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Nongastrointestinal Low-Grade Mucosa-Associated Lymphoid Tissue Lymphoma: Analysis of 75 Patients

From the Institute of Hematology and Medical Oncology "Seràgnoli," University of Bologna, Bologna; Hematology Division, University of Siena, Siena; Chair of Hematology "La Sapienza," Rome; Division of Pulmonary Medicine, Maggiore Hospital, Bologna; Chair of Hematology, University of Udine, Udine; Hematology Division, Catanzaro Hospital, Catanzaro; Hematology Division, Ravenna Hospital, Ravenna; Chair of Hematology "Tor Vergata," Rome; Oncology Division, Forlì Hospital, Forlì; and Hematology Unit, Cesena Hospital, Cesena, Italy.

Address reprint requests to Pier Luigi Zinzani, MD, Istituto di Ematologia e Oncologia Medica, Policlinico S. Orsola, Via Massarenti 9, 40138 Bologna, Italy; email seragnol{at}kaiser.alma.unibo.it

PURPOSE: Nongastrointestinal locations represent about 30% to 40% of all low-grade mucosa-associated lymphoid tissue (MALT) lymphomas. We report a retrospective analysis of 75 patients with nongastrointestinal low-grade MALT lymphoma, presenting their clinical, therapeutic, and follow-up data with respect to the initial location of the lymphoma.

PATIENTS AND METHODS: From January 1988 to October 1997, 75 patients with untreated nongastrointestinal low-grade MALT lymphoma were subjected to treatments ranging from local radiotherapy and local interferon alfa administration to chemotherapy. The lymphomas were located in the lung (19 patients), orbital soft tissue (16 patients), skin (seven patients), thyroid (seven patients), lachrymal gland (six patients), conjunctiva (six patients), salivary gland (six patients), breast (three patients), eyelid (two patients), larynx (one patient), bone marrow (one patient), and trachea (one patient).

RESULTS: Complete and partial remissions were achieved in 59 (79%) and 16 (21%) of the 75 patients, respectively, with an overall response rate of 100%. All but two of the patients are still alive, with a median follow-up of 47 months; these two patients died from other causes. The estimated time to treatment failure rate is 30% at 5 years. In the thyroid and lachrymal gland sites, no relapses were reported.

CONCLUSION: Our data regarding the largest reported series of nongastrointestinal MALT lymphomas confirm the good prognosis of this particular clinicopathologic entity and the significant efficacy of different therapeutic approaches to specific sites.

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