Oral chlorambucil for extranodal, marginal zone, B-cell lymphoma of mucosa-associated lymphoid tissue of the orbit

Purpose: To report the outcome of oral chlorambucil as a single treatment in patients with orbital mucosa-associated lymphoid tissue (MALT) lymphoma. Design: Retrospective nonrandomized clinical study. Participants: Thirty-three patients with isolated orbital MALT lymphoma. Methods: Medical records of all patients with histology-verified orbital MALT lymphoma treated with oral chlorambucil at the Royal Melbourne Hospital were reviewed. Main Outcome Measures: Complete clinical response, partial response, local relapse, systemic extension (distant relapse), and survival. Results: Thirty-three patients (19 female; mean age, 69 years) participated in the study. Patients received an average of 4 courses of oral chlorambucil with a mean total dose of 600 mg. The lacrimal gland was the most frequent site of occurrence (24%), followed by the conjunctiva, eyelid, and superior orbit. Orbital mass, swelling, and diplopia were common presenting signs. Complete response was noted in 26 patients (79%). In 2 of the patients with complete clinical response, mild residual thickening was noted on follow-up orbital imaging studies. Four patients (12%) showed disease recurrence or relapse. Mean follow-up time was 32 (+/- 20) months (range, 8 months-6 years; median, 26 months). None of the patients developed granulocytopenia secondary to chemotherapy, and none suffered significant nausea or vomiting. One patient with malignant transformation died 12 months after diagnosis and initial treatment. Conclusions: Systemic chemotherapy with chlorambucil is a reasonable option in patients with orbital MALT lymphoma. it is associated with minimal to no side effects. Additionally, it may be well tolerated by elderly patients and also may treat subclinical disease elsewhere.