Korean J Gastroenterol.  2022 Oct;80(4):190-194. 10.4166/kjg.2022.076.

Gastric CD56-negative Extranodal Natural Killer/T-cell Lymphoma: A Case Report

• Chung J
• Jee SR
• Choi E
• Yu SJ
• Yoon JS
• Lee HS
• Lee SH
• Park SJ
• Park HY

• KMID: 2534468
• DOI: http://doi.org/10.4166/kjg.2022.076

Abstract

Extranodal natural killer (NK)/T-cell lymphoma, nasal type (ENKTCL-NT) is the most common subtype of Epstein-Barr virus-associated NK/T-cell lymphomas. ENKTCL-NT occurs infrequently in the gastrointestinal tract. In particular, reports of ENKTCL-on NT arising from the stomach are extremely rare. Several clusters of differentiation (CDs) have been useful in recognizing NK-cells, T-cells, and tumor cells of NK/T-cell lymphomas. Among them, the CD56 antigen is considered the most sensitive marker for ENKTCL-NT and is expressed in almost all cases of ENKTCL-NT. Thus, the development of CD56-negative ENKTCL-NT is highly atypical. This paper reports a case of a young Asian female who presented with gastric ulcer bleeding. The patient was histologically diagnosed with ENKTCL-NT. No tumor cells for CD56 were observed, whereas no monoclonality of the T-cell receptor gamma gene rearrangement was detected in the tumor cells. The patient was scheduled for systemic chemotherapy six times and achieved complete remission. Peripheral blood-hematopoietic stem cell transplantation was performed later.

Keyword

Lymphoma; extranodal NK-T-cell; Stomach ulcer; CD56 antigen; T-lymphocytes

Figure

• 

  Fig. 1 Upper gastrointestinal endoscopic examination showing a 3×3 cm-sized, single, round, cratered ulcer with blood clots at the gastric fundus.

• 

  Fig. 2 Specimens stained with hematoxylin and eosin stain (H&E). (A) Specimen showing ulcer and necrosis with the infiltration of atypical lymphocytes (×100). (B) Specimen showing atypical lymphocyte infiltration into the lamina propria layer (×200).

• 

  Fig. 3 Immunohistochemical staining results for tumor cells. (A) Tumor cells showing positive findings for polyclonal rabbit antihuman CD3 antibody against the CD3 antigen (×400). (B) Tumor cells showing completely negative findings for the monoclonal anti-CD56 antibody against CD56 antigen (×400).

• 

  Fig. 4 EBERs-ISH revealing positive signals with deeper purple color (red arrows) on the nuclear staining in a large number of tumor cells (×400). EBERs-ISH, Epstein-Barr virus (EBV)-encoded small nuclear early RNAs by in situ hybridization.

• 

  Fig. 5 Positron emission tomography-computed tomography showing multiple lymphomatous lesions. (A) Gastric fundus, (B, C, black arrows) spleen, (C, white arrow) left adrenal gland, (D) omentum of the left upper abdomen, (E) both paratracheal lymph nodes, and (F) parenchyma of both lungs.

Reference

1. Nagata H, Konno A, Kimura N, et al. 2001; Characterization of novel natural killer (NK)-cell and gammadelta T-cell lines established from primary lesions of nasal T/NK-cell lymphomas associated with the Epstein-Barr virus. Blood. 97:708–713. DOI: 10.1182/blood.V97.3.708. PMID: 11157488.

2. Swerdlow SH, Campo E, Pileri SA, et al. 2016; The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood. 127:2375–2390. DOI: 10.1182/blood-2016-01-643569. PMID: 26980727. PMCID: PMC4874220.

3. Harabuchi Y, Takahara M, Kishibe K, Nagato T, Kumai T. 2019; Extranodal natural killer/T-cell lymphoma, nasal type: basic science and clinical progress. Front Pediatr. 7:141. DOI: 10.3389/fped.2019.00141. PMID: 31041299. PMCID: PMC6476925.

4. Vainder C, Ho J, Swerdlow SH, Akilov OE. 2016; CD56-extranodal natural killer (NK)/T-cell lymphoma, nasal type presenting as skin ulcers in a white man. JAAD Case Rep. 2:390–396. DOI: 10.1016/j.jdcr.2016.05.020. PMID: 27752533. PMCID: PMC5061065.

5. Tse E, Kwong YL. 2017; The diagnosis and management of NK/T-cell lymphomas. J Hematol Oncol. 10:85. DOI: 10.1186/s13045-017-0452-9. PMID: 28410601. PMCID: PMC5391564.

6. Tse E, Kwong YL. 2013; How I treat NK/T-cell lymphomas. Blood. 121:4997–5005. DOI: 10.1182/blood-2013-01-453233. PMID: 23652805.

7. Kim SJ, Jung HA, Chuang SS, et al. 2013; Extranodal natural killer/T-cell lymphoma involving the gastrointestinal tract: analysis of clinical features and outcomes from the Asia Lymphoma Study Group. J Hematol Oncol. 6:86. DOI: 10.1186/1756-8722-6-86. PMID: 24238138. PMCID: PMC4225665.

8. Kim EK, Jang M, Yang WI, Yoon SO. 2021; Primary gastrointestinal T/NK cell lymphoma. Cancers (Basel). 13:2679. DOI: 10.3390/cancers13112679. PMID: 34072328. PMCID: PMC8199162.

9. Li H, Lyu W. 2020; Intestinal NK/T cell lymphoma: a case report. World J Gastroenterol. 26:3989–3997. DOI: 10.3748/wjg.v26.i27.3989. PMID: 32774072. PMCID: PMC7385560.

10. Chiu KW, Changchien CS, Chuah SK, Chen CL. 1995; Endoscopic and image features in primary gastro-intestinal lymphoma: a 7-year experience. Hepatogastroenterology. 42:367–370.

11. Kim JH, Lee JH, Lee J, et al. 2007; Primary NK-/T-cell lymphoma of the gastrointestinal tract: clinical characteristics and endoscopic findings. Endoscopy. 39:156–160. DOI: 10.1055/s-2006-945114. PMID: 17657701.

12. Polyatskin IL, Artemyeva AS, Krivolapov YA. 2019; Revised WHO classification of tumors of hematopoietic and lymphoid tissues, 2017 (4th edition):lymphoid tumors. Arkh Patol. 81:59–65. DOI: 10.17116/patol20198103159. PMID: 31317932.

13. Morice WG. 2007; The immunophenotypic attributes of NK cells and NK-cell lineage lymphoproliferative disorders. Am J Clin Pathol. 127:881–886. DOI: 10.1309/Q49CRJ030L22MHLF. PMID: 17509985.

14. Kim KJ, Lee GH, Hong SS, et al. 2005; A case of duodenal relapse of Epstein-Barr virus-positive, CD56-negative extranodal NK/T-cell lymphoma, nasal type. Korean J Gastrointest Endosc. 30:204–209.

15. Zhang L, Zhang P, Wen J, Chen X, Zhang H. 2016; Primary gastric natural killer/T-cell lymphoma with diffuse CD30 expression and without CD56 expression: a case report. Oncol Lett. 11:969–972. DOI: 10.3892/ol.2015.4015. PMID: 26893677. PMCID: PMC4734030.

16. Pongpruttipan T, Sukpanichnant S, Assanasen T, et al. 2012; Extranodal NK/T-cell lymphoma, nasal type, includes cases of natural killer cell and αβ, γδ, and αβ/γδ T-cell origin: a comprehensive clinicopathologic and phenotypic study. Am J Surg Pathol. 36:481–499. DOI: 10.1097/PAS.0b013e31824433d8. PMID: 22314189.