[The clinical features and outcomes in 21 patients with ocular adnexal mucosa-associated lymphoid tissue lymphoma].

OBJECTIVE: To evaluate the clinical features, therapies and prognosis in patients with mucosa-associated lymphoid tissue (MALT) lymphoma in ocular adnexal marginal zone (OAML).

METHODS: A retrospective analysis was made upon clinical data from 21 patients with OAML admitted into Beijing Tongren Hospital from June, 2008 to December, 2011.

RESULTS: There were 12 (57.1%) men and 9 (42.9%) women, with a median age of 57 (23 - 79) years old. Majority of patients had localized pathological changes. Among them, 16 patients (76.2%) were in stage IE, and 5 (23.8%) in stage IVE. Surgical resection as the sole treatment was performed in 13 patients (61.9%), and positron emission tomography CT(PET-CT) imaging demonstrated normal fluorine 18-fluorodeoxyglucose (FDG) uptake after surgical resection, who were managed with no further therapy. All the 13 patients were followed up for median 14 (5 - 38) months, and all in complete remission. Combination chemotherapy was given to 8 (38.1%) patients. Three patients in stage IE treated with COP (cyclophosphamide, vincristine and prednisone) or CHOP (cyclophosphamide, adriamycin, vincristine and prednisone) were all in partial remission. Five patients in stage IVE were treated with COP/CHOP in combination with rituximab, and all in complete remission. The 3-year overall survival rate and disease-free survival rate in the total patients were 100.0% and 74.9% respectively.

CONCLUSIONS: The patients with OAML generally have localized disease, show indolent clinical course, and present low lymphoma-related mortality. Surgical resection is a very important treatment in the patients with local disease. Systemic chemotherapy should be considered in patients at advanced stages. Rituximab in combination with chemotherapy can improve the remission rate.