Intestinal Marginal Zone B-Cell Lymphoma of MALT Type: Clinical Manifestation and Outcome of a Rare Disease.

Purpose: Intestinal marginal zone B-cell lymphoma of MALT type (I-MZL) is a relatively uncommon type of lymphoma. Because of the rarity, the natural history and optimal treatment modality has not been well defined. Therefore, we performed a retrospective analysis of the clinical features and treatment outcomes of I-MZL.

Methods: From 1990 to 2005, a total of 27 patients with histologically confirmed I-MZL were analyzed.

Results: The median age was 51 (range: 19–83) years. The study involved 16 males (59%) and 11 females (41%). Patients initially presented with abdominal pain (62.9%), diarrhea (22.2%) and various symptoms. The most common involving site was ileo-cecal area (40.7%) followed by small intestine (25.9%), colorectal (18.5%), multiple intestine involvements (14.8%) in the decreasing order of frequency. Musshoff stage I_E, II_E1, II_E2, III_E, and IV were present in 44%, 15%, 11%, 7.4%, and 22%, respectively. B symptom was observed in 11%. Twenty one (78%) and five (19%) patients were in low or low-intermediate risk group according to international prognostic index (IPI). Sixty-three percents (17/27) were in low risk group in follicular lymphoma international prognostic index (FLIPI). Treatment was mainly combined with surgical modality. Complete and partial remissions were achieved in 22 (82%) and 1(4%). After the median follow-up duration of 56.1 months, Median progression free survival (PFS) were 8.8 (95% CI: 3.8–13.8) years. The estimated 5-year overall survival (OS) and PFS were 86% and 54%, respectively. Stage ≥ II_E2 was poor prognostic factor of PFS and OS.

Conclusion: I-MZL trends to be an indolent disease - prolonged survival with frequent relapse like other site MZL of MALT type. They present commonly early stage, low risk state and respond well local and systemic treatment. I-MZL has favorable prognosis.