Adult diffuse hepatic hemangiomatosis

Bhardwaj, Neha; Parkhi, Mayur; Kumar, Manish; Kaman, Lileswar; Mitra, Suvradeep

doi:10.4322/acr.2021.401

Neha Bhardwaj

Post Graduate Institute and Medical Education and Research (PGIMER), Department of Pathology, Chandigarh, India

http://orcid.org/0000-0002-2476-641X

Mayur Parkhi

Post Graduate Institute and Medical Education and Research (PGIMER), Department of Histopathology, Chandigarh, India

http://orcid.org/0000-0002-6040-6737

Manish Kumar

Post Graduate Institute and Medical Education and Research (PGIMER), Department of General Surgery, Chandigarh, India

http://orcid.org/0000-0003-1829-1208

Lileswar Kaman

Post Graduate Institute and Medical Education and Research (PGIMER), Department of General Surgery, Chandigarh, India

http://orcid.org/0000-0003-1837-2614

Suvradeep Mitra

Post Graduate Institute and Medical Education and Research (PGIMER), Department of Histopathology, Chandigarh, India

http://orcid.org/0000-0002-5520-8306

Authors’ contributions: Neha Bhardwaj and Mayur Parkhi collected the data, performed the literature search, and prepared the manuscript. Manish Kumar provided the clinical data and follow-up and helped in the preparation the manuscript. Lileswar Kaman was involved in the clinical and radiological diagnoses, patient management, and critically reviewed the manuscript. Mayur Parkhi and Suvradeep Mitra were involved in the histopathological diagnosis. Further, Suvradeep Mitra was involved in the literature search, conceptualization, and critical review of the paper. Neha Bhardwaj and Mayur Parkhi will share the first authorship of this manuscript.

Conflict of interest: None.

Correspondence Suvradeep Mitra Post Graduate Institute and Medical Education and Research, Department of Histopathology Chandigarh, PIN – 160012, India Phone: +91 985 566 3999 sugreebm@gmail.com

Ref	Age/Sex	Clinical features	GCH	Gross	Histopathology	Treatment	Outcome
7	56/F	Generalized bone pain, abdominal pain and abdominal swelling due to hepatic mass	NA	NA	Liver and bone with numerous capillary angiomas consistent with hemangiomatosis. Cystic spaces lined by thin, homogeneously stained endothelial cells. Areas of tumor with numerous intraluminal red blood cells.	Prednisone	Died after 2 weeks
8	30/F	Hepatosplenomegaly, anemia and thrombocytopenia	NA	Liver weighed 6,790 g, was reddish brown, with numerous dark soft areas and whitish discoloration.	Hepatic parenchyma replaced by hemorrhagic cystic tumors. Similar vascular lesion was seen in the spleen, bone marrow, intestine, and peripancreatic lymph nodes.	Radiotherapy Splenectomy	Died 12 months after first presentation due to consumptive coagulopathy
9	22/F	Right upper abdominal pain	NA	Both hepatic lobes with innumerable irregular, sometimes confluent reddish nodules of 0.3-2 cm	Numerous irregularly formed slit-like blood vessels lined by endothelium and slight focal portal fibrosis close to the central vein. The portal triads showed capillary sprouting. The liver capsule was retracted by bands containing capillaries and collagenous fibers.	Stoppage of drug	Regression of lesions
3	35/F	Abdominal pain, weight loss, night sweats and fever	NA	Multiple purple nodules < 2 cm found throughout the tumor; mostly beneath the Glisson’s capsule	Cavernous and diffuse, hemangiomatosis and hemorrhage within the tumor. Innumerable, confluent vascular channels lined by flat endothelial cells linked the hemangiomas.	Left hepatectomy	Recurrence and growth into the right hepatic lobe after two years of surgery. Still progressing at 6 years of follow up.
10	50/F	Tenderness in right upper abdominal quadrant	Yes	Size: 17×14×9 cm, multiple blood-filled honey comb areas of 3 mm to 3 cm	Cavernous hemangioma surrounded by hepatic parenchyma. Vascular channels lined by flattened endothelial cells. No cellular atypia	Right hepatectomy	No mass detected on ultrasonography 9 months post-surgery
11	33/F	Abdominal distension, edema and hepatomegaly	NA	NA	Prominent cavernous vascular proliferation and fibrosis without angiosarcomatous components	None	Patient expired due to liver failure 10 days after admission
12	33/F	Abdominal distension and shortness of breath	NA	NA	Variably dilated non-anastomotic vascular spaces lined by flat endothelial cells (CD 34 +).	NA	Died of liver failure after 12 days while waiting for liver transplant
13	78/M	Abdominal pain and distension	Yes	NA	Cavernous hemangioma with irregularly dilated non-anastamotic vascular spaces lined by flat endothelial cells alternating with normal hepatic parenchyma	None	Improvement of discomfort and quality of life after 9 months follow up.
14	35/F	Epigastric pain and abdominal fullness	Yes	The resected tumor was 20× 14× 8.5 cm in size and 910 g in weight	Hemangiomatous lesions were scattered around the Glisson’s capsule	Right hepatectomy	Discharged on POD9 without identifiable lesions
15	68/M	Asymptomatic	NA	NA	Endothelial-lined sinusoidal proliferation with erythrocyte content, consistent with hepatic hemangiomatosis	None	Stable at two years of follow up
16	59/M	Asymptomatic	NA	NA	H&E showed hemangiomas with (CD34+, CD31+, anti-desmin negative) no cellular atypia.	NA	Died due to hepatic failure
17	50/F	Abdominal pain, hepatomegaly and dyspnea	Yes	Well-defined sponge-like reddish brown mass. Remaining parenchyma with similar small lesion.	Main mass with variable-sized vascular spaces, lined by endothelial cells. Multiple scattered small hemangiomas also present.	Liver transplant	Stable for 1.5 years after surgery
18	83/F	Abdominal distension and hepatomegaly	NA	Multiple characteristic dark red nodules on liver surface (laparoscopic finding)	Irregularly dilated vascular spaces, mostly close to the portal tract, lined by endothelial cells (CD34+ and CD31+) without cellular atypia	Bevacizumab	Died 12 months after diagnosis due to multiple organ failure (KMS, hemolytic anemia, heart failure, DIC)
19	62/M	Asymptomatic	NA	NA	Focal areas of sinusoidal dilatations lined by flattened endothelial cells.	NA	NA
20	29/F	H/O Endometriosis and received OCPs	Yes	Lesion in segment IVa with surrounding changes and a well-defined lesion containing 6.5-cm blood clot in segment IVb	Disseminated aggregate of blood vessels lined by endothelium without atypia.	Stoppage of drug and left liver lobectomy	No recurrent liver lesion after 1 year of surgery.
21	40/F	Abdominal pain and distension	Yes	NA	DHH with a giant hemangioma. No mitosis. STAT6, WT1, Desmin, p53, D2-40 were negative.	Chemotherapy, trans-arterial embolization and Liver transplant	Good condition after 6 months of follow-up.
4	63/M	Abdominal bloating and constipation	Yes	NA	NA	None	NA
6	62/M	Backache, Hepatomegaly	NA	NA	Lesion filled with red blood cells, lined by flat endothelial cells (CD34+) without atypia.	None	Good condition after 6 months of follow-up.
22	56/F	Chronic abdominal discomfort	Yes	NA	NA	NA	NA
Our case	37/F	Right upper quadrant fullness and shortness of breath	Yes	Well encapsulated, dark red to tan-brown colored masses and few perilesional cherry-colored honeycomb foci.	Microscopic insinuation of the vascular lesion margins into the normal hepatic sinusoids. Lesions with variably-sized to large, thin-walled, non-anastomosing vascular spaces, and irregularly attenuated thick muscle walls. Vascular spaces lined by single layer of endothelial cells. Fibrin thrombi were noted within these vascular spaces. Stroma with fibrosis, myxoid degeneration, hyalinization, and calcification.	Surgical enucleation	Good condition after 6 months of follow-up.

[1] Authors’ contributions: Neha Bhardwaj and Mayur Parkhi collected the data, performed the literature search, and prepared the manuscript. Manish Kumar provided the clinical data and follow-up and helped in the preparation the manuscript. Lileswar Kaman was involved in the clinical and radiological diagnoses, patient management, and critically reviewed the manuscript. Mayur Parkhi and Suvradeep Mitra were involved in the histopathological diagnosis. Further, Suvradeep Mitra was involved in the literature search, conceptualization, and critical review of the paper. Neha Bhardwaj and Mayur Parkhi will share the first authorship of this manuscript.

Brasil

Brasil

Adult diffuse hepatic hemangiomatosis

Abstract