Non-gastric mucosa-associated lymphoid tissue (MALT) lymphomas: analysis of 14 patients

BACKGROUND: Mucosa-associated lymphoid tissue (MALT) lymphomas are a well d efined group of B-cell non-Hodgkin's lymphomas, that arise in a wide variet y of extranodal sites, most frequently in the stomach and related to Helico bacter pylori infection. The aim of the present study was to analyze the pr esenting features, natural history and outcome in 14 patients with non-gast ric MALT lymphoma. PATIENTS AND METHODS: The main clinical data, treatment and outcome were re corded for the 14 patients with non-gastric MALT lymphoma diagnosed at a si ngle institution in a 12 year period. The median age was 68 years and 13 pa tients were females. Diagnosis was made according to the REAL classificatio n criteria. RESULTS: The initial location was thyroid (3 patients), parotid (three), su bmaxilar gland (three), skin (two), Waldeyer's ring tone), breast tone), lu ng tone), small bowel tone), liver tone) and ovary (one). At diagnosis 3 pa tients had greater than or equal to 2 extranodal involved sites. Autoimmune disorders were present in 5 patients: Hashimoto's thyroiditis (three), Sjo gren's syndrome tone) and both tone). Two patients had a poor performance s tatus (ECOG > 1) and B-symptoms. Five patients (36%) were in stage IV, two of them because of bone marrow infiltration. All patients had a normal seru m LDH level, and 5 had high beta(2)-microglobulin level. The treatment cons isted in surgical resection (2 patients), surgery and radiotherapy tone), s urgery and chemotherapy (two), chemotherapy and radiotherapy (two) and chem otherapy alone (7 patients, three of them with doxorubicin-containing regim ens). Twelve patients were evaluable for response. Complete response, parti al response and failure rates were 75, 17 and 8%, respectively. Two of the 11 responders progressed, one of them with advanced stage disease. The actu arial 4-year disease-free survival was 77% (CI 95%: 47-100%). After a media n follow-up of 3.4 years, 100% of the patients were alive. CONCLUSION: Non-gastric MALT lymphomas may be associated with autoimmune di sorders, may present as disseminated disease and have a very good outcome.