Lacrimal Gland Extranodal Marginal Zone B-cell Lymphoma of MALT-type

Purpose: To evaluate the clinical features and outcome of extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue type (MALT lymphoma) in the lacrimal gland. Design: Retrospective, noncomparative, observational case series in an academic referral setting. Methods: A consecutive series of 13 histologically verified MALT lymphoma in the lacrimal gland at presentation was studied. Clinical characteristics, treatment, and prognosis were analyzed. Results: Eight males and five females with a median age of 64 years and a median follow-up time of 48 months were included. All patients had no prior lymphoma and initially presented as MALT lymphoma in the lacrimal gland. Extraorbital involvement at diagnosis was noted in six patients (46.2%). Two patients had autoimmune disease, and both had Stage IV disease at presentation. Treatment consisted of surgical resection in one patient, radiotherapy in four, chemotherapy in four, and combined radiotherapy and chemotherapy in four. Complete remissions were obtained in eight patients (61.5%). Patients with bilateral disease (61.5%) had a higher rate of advanced-stage disease and a poor outcome. Recurrence was noted in two patients. At the last follow-up, eight patients were free of disease, three were alive with disease, one died of sepsis as a complication of chemotherapy, and one died of lymphoma. Conclusions: MALT lymphoma in the lacrimal gland has a high rate of extraorbital involvement and synchronous bilateral lacrimal gland involvement at presentation. The prognosis is relatively poor, especially in patients with advanced disease and bilateral involvement. Extensive staging and long-term follow-up are warranted for these patients.