Introduction: Primary gastrointestinal tract mantle cell lymphoma is very rare. There is paucity of literature regarding natural history and outcome of this unusual entity.
Aims and objectives: The aim of the present study was to analyze clinical profile, epidemiological parameters, and outcome of primary gastrointestinal mantle cell lymphoma patients treated at our institute.
Materials and methods: This was a retrospective observational study of consecutive patients diagnosed with primary gastrointestinal mantle cell lymphoma at our institute between 2001 and 2012.
Results: A total of seven patients, all males with a median age of 67.7 years, were diagnosed with primary gastrointestinal mantle cell lymphoma. Sites involved were the stomach, colon, and rectum. Blastoid and diffuse variants were observed in three patients each, and one patient had nodular pattern. Five patients received cyclophosphamide, adriamycin, vincristine, and prednisolone (CHOP) chemotherapy. Only one patient achieved complete remission and remained disease free for 21 months before being lost to follow up. The remaining four patients had inadequate response to CHOP chemotherapy with a median survival of 6 months. Cyclophosphamide, vincristine, prednisolone (CVP) was given to one patient in view of poor performance status. He had progressive disease and died after first cycle of chemotherapy. One patient is currently undergoing treatment and receiving rituximab with chemotherapy.
Conclusions: Primary gastrointestinal mantle cell lymphoma is highly aggressive with the Mantle Cell Lymphoma International Prognostic Index (MIPI) scores in high-risk group; survival is poor compared to nodal mantle cell lymphoma involving the gastrointestinal tract; patients respond poorly to CHOP chemotherapy. As majority of patients are elderly and not eligible for transplant, the use of rituximab in remission induction and maintenance should be considered to improve outcome of these patients.