Autopsy and Case Reports
Autopsy and Case Reports
Autopsy Case Report

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

Mario L. Marques-Piubelli; Gabriel Teixeira Montezuma Sales; Letícia Campos Clemente; Lidiane Inês Rosa; Mariana Lorenzi Savioli; Ricardo Pires Alvim; Raquel Megale Moreira; Fernando Pereira Frassetto; Ellen Caroline Toledo do Nascimento; Sheila Aparecida Coelho Siqueira

Downloads: 3
Views: 1107


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.


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


1 Haverkos BM, Pan Z, Gru AA, et al. Extranodal NK/T Cell Lymphoma, Nasal Type (ENKTL-NT): an update on epidemiology, clinical presentation, and natural history in north american and european cases. Curr Hematol Malig Rep. 2016;11(6):514-27. PMid:27778143.

2 Kim WY, Montes-Mojarro IA, Fend F, Quintanilla-Martinez L. Epstein-barr virus-associated T and NK-Cell Lymphoproliferative Diseases. Front Pediatr. 2019;7:71. PMid:30931288.

3 Au WY, Weisenburger DD, Intragumtornchai T, et al. Clinical differences between nasal and extranasal natural killer/T-cell lymphoma: a study of 136 cases from the International Peripheral T-Cell Lymphoma Project. Blood. 2009;113(17):3931-7. PMid:19029440.

4 Kim TM, Lee SY, Jeon YK, et al. Clinical heterogeneity of extranodal NK/T-cell lymphoma, nasal type: a national survey of the Korean Cancer Study Group. Ann Oncol. 2008;19(8):1477-84. PMid:18385201.

5 Li S, Feng X, Li T, et al. Extranodal NK/T-cell lymphoma, nasal type: a report of 73 cases at MD Anderson Cancer Center. Am J Surg Pathol. 2013;37(1):14-23. PMid:23232851.

6 Gualco G, Domeny-Duarte P, Chioato L, Barber G, Natkunam Y, Bacchi CE. Clinicopathologic and molecular features of 122 Brazilian cases of nodal and extranodal NK/T-cell lymphoma, nasal type, with EBV subtyping analysis. Am J Surg Pathol. 2011;35(8):1195-203. PMid:21716086.

7 Dojcinov SD, Fend F, Quintanilla-Martinez L. EBV-Positive Lymphoproliferations of B- T- and NK-Cell Derivation in Non-Immunocompromised Hosts. Pathogens. 2018;7(1):28. PMid:29518976.

8 Lepeak LM, Yang DT, Chang JE. Extranodal NK/T-cell lymphoma presenting with primary cardiac involvement. Hematol Rep. 2011;3(2):e9. PMid:22184541.

9 Wang X, Gong Z, Li SX, Yan W, Song Y. Extranodal nasal-type natural killer/T-cell lymphoma with penile involvement: a case report and review of the literature. BMC Urol. 2017;17(1):77. PMid:28874193.

10 Li S, Feng X, Li T, et al. Extranodal NK/T-cell Lymphoma, Nasal Type: A report of 73 Cases at MD Anderson Cancer Center. Am J Surg Pathol. 2013;37(1):14-23. PMid:23232851.

11 Chinen K, Izumo T. Cardiac involvement by malignant lymphoma: a clinicopathologic study of 25 autopsy cases based on the WHO classification. Ann Hematol. 2005;84(8):498-505. PMid:15782345.

12 McDonnell PJ, Mann RB, Bulkley BH. Involvement of the heart by malignant lymphoma: A clinicopathologic study. Cancer. 1982;49(5):944-51.<944::AID-CNCR2820490519>3.0.CO;2-C. PMid:7037154.

13 Kawasaki H, Shigeno K, Ohnishi K, et al. A case of primary cutaneous natural killer/T-cell lymphoma, nasal type, directly invading to the heart. Leuk Lymphoma. 2008;49(5):1008-11. PMid:18464123.

14 Baek YS, Shin SH, Yi HG, et al. Cardiac involvement in CD56 negative primary pancreatic extranodal NK/T-cell lymphoma, nasal type, presenting with ventricular tachycardia during the early stages of chemotherapy. Intern Med. 2014;53(20):2333-6. PMid:25318798.

15 Kanesvaran R, Tao M, Huat IT, Weng DT, Eng DN, Thye LS. Malignant arrhythmia: a case report of nasal NK/T-cell lymphoma with cardiac involvement. Acta Oncol. 2009;48(4):637-9. PMid:19191047.

16 Li Y, Damjanov I. Extranodal NK/T Cell Lymphoma Causing Cardiorespiratory Failure. Case Rep Hematol. 2016;2016:2394809. PMid:27493813.

17 Deepti AN, Noone ML, Mahadevan A, et al. Primary cardiac cytotoxic T-cell lymphoma presenting with neurological deficits: a case report. Cardiovasc Pathol. 2008;17(5):334-8. PMid:18402804.

18 Gurion R, Mehta N, Migliacci JC, et al. Central nervous system involvement in T-cell lymphoma: A single center experience. Acta Oncol. 2016;55(5):561-6. PMid:27046135.

19 Mei M, Wang Y, Zhang M. Causes of mortality in cases with extra nodal natural killer/T-cell lymphoma, nasal type: A cohort study. PLoS One. 2019;14(4):e0214860. PMid:30995261.

20 Marques-Piubelli ML, Salas YI, Pachas C, Becker-Hecker R, Vega F, Miranda RN. Epstein-Barr virus-associated B-cell lymphoproliferative disorders and lymphomas: a review. Pathology. 2020;52(1):40-52. PMid:31706670.

Submitted date:

Accepted date:

Publication date:

605a4383a953957574021ec2 autopsy Articles
Links & Downloads

Autops Case Rep

Share this page
Page Sections