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Monomorphic epitheliotropic intestinal T-cell lymphoma

MedGen UID:
474158
•Concept ID:
C3272525
•
Neoplastic Process
Synonyms: Monomorphic CD56+ Intestinal T-Cell Lymphoma; Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma; monomorphic epitheliotropic intestinal T-cell lymphoma; Type II EATL
SNOMED CT: Monomorphic epitheliotropic intestinal T-cell lymphoma (787036009)
 
Monarch Initiative: MONDO:0958096

Definition

A mature T-cell and NK-cell non-Hodgkin lymphoma of intraepithelial T-lymphocytes. It usually arises from the small intestine. A minority of cases arise from the large intestine or the stomach. It is characterized by the presence of a monomorphic cellular infiltrate of small to medium-sized T-lymphocytes that are cytotoxic and express CD56. It is not associated with celiac disease. [from NCI]

Recent clinical studies

Etiology

Monomorphic epitheliotropic intestinal T-cell lymphoma comprises morphologic and genomic heterogeneity impacting outcome.
Veloza L, Cavalieri D, Missiaglia E, Ledoux-Pilon A, Bisig B, Pereira B, Bonnet C, Poullot E, Quintanilla-Martinez L, Dubois R, Llamas-Gutierrez F, Bossard C, De Wind R, Drieux F, Fontaine J, Parrens M, Sandrini J, Fataccioli V, Delfau-Larue MH, Daniel A, Lhomme F, Clément-Filliatre L, Lemonnier F, Cairoli A, Morel P, Glaisner S, Joly B, El Yamani A, Laribi K, Bachy E, Siebert R, Vallois D, Gaulard P, Tournilhac O, De Leval L
Haematologica 2023 Jan 1;108(1):181-195. doi: 10.3324/haematol.2022.281226. PMID: 35708139Free PMC Article
Diagnostic approach to T- and NK-cell lymphoproliferative disorders in the gastrointestinal tract.
Susan SH, Ng SB, Wang S, Tan SY
Semin Diagn Pathol 2021 Jul;38(4):21-30. Epub 2021 Mar 31 doi: 10.1053/j.semdp.2021.03.004. PMID: 34016481
T- and NK-cell lymphoproliferative disorders of the gastrointestinal tract: review and update.
van Vliet C, Spagnolo DV
Pathology 2020 Jan;52(1):128-141. Epub 2019 Nov 11 doi: 10.1016/j.pathol.2019.10.001. PMID: 31727264
Gastrointestinal T- and NK-cell lymphomas and indolent lymphoproliferative disorders.
Soderquist CR, Bhagat G
Semin Diagn Pathol 2020 Jan;37(1):11-23. Epub 2019 Aug 28 doi: 10.1053/j.semdp.2019.08.001. PMID: 31522873
Rare T-Cell Subtypes.
van der Weyden C, McCormack C, Lade S, Johnstone RW, Prince HM
Cancer Treat Res 2019;176:195-224. doi: 10.1007/978-3-319-99716-2_10. PMID: 30596220

Diagnosis

Diagnostic approach to T- and NK-cell lymphoproliferative disorders in the gastrointestinal tract.
Susan SH, Ng SB, Wang S, Tan SY
Semin Diagn Pathol 2021 Jul;38(4):21-30. Epub 2021 Mar 31 doi: 10.1053/j.semdp.2021.03.004. PMID: 34016481
T- and NK-cell lymphoproliferative disorders of the gastrointestinal tract: review and update.
van Vliet C, Spagnolo DV
Pathology 2020 Jan;52(1):128-141. Epub 2019 Nov 11 doi: 10.1016/j.pathol.2019.10.001. PMID: 31727264
Gastrointestinal T- and NK-cell lymphomas and indolent lymphoproliferative disorders.
Soderquist CR, Bhagat G
Semin Diagn Pathol 2020 Jan;37(1):11-23. Epub 2019 Aug 28 doi: 10.1053/j.semdp.2019.08.001. PMID: 31522873
Rare T-Cell Subtypes.
van der Weyden C, McCormack C, Lade S, Johnstone RW, Prince HM
Cancer Treat Res 2019;176:195-224. doi: 10.1007/978-3-319-99716-2_10. PMID: 30596220
Update on Gastrointestinal Lymphomas.
Weindorf SC, Smith LB, Owens SR
Arch Pathol Lab Med 2018 Nov;142(11):1347-1351. doi: 10.5858/arpa.2018-0275-RA. PMID: 30407861

Therapy

PEG-Asparaginase Single-Agent Rescue in an Advanced Case of Monomorphic Epitheliotropic Intestinal T Cell Lymphoma.
Barbieri E, Pozzi S, Gelmini R, Roncati L, Maccaferri M, Potenza L, Marasca R, Luppi M, Leonardi G
J Gastrointest Cancer 2023 Mar;54(1):304-308. Epub 2022 Mar 1 doi: 10.1007/s12029-022-00808-0. PMID: 35230648
Monomorphic epitheliotropic intestinal T-cell lymphoma presenting as melena with long-term survival: A case report and review of literature.
Ozaka S, Inoue K, Okajima T, Tasaki T, Ariki S, Ono H, Ando T, Daa T, Murakami K
World J Gastroenterol 2021 Oct 14;27(38):6501-6510. doi: 10.3748/wjg.v27.i38.6501. PMID: 34720538Free PMC Article
Early central nervous system relapse of monomorphic epitheliotropic intestinal T-cell lymphoma after cord blood transplantation.
Nato Y, Miyazaki K, Imai H, Nakano E, Kageyama Y, Ino K, Fujieda A, Matsumoto T, Tawara I, Tanaka K, Yamaguchi M, Katayama N
Int J Hematol 2021 Jul;114(1):129-135. Epub 2021 Mar 1 doi: 10.1007/s12185-021-03107-9. PMID: 33646526
Multicenter retrospective analysis of the clinicopathologic features of monomorphic epitheliotropic intestinal T-cell lymphoma.
Yi JH, Lee GW, Do YR, Jung HR, Hong JY, Yoon DH, Suh C, Choi YS, Yi SY, Sohn BS, Kim BS, Oh SY, Park J, Jo JC, Lee SS, Oh YH, Kim SJ, Kim WS
Ann Hematol 2019 Nov;98(11):2541-2550. Epub 2019 Sep 6 doi: 10.1007/s00277-019-03791-y. PMID: 31493002
Enteropathy-Associated T-Cell Lymphoma.
Ondrejka S, Jagadeesh D
Curr Hematol Malig Rep 2016 Dec;11(6):504-513. doi: 10.1007/s11899-016-0357-7. PMID: 27900603

Prognosis

Diagnostic approach to T- and NK-cell lymphoproliferative disorders in the gastrointestinal tract.
Susan SH, Ng SB, Wang S, Tan SY
Semin Diagn Pathol 2021 Jul;38(4):21-30. Epub 2021 Mar 31 doi: 10.1053/j.semdp.2021.03.004. PMID: 34016481
Monomorphic Epitheliotropic Intestinal T-cell Lymphoma: A Study of Four Cases and Review of Literature.
Fei F, Reddy V, Patel CR, Dhall D, Lee G, Meng-Jun X, Al Diffalha S
Ann Clin Lab Sci 2020 Nov;50(6):806-812. PMID: 33334797
T- and NK-cell lymphoproliferative disorders of the gastrointestinal tract: review and update.
van Vliet C, Spagnolo DV
Pathology 2020 Jan;52(1):128-141. Epub 2019 Nov 11 doi: 10.1016/j.pathol.2019.10.001. PMID: 31727264
SYK expression in monomorphic epitheliotropic intestinal T-cell lymphoma.
Mutzbauer G, Maurus K, Buszello C, Pischimarov J, Roth S, Rosenwald A, Chott A, Geissinger E
Mod Pathol 2018 Mar;31(3):505-516. Epub 2017 Oct 20 doi: 10.1038/modpathol.2017.145. PMID: 29052597
Enteropathy-Associated T-Cell Lymphoma.
Ondrejka S, Jagadeesh D
Curr Hematol Malig Rep 2016 Dec;11(6):504-513. doi: 10.1007/s11899-016-0357-7. PMID: 27900603

Clinical prediction guides

Clinicopathological and molecular genetic alterations in monomorphic-epitheliotropic intestinal T-cell lymphoma of the small intestine.
Zhou B, Guo M, Li X, Duan T, Peng L, Hao H
Eur J Med Res 2024 Mar 23;29(1):191. doi: 10.1186/s40001-024-01797-5. PMID: 38520011Free PMC Article
Monomorphic epitheliotropic intestinal T-cell lymphoma presenting as melena with long-term survival: A case report and review of literature.
Ozaka S, Inoue K, Okajima T, Tasaki T, Ariki S, Ono H, Ando T, Daa T, Murakami K
World J Gastroenterol 2021 Oct 14;27(38):6501-6510. doi: 10.3748/wjg.v27.i38.6501. PMID: 34720538Free PMC Article
Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma With Secondary Cutaneous Involvement: A Diagnostic Challenge.
Olmos-Alpiste F, Vázquez I, Gallardo F, Sánchez-Gonzalez B, Colomo L, Pujol RM
Am J Dermatopathol 2021 Apr 1;43(4):300-304. doi: 10.1097/DAD.0000000000001855. PMID: 33264131
Monomorphic Epitheliotropic Intestinal T-cell Lymphoma of the Stomach: Two Case Reports and a Literature Review.
Lu S, Zhou G, Chen M, Liu W, Zhao S
Int J Surg Pathol 2021 Jun;29(4):410-419. Epub 2020 Aug 28 doi: 10.1177/1066896920953906. PMID: 32856508
Endoscopic and clinicopathological characteristics of colorectal T/NK cell lymphoma.
Ishibashi H, Nimura S, Hirai F, Harada N, Iwasaki H, Kawauchi S, Oshiro Y, Matsuyama A, Nakamura S, Takamatsu Y, Yonemasu H, Shimokama T, Takeshita M
Diagn Pathol 2020 Oct 21;15(1):128. doi: 10.1186/s13000-020-01044-5. PMID: 33087157Free PMC Article

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