Bissessur AS, Zhou JC, Xu L, Li ZQ, Ju SW, Jia YL, Wang LB. Surgical management of monomorphic epitheliotropic intestinal T-cell lymphoma followed by chemotherapy and stem-cell transplant: A case report and review of the literature. World J Gastrointest Oncol 2022; 14(11): 2273-2287 [PMID: 36438712 DOI: 10.4251/wjgo.v14.i11.2273]
Corresponding Author of This Article
Lin-Bo Wang, Doctor, MBBS, MD, PhD, Attending Doctor, Chief Doctor, Director, Professor, Surgical Oncologist, Department of Surgical Oncology, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, No. 3 Eastern Qingchun Road, Hangzhou 310006, Zhejiang Province, China. linbowang@zju.edu.cn
Research Domain of This Article
Oncology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Gastrointest Oncol. Nov 15, 2022; 14(11): 2273-2287 Published online Nov 15, 2022. doi: 10.4251/wjgo.v14.i11.2273
Surgical management of monomorphic epitheliotropic intestinal T-cell lymphoma followed by chemotherapy and stem-cell transplant: A case report and review of the literature
Abdul Saad Bissessur, Ji-Chun Zhou, Ling Xu, Zhao-Qing Li, Si-Wei Ju, Yun-Lu Jia, Lin-Bo Wang
Abdul Saad Bissessur, Ji-Chun Zhou, Ling Xu, Zhao-Qing Li, Si-Wei Ju, Lin-Bo Wang, Department of Surgical Oncology, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou 310006, Zhejiang Province, China
Yun-Lu Jia, Department of Medical Oncology, First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310006, Zhejiang Province, China
Author contributions: Bissessur AS and Zhou JC wrote this paper; Bissessur AS conducted literature review; Xu L, Li ZQ, Ju SW, and Jia YL contributed in data acquisition and manuscript preparation; Jia YL, Zhou JC, and Wang LB were responsible for patient care and data analysis; Zhou JC and Wang LB substantively reviewed the manuscript; all authors read and approved the final manuscript.
Informed consent statement: Informed written consent was obtained from the patients for the publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflict of interest to disclose.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Lin-Bo Wang, Doctor, MBBS, MD, PhD, Attending Doctor, Chief Doctor, Director, Professor, Surgical Oncologist, Department of Surgical Oncology, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, No. 3 Eastern Qingchun Road, Hangzhou 310006, Zhejiang Province, China. linbowang@zju.edu.cn
Received: July 26, 2022 Peer-review started: July 26, 2022 First decision: August 8, 2022 Revised: August 22, 2022 Accepted: October 11, 2022 Article in press: October 11, 2022 Published online: November 15, 2022 Processing time: 111 Days and 17.4 Hours
Abstract
BACKGROUND
Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a rare and rapidly progressive intestinal T-cell non-Hodgkin lymphoma associated with a very poor prognosis and a median survival of 7 mo. Advances in the identification of MEITL over the last two decades have led to its recognition as a separate entity. MEITL patients, predominantly male, typically present with vague and nonspecific symptoms and diagnosis is predominantly confirmed at laparotomy. Currently, there are no standardized treatment protocols, and the optimal therapy remains unclear.
CASE SUMMARY
We report a case of MEITL that was initially considered to be gastrointestinal stromal tumor (GIST) and Imatinib was administered for one cycle. The 62-year-old man presented with abdominal pain, abdominal distension, and weight loss of 20 pounds. Within 2 wk, the size of the mass considerably increased on computed tomography scans. The patient underwent surgery followed by chemotherapy with CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) and stem-cell transplant. A correct diagnosis of MEITL was established based on postoperative pathology. Immunophenotypically, the neoplastic cells fulfilled the diagnostic criteria for MEITL as they were CD3+, CD4+, CD8+, CD56+, and TIA-1+.
CONCLUSION
Given that MEITL has no predisposing factor and presents with vague symptoms with rapid progression, the concomitant presence of abdominal symptoms and B symptoms (weight loss, fever, and night sweats) with hypoalbuminemia, anemia, low lymphocytic count and endoscopic findings of diffuse infiltrating type lesions should alert physicians to this rare disease, especially when it comes to Asian patients. Immediate laparotomy should then be carried out followed by chemotherapy and stem-cell transplant.
Core Tip: Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a rare and rapidly progressive intestinal T-cell non-Hodgkin lymphoma. Currently, there is no standardized treatment or diagnostic protocols for MEITL. Chemotherapy followed by stem-cell transplant postoperatively has shown promising results in terms of remission and progression free survival. Since MEITL is associated with a poor prognosis and high recurrence, it is crucial that the oncologist should follow and monitor any relapsing signs.
