Efficacy of chemotherapy as a first-line treatment in ocular adnexal extranodal marginal zone B-cell lymphoma

Background: Radiotherapy is commonly used as a first-line treatment for localized ocular adnexal extranodal marginal zone B-cell lymphoma (EMZBL), despite its ophthalmologic complications. This study was undertaken to analyze the efficacy of first-line chemotherapy in treating EMZBL. Chemotherapy was followed by radiotherapy only in recurrent cases. Patients and methods: Twenty-one patients with histologically confirmed EMZBL were treated with combination of cyclophosphamide, vincristine, and prednisolone (CVP). Radiotherapy was given to CVP failure cases. Results: CVP alone resulted in overall response rate of 100% [complete remission (CR), 76.2%]. After a median follow-up of 58 months, 14 (66.7%) of 21 cases were disease free with CVP alone, while seven cases showed disease progression, including two extra-orbital and five local failures. Radiotherapy was delivered to five local failure cases, who subsequently achieved CR with late ophthalmologic complications. There were tolerable adverse events associated with CVP. Conclusions: Front-line CVP, in conjunction with radiotherapy in recurrent cases, is effective and well tolerated in patients with localized ocular adnexal EMZBL.