From the archives of MD Anderson Cancer Center: Monomorphic epitheliotropic intestinal T-cell lymphoma: A case with an unusual immunophenotype and discussion of differential diagnosis.

Autor: Dcunha NJ; Department of Pathology, Baylor University College of Medicine and The University of Texas MD Anderson Cancer Center, Houston, TX, United States of America., Wei Q; Department of Pathology, Baylor University College of Medicine and The University of Texas MD Anderson Cancer Center, Houston, TX, United States of America., Thakral B; Department of Pathology, Baylor University College of Medicine and The University of Texas MD Anderson Cancer Center, Houston, TX, United States of America., Medeiros LJ; Department of Pathology, Baylor University College of Medicine and The University of Texas MD Anderson Cancer Center, Houston, TX, United States of America. Electronic address: ljmedeiros@mdanderson.org.
Jazyk: angličtina
Zdroj: Annals of diagnostic pathology [Ann Diagn Pathol] 2024 Jun; Vol. 70, pp. 152293. Date of Electronic Publication: 2024 Mar 08.
DOI: 10.1016/j.anndiagpath.2024.152293
Abstrakt: Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a rare and aggressive T-cell neoplasm associated with poor survival. We report a case of MEITL that presented as an ulcerated mass in the jejunum with perforation. Microscopic examination showed that the neoplasm involved the full thickness of the intestinal wall, extended into the mesentery, and was composed of monomorphic, small to medium-size cells. Immunohistochemical analysis showed that the neoplastic cells were positive for T-cell receptor (TCR) delta, CD3, CD7, CD8 (small subset), BCL-2 and TIA-1, and negative for TCR beta, CD4, CD5, CD10, CD20, CD30, CD34, CD56, CD57, CD99, ALK, cyclin D1, granzyme B, MUM1/IRF4, and TdT. The Ki-67 proliferation index was approximately 50 %. In situ hybridization for Epstein-Barr virus-encoded RNA (EBER ISH) was negative. Next-generation sequencing (NGS) analysis showed mutations involving SETD2 and STAT5B. The patient was treated with aggressive chemotherapy and consolidative autologous stem cell transplant and had clinical remission, but relapsed after about one year. Retreatment led to another one-year interval of clinical remission, but at last follow up the patient has relapsed disease involving the ileum and colon. We also discuss the differential diagnosis of MEITL.
Competing Interests: Declaration of competing interest None.
(Copyright © 2024 Elsevier Inc. All rights reserved.)
Databáze: MEDLINE
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