Radiation therapy in primary orbital lymphoma: a single institution retrospective analysis

Luigi De Cicco,Giulio Bonavolontà,Raffaele Liuzzi,Marco Salvatore,Fausto Tranfa,Manuel Conson,Raffaele Solla,Laura Cella,Giorgio Punzo,Diego Strianese,Antonio Farella,Roberto Pacelli

doi:10.1186/1748-717x-4-60

Abstract

BackgroundPrimary orbital lymphoma is a rare disease that accounts for 10% of all orbital tumors. Radiotherapy on the orbital cavity is the treatment of choice for this unusual presentation of localized non-Hodgkin's lymphoma (NHL). The aim of this study is to retrospectively evaluate the effectiveness and the toxicity of radiation treatment in patients with primary orbital lymphoma.MethodsForty-seven consecutive patients having primary orbital lymphoma treated in our department between May 1983 and September 2006 were investigated in a retrospective study. Either 60Co γ rays or 6 MV X rays were used to deliver daily fractions of 1.8 or 2.0 Gy, 5 times/week, with total doses ranging from 34.2 to 50 Gy. Forty-three patients had stage IE, three had stage II and one stage IV disease. Thirty-eight patients had marginal zone B-cell lymphoma, 5 diffuse large B cell lymphoma, 3 mantle cell lymphoma and 1 Burkitt lymphoma. Local control (LC), disease free survival (DFS), overall survival (OS) and late side effects were evaluated in all patients.ResultsWith a median follow up of 45 months, LC was obtained in 100% of patients. The estimated 5- and 7-year DFS rates were 75.8% and 55.3%, and the 5- and 7-year OS rates were 88.7% and 79.9% respectively. Acute toxicity was minimal. Late toxicity such as cataract, keratitis, retinopathy and xerophthalmia occurred respectively in 12 (25.5%), 5 (10.6%), 1 (2.1%), and 9 (19.1%) patients.ConclusionRadiotherapy is an effective and at the same time well tolerated treatment for primary orbital lymphoma.

Highlights

Primary orbital lymphoma is a rare disease that accounts for 10% of all orbital tumors
The orbit is a rare site of presentation of non-Hodgkin's lymphoma (NHL)
The vast majority (38 out of 47) of patients had extranodal marginal zone B-cell lymphoma of mucosa-associated limphoid tissue (MALT) type NHL according to REAL classification [27]

Summary

Introduction

Primary orbital lymphoma is a rare disease that accounts for 10% of all orbital tumors. Radiotherapy on the orbital cavity is the treatment of choice for this unusual presentation of localized non-Hodgkin's lymphoma (NHL). The aim of this study is to retrospectively evaluate the effectiveness and the toxicity of radiation treatment in patients with primary orbital lymphoma. The orbit is a rare site of presentation of non-Hodgkin's lymphoma (NHL). Primary orbit lymphoma (POL) represents 1% of all NHLs and 8% of extranodal NHLs [1]. The most frequent histology of POL is indolent NHL such as extranodal marginal B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) [5,6,7,8]. For patients with advanced disease, sequential chemo-radiation treatment is preferred [8]

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