Primary CNS T-Cell Lymphoma of the Spinal Cord: Case Report and Literature Review

Melissa Guzzetta, DO; Steven Drexler, MD; Brian Buonocore, MD; Virginia Donovan, MD

Disclosures

Lab Med. 2015;46(2):159-163. 

In This Article

Abstract and Introduction

Abstract

Primary central nervous system lymphoma (PCNSL) accounts for 1% of all lymphoma diagnoses and as many as 6% of all central nervous system (CNS) tumors. Most cases of PCNSL are of B-cell type; few are of T-cell lineage. PCNSL mainly occurs intracranially; primary spinal-cord lymphoma only occurs rarely. Moreover, intramedullary presentation without intracranial lesions is virtually unknown. Herein, we present a case of primary T-cell CNS lymphoma limited to the intramedullary spinal cord in an 82-year-old white man, along with a review of the literature on this condition and similar conditions.

Introduction

Primary central nervous system lymphoma (PCNSL) accounts for only 1% of all lymphoma diagnoses and between 0.8% to 6.6% of all central nervous system (CNS) neoplasms.[1] Most CNS tumors involve the supratentorial space (60%); in these tumors, the posterior fossa (13%) is less commonly involved and the spinal cord (1%) is rarely involved.[1,2] Moreover, spinal cord neoplasms are generally uncommon. These lesions are classified as intramedullary (occurring within the spinal cord parenchyma) or extramedullary (occurring outside the spinal cord). Intramedullary spinal tumors account for 5% to 10% of spinal tumors, most of which are glial in origin; an extremely small percentage of these are lymphomas.[3] This finding contrasts with that of spinal involvement in systemic lymphoma, in which diffuse leptomeningeal involvement and extradural nodules are usually present.[4] Most lymphoma diagnoses are of B-cell lineage and are seldom of T-cell lineage. There are only a few reported cases of T-cell PCNSL.[1,5] For instance, approximately 20 years ago, Memorial Sloan Kettering Cancer Center in New York City reported 200 cases of PCNSL, of which only 2 were of T-cell origin.[6]

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