Primary intestinal B-cell lymphoma: a prospective multicentre clinical study of 91 cases.

BACKGROUND & AIMS: Clinical presentation, diagnosis and prognosis of different primary intestinal lymphomas have not been well described and compared so far. Our aim was to prospectively analyse a series of consecutive patients presenting different types of B-cell primary intestinal lymphomas.

METHODS: Adult patients with primary intestinal lymphoma, collected between 1991 and 2000 within the multicenter national study in France were evaluated and followed up prospectively. Clinical features and treatment outcomes were analyzed and compared among different groups of lymphomas.

RESULTS: Among 91 cases of B-cell primary intestinal lymphomas identified, 38 (41%) were diffuse large B cell lymphomas, 34 (37%) mantle cell lymphomas, 12 (13%) follicular lymphomas, 5 (5%) marginal zone MALT-lymphomas, and 2 (3%) Burkitt's lymphomas. A differential diagnosis could be made on the basis of tumour cell morphology and phenotype assessed by immunohistochemistry. Clinical presentation of the different types of lymphomas varied with respect to age, symptoms, circumstances of diagnosis, and stage. Overall survival was the poorest for mantle cell lymphomas while diffuse large B cell lymphomas could be cured if in complete remission after first line treatment.

CONCLUSIONS: This study underlines the existence, within the B-cell primary intestinal lymphomas, of several distinct entities with different clinico-pathological features and prognosis, whose identification is important for choosing appropriate therapeutic strategy.