Extranodal NK/T-cell lymphoma, nasal type with extensive cardiopulmonary involvement

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.

Keywords
Lymphoma, Extranodal NK-T-Cell; Epstein-Barr Virus Infections; Lymphoma; Lymphoma, Non-Hodgkin

Authorship

Mario L. Marques-Piubelli

Universidade de São Paulo, Faculdade de Medicina, Departamento de Patologia. São Paulo, SP, BrasilUniversidade de São PauloBrasilSão Paulo, SP, BrasilUniversidade de São Paulo, Faculdade de Medicina, Departamento de Patologia. São Paulo, SP, Brasil

The University of Texas MD Anderson Cancer Center, Department of Translational Molecular Pathology. Houston, Texas, USAThe University of Texas MD Anderson Cancer CenterUSAHouston, Texas, USAThe University of Texas MD Anderson Cancer Center, Department of Translational Molecular Pathology. Houston, Texas, USA

http://orcid.org/0000-0002-6324-2096

Gabriel Teixeira Montezuma Sales

Universidade de São Paulo, Faculdade de Medicina, Departamento de Nefrologia. São Paulo, SP, BrasilUniversidade de São PauloBrasilSão Paulo, SP, BrasilUniversidade de São Paulo, Faculdade de Medicina, Departamento de Nefrologia. São Paulo, SP, Brasil

http://orcid.org/0000-0002-7618-2782

Letícia Campos Clemente

http://orcid.org/0000-0002-7917-8543

Lidiane Inês Rosa

Universidade de São Paulo, Faculdade de Medicina, Departamento de Hematologia e Hemoterapia. São Paulo, SP, BrasilUniversidade de São PauloBrasilSão Paulo, SP, BrasilUniversidade de São Paulo, Faculdade de Medicina, Departamento de Hematologia e Hemoterapia. São Paulo, SP, Brasil

Mariana Lorenzi Savioli

Ricardo Pires Alvim

Universidade de São Paulo, Faculdade de Medicina, Departamento de Neurologia. São Paulo, SP, Brasil Universidade de São PauloBrasilSão Paulo, SP, BrasilUniversidade de São Paulo, Faculdade de Medicina, Departamento de Neurologia. São Paulo, SP, Brasil

http://orcid.org/0000-0002-8771-8968

Raquel Megale Moreira

http://orcid.org/0000-0003-4345-8911

Fernando Pereira Frassetto

http://orcid.org/0000-0003-4720-6834

Ellen Caroline Toledo do Nascimento

http://orcid.org/0000-0002-5711-1997

Sheila Aparecida Coelho Siqueira

http://orcid.org/0000-0001-8756-7672

Authors’ contributions: Marques-Piubelli ML, Clemente LC, Frassetto FP, Nascimento ECT, and Siqueira SAC were responsible for the pathologic diagnosis and performed the autopsy. Sales GTM, Moreira, RM Savioli ML, and Alvim RP were responsible for the clinical care. All authors have equally contributed to the elaboration of the manuscript.

Conflict of interest: The authors have no conflict of interest to declare.

CorrespondenceMario L. Marques-Piubelli The University of Texas MD Anderson Cancer Center, Department of Translational Molecular Pathology 2130 W Holcombe Blvd, Houston, TX 77030 Telephone: +1 (713) 745-9200 (36885)MLPiubelli@mdanderson.org; marquemario@gmail.com

SCIMAGO INSTITUTIONS RANKINGS

Figures

Figures (3)

Thumbnail

Figure 1
Macroscopic appearance of lung and heart. A – Gross view of the lung with multiple greyish nodular lesions (white arrows) in a background of a hemorrhagic parenchyma; B – Gross view of the heart shows very delicate irregular areas of pale and greyish color in the left ventricle (black arrow).

Thumbnail

Figure 2
Histological appearance of a lung nodule: A - intense and diffuse hemorrhage, necrosis of the alveolar spaces, and diffuse lymphoid infiltrate (H&E, 40x); B - intense and atypical lymphoid infiltrate with angiocentric distribution (H&E, 100x); C - atypical lymphoid infiltrate composed by medium sized cells, irregular nuclei contour, condensed chromatin, and scant cytoplasm (H&E, 400x); D - immunohistochemistry for CD20 shows negative stain for lymphoma cells and positive reactive B-cells in the background (50x); E - immunohistochemistry for CD3 stain shows partial and strong positivity of the lymphoma cells (100x); F - immunohistochemistry for CD30 stain shows weak and partial positivity of the lymphoma cells (100x); G - immunohistochemistry for CD15 shows negativity of the lymphoma cells (100x); H - immunohistochemistry stain for CD56 shows diffuse and moderate stain of the lymphoma cells (100x); I - immunohistochemistry for ALK-1 protein shows negative stain of the lymphoma cells (100x); J - immunohistochemistry for CD68 shows negativity of the lymphoma cells and positivity of macrophages in the background (400x); K - in situ hybridization to EBV-encoded RNA (EBER) shows positivity in the lymphoma cells disposed in an angiocentric pattern (100x); L - Immunohistochemistry for Ki-67 shows high proliferation index (90%) in the lymphoma cells (100x).

Thumbnail

Figure 3
Photomicrographs of the heart: A – Hematoxylin & Eosin (H&E) section shows cardiomyocytes with intense and interstitial infiltrate composed by atypical lymphoid cells of small to medium size (100x); B – in situ hybridization to EBV-encoded RNA (EBER) showing diffuse positivity of the atypical lymphoid cells (200x).

Figure 1 Macroscopic appearance of lung and heart. A – Gross view of the lung with multiple greyish nodular lesions (white arrows) in a background of a hemorrhagic parenchyma; B – Gross view of the heart shows very delicate irregular areas of pale and greyish color in the left ventricle (black arrow).

Figure 2 Histological appearance of a lung nodule: A - intense and diffuse hemorrhage, necrosis of the alveolar spaces, and diffuse lymphoid infiltrate (H&E, 40x); B - intense and atypical lymphoid infiltrate with angiocentric distribution (H&E, 100x); C - atypical lymphoid infiltrate composed by medium sized cells, irregular nuclei contour, condensed chromatin, and scant cytoplasm (H&E, 400x); D - immunohistochemistry for CD20 shows negative stain for lymphoma cells and positive reactive B-cells in the background (50x); E - immunohistochemistry for CD3 stain shows partial and strong positivity of the lymphoma cells (100x); F - immunohistochemistry for CD30 stain shows weak and partial positivity of the lymphoma cells (100x); G - immunohistochemistry for CD15 shows negativity of the lymphoma cells (100x); H - immunohistochemistry stain for CD56 shows diffuse and moderate stain of the lymphoma cells (100x); I - immunohistochemistry for ALK-1 protein shows negative stain of the lymphoma cells (100x); J - immunohistochemistry for CD68 shows negativity of the lymphoma cells and positivity of macrophages in the background (400x); K - in situ hybridization to EBV-encoded RNA (EBER) shows positivity in the lymphoma cells disposed in an angiocentric pattern (100x); L - Immunohistochemistry for Ki-67 shows high proliferation index (90%) in the lymphoma cells (100x).

Figure 3 Photomicrographs of the heart: A – Hematoxylin & Eosin (H&E) section shows cardiomyocytes with intense and interstitial infiltrate composed by atypical lymphoid cells of small to medium size (100x); B – in situ hybridization to EBV-encoded RNA (EBER) showing diffuse positivity of the atypical lymphoid cells (200x).

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Hospital Universitário da Universidade de São Paulo Hospital Universitário da Universidade de São Paulo, Av. Prof. Lineu Prestes, 2565 - Cidade Universitária, 05508-000 - São Paulo - SP - Brasil, (16) 3307-2068, (16) 3307-2068 - São Paulo - SP - Brazil
E-mail: autopsy.hu@gmail.com

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Extranodal NK/T-cell lymphoma, nasal type with extensive cardiopulmonary involvement

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[1] Authors’ contributions: Marques-Piubelli ML, Clemente LC, Frassetto FP, Nascimento ECT, and Siqueira SAC were responsible for the pathologic diagnosis and performed the autopsy. Sales GTM, Moreira, RM Savioli ML, and Alvim RP were responsible for the clinical care. All authors have equally contributed to the elaboration of the manuscript.