Blastic transformation of a mantle cell lymphoma presenting as an enlarging unilateral orbital mass

Abstract

Mantle cell lymphoma (MCL) is an uncommon form of non-Hodgkin lymphoma predominantly affecting male individuals of advanced age. Approximately 1-9% of cases of lymphoma affecting the orbital and ocular adnexal regions are attributed to MCL. We describe the case of a 65-year-old man with a 24-year history of MCL with initial remission followed by multiple relapses who presented with acute-onset binocular diplopia and proptosis of the left eye. Subsequent imaging demonstrated a new left superior orbital mass. Biopsy of the mass revealed two clonally related, yet distinct cellular components demonstrating the classical mantle cell morphology and large cells that appeared to have transformed to the blastoid variant of MCL. Transformation of classical MCL to the blastoid variant of MCL is rare, with few reports in the literature. The blastoid variant of MCL tends to be aggressive and associated with a poor prognosis. The case we describe represents perhaps the first report of MCL transformation observed in the orbit.