Primary high-grade ocular adnexal lymphoma: Clinicopathological characteristics and prognostic factors of a single-centre series

Abstract

The majority of ocular adnexal lymphomas (OAL) occurring in ophthalmologic sites are low-grade B-cell non-Hodgkin lymphomas, and about 15% of all cases are high-grade. Although numerous reports have already studied the features of low-grade OAL, high-grade OAL have been usually described as part of various published series of OAL without specifically focusing on these histopathologic subtypes of lymphoma. The aim of this study was therefore to retrospectively review a single-centre series of 15 patients with high-grade B-cell OAL treated at the Institut Curie. The main clinical and laboratory features of our selected patients, i.e., median age (66 years), PS greater than 1 (20%), stage IV (53%), bone marrow involvement (17%), more than one extranodal site (27%), and elevated serum LDH (14%), were not significantly different from those of nodal non-Hodgkin's lymphomas (NHL), except for a lower proportion of OAL patients with elevated LDH level. Similarly, the prognosis of these patients was not different, with a 5-year overall survival of 54%. When high-grade OAL were compared to a historical series of low-grade OAL, a lower rate of conjunctival involvement was observed, but both types presented a majority of orbital and/or lacrimal gland involvement as well as bilateral ophthalmological tumors. The recommended treatment for high-grade B-cell OAL can be the same as that for nodal CD20-positive NHL, namely a combination of rituximab and CHOP-like regimen.