalicular analysis on histological liver biopsy samples.

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ABSTRACT Amniotic fluid embolism is a rare, often fatal complication of labor and delivery. The classic presentation is the sudden onset of a triad of clinical manifestations: hypoxia, hypotension and coagulopathy. Understanding of the syndrome as an immunologically mediated, complicated and often catastrophic maternal response to fetal or placental antigens is coming into focus. New treatments such as extracorporeal membrane oxygenation (ECMO) and better use of old treatments such as transfusion offer hope, but the condition is often rapidly fatal, so saving the maternal and fetal lives depends on rapid recognition of the syndrome. This series of three cases illustrates the clinical features enabling the rapid recognition needed for successful treatment of amniotic fluid embolism syndrome.

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ABSTRACT Paraquat is a potent herbicide widely used in the Indian agriculture industry. Human fatality due to paraquat poisoning is not uncommon in this country. The primary effect of paraquat is on the lungs, and the resultant pulmonary damage leads to the patient's demise. There is a high mortality rate in paraquat poisoning as the treatment is usually supportive with no known antidote. There are limited human studies that have observed the histopathological changes in lungs in paraquat poisoning. The authors have discussed the time-related histopathological changes in lungs in paraquat poisoning on autopsy subjects. The role of anticoagulants and fibrinolytic agents in the treatment of this poisoning has also been discussed.

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ABSTRACT The modern era has brought an appreciation that renal cell carcinoma (RCC) includes diverse subtypes derived from the various parts of the nephron, each with its distinctive genetic basis and tumor biology. Carcinoma of the collecting ducts of Bellini (CDC) is a rare subtype of RCC, with a predictably poor prognosis. This rare subtype represents less than 1% of all kidney carcinomas. It derives from presumably numerous chromosomal losses. It is of chief importance to differentiate CDC from other types of renal cell cancer. Typically, it is characterized by a firm, centrally located tumor with infiltrative borders. Regarding the histopathologic characteristics, we can find complex, highly infiltrative cords with inflamed (desmoplastic) stroma, with high-grade nuclei and mitoses. Most reported cases of CDC had been high grade, advanced stage, and unresponsive to conventional therapies. This rare form of disease highlights the importance of multidisciplinary teams in the management of cancer patients.

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ABSTRACT Actinic prurigo (AP) is a type of photodermatosis that primarily affects the Latin American mestizo population. Histologically, AP cheilitis exhibits acanthosis with spongiosis and vacuolation of the basal cell layer overlying a dense lymphocytic inflammatory infiltrate that forms well-defined lymphoid follicles. Toluidine blue is a thiazide, acidophilic, and metachromatic dye used in vivo to selectively stain the acidic components of tissues such as sulfates, carboxylates, and phosphate radicals that are incorporated into DNA and RNA. It is necessary to develop a method that allows detecting, on clinical grounds the area of the lesion in which it is more feasible to find such structures. Thus to increase the sensitivity of the biopsy, in AP cheilitis to accurately identify where the lymphoid follicles reside, based on the higher concentration of DNA in such structures and thus confirm the diagnosis. In this study, staining was positive in 85% of patients with AP cheilitis, in 14 of whom 82% lymphoid follicles were observed by histopathology. One of the pathologist’s problems in establishing the diagnosis of AP is that the main histopathological characteristics are not always identified in the submitted samples because it is not easy to clinically identify the most representative site of the lesion selected for performing a biopsy. Based on our results, we propose using toluidine blue as an auxiliary method to choose a tissue sample to facilitate the diagnosis and allow clinicians to make clinical correlations between the histopathological and therapeutic findings.

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ABSTRACT Microscopic findings in key tissues are often critical to determine the cause of death in medical autopsies. The overall quality of histologic sections depends on numerous pre-analytic factors, among which are tissue section size and thickness. We designed a prospective quality improvement study to determine whether a simple intervention of formalin pre-fixation of myocardium, liver, and kidney tissues could improve the ease of cutting and quality of autopsy histologic sections as assessed by histotechnicians and pathologists. Of 46 autopsies included in the study, 21 were randomly assigned to formalin pre-fixation, and 25 underwent routine sectioning without formalin pre-fixation. A significant improvement in overall quality score by histotechnicians was detected in the sections from pre-fixed autopsy tissues compared to the control group (p=0.0327). There was no significant difference in quality score between the two groups as assessed by pathologists. Our autopsy quality improvement study demonstrates that a simple, low-cost intervention of formalin pre-fixation of fresh autopsy tissues for 90 minutes could significantly improve the overall quality of sections submitted for histologic processing.

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ABSTRACT Viral or bacterial co-infections with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) have been reported in the literature. However, the knowledge on Aspergillus co-infection among patients with coronavirus disease 2019 (COVID-19) is limited. COVID‐19‐associated pulmonary aspergillosis (CAPA) has been seen in critically ill COVID-19 patients with acute respiratory distress syndrome (ARDS), which has raised concerns about the worsening disease course of COVID-19 and increasing mortality. We describe a clinical case of CAPA infection and acute respiratory distress syndrome (ARDS) with a deathly outcome in a previously well, non-immunocompromised pregnant woman with intrauterine death of the fetus. Hence, we suggest that clinicians and pathologists keep alerting the possible occurrence of pulmonary aspergillosis in severe/critical COVID-19 patients, and aggressive investigations should be done to rule out the possibility of CAPA so that early treatment can be administrated.

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ABSTRACT As the COVID-19 pandemic spread to the United States, it was followed by unprecedented changes. These changes did not spare undergraduate and graduate medical students. Specifically, medical students applying for residency programs were faced with a novel challenge. In March 2020, as the pandemic became increasingly severe, the Association of American Medical Colleges (AAMC) recommended pulling medical students from in-person clinical rotations. By May 2020, the AAMC recommended that all residency interviews be conducted online for the 2020-2021 residency application cycle. These unprecedented modifications to the interview season required programs to quickly adapt and find ways to utilize online tools to convey what their program offered to applicants. In this paper, we will outline the adaptations, tools, and resources that residencies and applicants have used to navigate through the 2020/2021 interview cycle.