A case report and a literature review about central nervous system involvement in monomorphic epitheliotropic intestinal T cell lymphoma

Monomorphic epitheliotropic intestinal T cell lymphoma (MEITL) is a rare subtype of primary T cell lymphoma of the digestive tract, characterized by a highly aggressive clinical course. Surgery, radiotherapy, and chemotherapy (CT) following autologous hematopoietic stem cell transplantation are among the treatments selected for this disease. Nevertheless, there is currently no curative therapy. We present a case of a 60-year-old male patient without history of celiac disease who presented a jejunum perforation. In the specimen, a diffuse infiltration of small to medium-sized T cells with CD3, CD8, and CD56 expression and cytotoxic markers was observed, affecting all the layers of intestinal wall. The diagnosis of MEITL was established, and the patient received six cycles of CT. Disease progression with another intestinal perforation and central nervous system involvement was presented. The patient died 9 months after the diagnosis. Sixteen similar cases were found through PubMed search, and we describe their clinicopathological characteristics.