Objective To summarize the clinicopathologic characteristics and outcomes of monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL).

Methods The clinical profiles of patients diagnosed with MEITL in Zhongshan Hospital, Fudan University from Jan. 2014 to Mar. 2021 were retrospective analyzed.

Results All 7 patients were male. The median age of them at diagnosis was 55 (33-77) years old. The most frequent site of lesion involved was small intestine. Symptoms including abdominal pain, diarrhea, and weigh loss were common, and intestinal perforation occurred in most patients. Six patients were diagnosed through surgical resection of lesions, another patient was confirmed pathologically by gastroscope biopsy. Among patients underwent surgical resection, emergency surgeries were carried out in four patients to manage intestinal perforation or bleeding. Most tumors expressed CD8, T-cell intracellular antigen-1, and CD56, while none expressed Epstein-Barr virus-encoded RNA. Five patients were diagnosed at late stage, while another two were at relatively early stage. Five patients received chemotherapy, and chidamide-based combination regimens were used in 3 patients. Five patients died and another 2 patients at early stage who undergone selective surgery survived. Up to November 9, 2021, overall survival of this cohort was less than one year.

Conclusions Late stage at diagnosis and severe complications may contribute to dismal prognosis, while the prognosis of patients with selective surgery, good response to chemotherapy may be relatively improved. Novel diagnostic and therapeutic approaches are required to improve survival of MEITL patients.