Autopsy and Case Reports
https://www.autopsyandcasereports.org/article/doi/10.4322/acr.2023.445
Autopsy and Case Reports
Autopsy Case Report

Disseminated Nasal subtype Extranodal NK/T-cell lymphoma and its diagnostic difficulties in antemortem biopsies

Aravind Sekar; Siddharth Jain; Jaimanti Bakshi; Suneel Rachagiri; Harish Bhujade; Rajender Kumar; Amanjit Bal

Downloads: 0
Views: 424

Abstract

Extranodal NK/T- cell lymphoma (ENKTCL) is an aggressive lymphoma driven by Epstein-Barr virus (EBV) infection in genetically susceptible individuals. It was historically called a lethal midline granuloma. Due to the angio-destructive nature of ENKTCL, lymphoma cells are often accompanied and masked by necrosis and dense inflammation in the biopsy. Further, the biopsy may show vasculitis, which can mimic granulomatosis with polyangiitis. Due to these masquerades, ENKTCL is often misdiagnosed in the biopsy. Several biopsies may be required to establish the diagnosis. We describe the clinical course and autopsy findings of a young female who presented with a hard-palate ulcer. Antemortem biopsies failed to establish the diagnosis. The autopsy revealed an advanced nasal subtype of Extranodal NK/T-cell lymphoma with dissemination to the kidneys, adrenals, liver, spleen, and small intestine.

Keywords

Hard Palate, Epstein-Barr Virus Infections, Autopsy

References

1 Li W. The 5th Edition of the World Health Organization Classification of Hematolymphoid Tumors. In: Li W, editor. Leukemia [Internet]. Austrália: Exon Publications; 2022. [cited 2023 Apr 19]. Available from: http://www.ncbi.nlm.nih.gov/books/NBK586208/

2 Yoon SO, Suh C, Lee DH, et al. Distribution of lymphoid neoplasms in the Republic of Korea: analysis of 5318 cases according to the World Health Organization classification. Am J Hematol. 2010;85(10):760-4. http://dx.doi.org/10.1002/ajh.21824. PMid:20806229.

3 Yang QP, Zhang WY, Yu JB, et al. Subtype distribution of lymphomas in Southwest China: analysis of 6,382 cases using WHO classification in a single institution. Diagn Pathol. 2011;6(1):77. http://dx.doi.org/10.1186/1746-1596-6-77. PMid:21854649.

4 Quintanilla-Martinez L, Franklin JL, Guerrero I, et al. Histological and immunophenotypic profile of nasal NK/T cell lymphomas from Peru: high prevalence of p53 overexpression. Hum Pathol. 1999;30(7):849-55. http://dx.doi.org/10.1016/S0046-8177(99)90147-8. PMid:10414505.

5 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.http://dx.doi.org/10.1182/blood-2008-10-185256.

6 Kim SJ, Yoon DH, Jaccard A, et al. A prognostic index for natural killer cell lymphoma after non-anthracycline-based treatment: a multicentre, retrospective analysis. Lancet Oncol. 2016;17(3):389-400. http://dx.doi.org/10.1016/S1470-2045(15)00533-1. PMid:26873565.

7 Mendenhall WM, Olivier KR, Lynch JW Jr, Mendenhall NP. Lethal midline granuloma-nasal natural killer/T-cell lymphoma. Am J Clin Oncol. 2006;29(2):202-6. http://dx.doi.org/10.1097/01.coc.0000198738.61238.eb. PMid:16601443.

8 Borges A, Fink J, Villablanca P, Eversole R, Lufkin R. Midline Destructive Lesions of the Sinonasal Tract: Simplified Terminology Based onHistopathologic Criteria. AJNR Am J Neuroradiol. 2000;21(2):331-6. PMid:10696019.

9 Montes-Mojarro IA, Fend F, Quintanilla-Martinez L. EBV and the Pathogenesis of NK/T Cell Lymphoma. Cancers (Basel). 2021;13(6):1414. http://dx.doi.org/10.3390/cancers13061414. PMid:33808787.

10 Li Z, Xia Y, Feng LN, et al. Genetic risk of extranodal natural killer T-cell lymphoma: a genome-wide association study. Lancet Oncol. 2016;17(9):1240-7. http://dx.doi.org/10.1016/S1470-2045(16)30148-6. PMid:27470079.

11 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. http://dx.doi.org/10.3389/fped.2019.00071. PMid:30931288.

12 de Mel S, Hue SSS, Jeyasekharan AD, Chng WJ, Ng SB. Molecular pathogenic pathways in extranodal NK/T cell lymphoma. J Hematol Oncol. 2019;12(1):33. http://dx.doi.org/10.1186/s13045-019-0716-7. PMid:30935402.

13 Xiang CX, Chen ZH, Zhao S, et al. Laryngeal extranodal nasal-type natural killer/t-cell lymphoma: a clinicopathologic study of 31 cases in China. Am J Surg Pathol. 2019;43(7):995-1004. http://dx.doi.org/10.1097/PAS.0000000000001266. PMid:31045893.

14 Lee YT, Chang YS, Lai CC, Chen WS, Yang AH, Tsai CY. Natural killer (NK)/T-cell lymphoma mimicking granulomatosis with polyangiitis (Wegener’s). Scand J Rheumatol. 2012;41(5):407-8. http://dx.doi.org/10.3109/03009742.2012.696684. PMid:22827256.

15 Coordes A, Loose SM, Hofmann VM, et al. Saddle nose deformity and septal perforation in granulomatosis with polyangiitis. Clin Otolaryngol. 2018;43(1):291-9. http://dx.doi.org/10.1111/coa.12977. PMid:28881107.

16 Sinico RA, Di Toma L, Radice A. Renal involvement in anti-neutrophil cytoplasmic autoantibody associated vasculitis. Autoimmun Rev. 2013;12(4):477-82. http://dx.doi.org/10.1016/j.autrev.2012.08.006. PMid:22921791.

17 Sharma A, Naidu GSRSNK, Rathi M, et al. Clinical features and long-term outcomes of 105 granulomatosis with polyangiitis patients: A single center experience from north India. Int J Rheum Dis. 2018;21(1):278-84. http://dx.doi.org/10.1111/1756-185X.13071. PMid:28337860.

18 Marsh RA. Epstein-Barr virus and hemophagocytic lymphohistiocytosis. Front Immunol. 2018;8:1902. http://dx.doi.org/10.3389/fimmu.2017.01902. PMid:29358936.

19 Lim SW, Ryu KJ, Lee H, Ko YH, Kim WS, Kim SJ. Serum IL18 is associated with hemophagocytosis and poor survival in extranodal natural killer/T-cell lymphoma. Leuk Lymphoma. 2019;60(2):317-25. http://dx.doi.org/10.1080/10428194.2018.1480772. PMid:29979096.
 


Submitted date:
04/20/2023

Accepted date:
08/11/2023

Publication date:
10/05/2023

651e9c51a953954d1716ef32 autopsy Articles
Links & Downloads

Autops Case Rep

Share this page
Page Sections