We describe the clinicopathologic and molecular features of 2 cases of gastric monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), which were first diagnosed from gastric biopsies, one was primary whereas the other was gastric involvement by MEITL. Both cases were older men with stomach ulcers. Case 1 was admitted for a hemorrhage in the upper digestive tract and case 2 for edema. Histology of both cases showed infiltrated monomorphic and medium-sized lymphocytes with lymphoid epithelial phenomenon. An inflammatory background and vascular hyperplasia were also observed likely due to the ulceration. Neoplastic cells expressed CD2, CD3, CD7, CD8, CD56, TIA-1, and MYC, not CD5, CD4, Granzyme B, CD20, CD30, TdT, or EBER. Both lymphomas showed TCRG gene rearrangement and c-MYC gains. Moreover, we first affirmed polysomy of chromosome 8 in case 2. For correct diagnosis of this rare tumor at the rare location, it is important that pathologists raise the possibility and exclude other differential diagnoses.
SwerdlowSHCampoEPileriSA, et al. The 2016 revision of the World Health Organization (WHO) classification of lymphoid neoplasms. Blood. 2016;127:2375-2390.
2.
ChanJKCChanACLCheukW, et al. Type II enteropathy-associated T-cell lymphoma: a distinct aggressive lymphoma with frequent γδ T-cell receptor expression. Am J Surg Pathol. 2011;35:1557-1569.
3.
SunJLuZYangDChenJ.Primary intestinal T-cell and NK-cell lymphomas: a clinicopathological and molecular study from China focused on type II enteropathy-associated T-cell lymphoma and primary intestinal NK-cell lymphoma. Mod Pathol. 2011;24:983-992.
4.
TseEGillHLoongF, et al. Type II enteropathy-associated T-cell lymphoma: a multicenter analysis from the Asia Lymphoma Study Group. Am J Hematol. 2012;87:663-668.
5.
PengJCZhongLRanZH.Primary lymphomas in the gastrointestinal tract. J Dig Dis. 2015;16:169-176.
6.
IshibashiHNimuraSKayashimaY, et al. Multiple lesions of gastrointestinal tract invasion by monomorphic epitheliotropic intestinal T-cell lymphoma, accompanied by duodenal and intestinal enteropathy-like lesions and microscopic lymphocytic proctocolitis: a case series. Diagn Pathol. 2016;11:66.
7.
TianSXiaoSYChenQLiuHPingJ.Monomorphic epitheliotropic intestinal T-cell lymphoma may mimic intestinal inflammatory disorders. Int J Immunopathol Pharmacol. 2019;33:2058738419829387.
8.
KikumaKYamadaKNakamaraS, et al. Detailed clinicopathological characteristics and possible lymphomagenesis of type II intestinal enteropathy associated T-cell lymphoma in Japan. Hum Pathol. 2014;45:1276-1284.
9.
ChanTSYLeeEKhongPLTseEWCKwongYL. Positron emission tomography computed tomography features of monomorphic epitheliotropic intestinal T-cell lymphoma. Hematology. 2018;23:10-16.
10.
La RosaSBernasconiBVanoliA, et al. c-MYC amplification and c-myc protein expression in pancreatic acinar cell carcinomas. New insights into the molecular signature of these rare cancers. Virchows Arch. 2018;473:435-441.
11.
DelabieJHolteHVoseJM, et al. Enteropathy-associated T-cell lymphoma: clinical and histological findings from the international peripheral T-cell lymphoma project. Blood. 2011;118:148-155.
12.
WangLLiuYLinXY, et al. A case of enteropathy-associated T-cell lymphoma (Type I) arising in stomach without refractory celiac disease. Diagn Pathol. 2012;7:172.
13.
TanSYOoiASAngMK, et al. Nuclear expression of MATK is a novel marker of type II enteropathy-associated T-cell lymphoma. Leukemia. 2011;25:555-557.
14.
Juárez-SalcedoLMSokolLChavezJCDaliaS.Primary gastric lymphoma, epidemiology, clinical diagnosis, and treatment. Cancer Control. 2018;25:1073274818778256.
15.
ThieblemontCZuccaE.Clinical aspects and therapy of gastrointestinal MALT lymphoma. Best Pract Res Clin Haematol. 2017;30:109-117.
16.
MargolskeeEJobanputraVLewisSKAlobeidBGreenPHRBhagatG.Indolent small intestinal CD4+ T-cell lymphoma is a distinct entity with unique biologic and clinical features. PLoS One. 2013;8:e68343.
17.
McElroyMKReadWLHarmonGSWeidnerN.A unique case of an indolent CD56-positive T-cell lymphoproliferative disorder of the gastrointestinal tract: a lesion potentially misdiagnosed as natural killer/T-cell lymphoma. Ann Diagn Pathol. 2011;15:370-375.
18.
CheminantMBruneauJMalamutG, et al; CELAC Network. NKp46 is a diagnostic biomarker and may be a therapeutic target in gastrointestinal T-cell lymphoproliferative diseases: a CELAC study. Gut. 2019;68:1396-1405.
19.
ZhangYCShaZhaoYuJB, et al. Gastric involvement of extranodal NK/T-cell lymphoma, nasal type: a report of 3 cases with literature review. Int J Surg Pathol. 2008;16:450-454.
20.
YangHWuMYinWSunWZhangG.Diagnosis and treatment of a patient with primary gastric extranodal natural killer/T-cell lymphoma, nasal type. Leuk Lymphoma. 2010;51:2137-2140.
21.
TakataKNoujima-HaradaMMiyata-TakataT, et al. Clinicopathologic analysis of 6 lymphomatoid gastropathy cases expanding the disease spectrum to CD4-CD8+ cases. Am J Surg Pathol. 2015;39:1259-1266.
22.
TakeuchiKYokoyamaMIshizawaS, et al. Lymphomatoid gastropathy: a distinct clinicopathologic entity of self-limited pseudomalignant NK-cell proliferation. Blood. 2010;116:5631-5637.
23.
DaumSUllrichRHeiseW, et al. Intestinal non-Hodgkin’s lymphoma: a multicenter prospective clinical study from the German Study Group on Intestinal non-Hodgkin’s Lymphoma. J Clin Oncol. 2003;21:2740-2746.
24.
SieniawskiMAngamuthuNBoydK, et al. Evaluation of enteropathy-associated T-cell lymphoma comparing standard therapies with a novel regimen including autologous stem cell transplantation. Blood. 2010;115:3664-3670.
25.
NairismagiMLTanJLimJQ, et al. JAK/STAT and G-protein-coupled receptor signaling pathways are frequently altered in epitheliotropic intestinal T-cell lymphoma. Leukemia. 2016;30:1311-1319.
26.
MutzbauerGMaurusKBuszelloC, et al. SYK expression in monomorphic epitheliotropic intestinal T-cell lymphoma. Mod Pathol. 2018;31:505-516.
27.
MoffittABOndrejkaSLMcKinneyM, et al. Enteropathy-associated T cell lymphoma subtypes are characterized by loss of function of SETD2. J Exp Med. 2017;214:1371-1386.
28.
PaneaRILoveCLShingletonJR, et al. The whole genome landscape of Burkitt lymphoma subtypes. Blood. 2019;134: 1598-1607.
29.
DeleeuwRJZettlAKlinkerE, et al. Whole-genome analysis and HLA genotyping of enteropathy-type T-cell lymphoma reveals 2 distinct lymphoma subtypes. Gastroenterology. 2007;132:1902-1911.
30.
TakeshitaMNakamaraSKikumaK, et al. Pathological and immunohistological findings and genetic aberrations of intestinal enteropathy-associated T cell lymphoma in Japan. Histopathology. 2011;58:395-407.
31.
MaljaieSHBrito-BabapulleVMatutesEHiornsLRDe SchouwerPJCatovskyD.Expression of c-myc oncoprotein in chronic T cell leukemias. Leukemia. 1995;9:1694-1699.
32.
IslamSPaekALHammerM, et al. Drug induced aneuploidy and polyploidy is a mechanism of disease relapse in MYC/BCL2-addicted diffuse large B-cell lymphoma. Oncotarget. 2018;9:35875-35890.
