Primary Dural Lymphomas: A Review

Fabio M. Iwamoto, MD; Lauren E. Abrey, MD

Disclosures

Neurosurg Focus. 2006;21(5) 

In This Article

Abstract and Introduction

Abstract

Primary dural lymphoma (PDL) is a rare subtype of primary central nervous system lymphoma (PCNSL) that arises from the dura mater and differs biologically from other CNS lymphomas. The PDL is usually a low-grade marginal zone lymphoma (MZL), whereas other types of PCNSLs are usually high-grade, diffuse, large B-cell lymphomas. A PDL usually presents with single or multiple extraaxial masses that enhance diffusely with addition of contrast material and can be confused with meningioma. The MZLs respond well to local therapy such as surgery and radiation treatment. Most patients attain complete response and have good local disease control. Nevertheless, the risk of systemic relapse appears to be high, probably because the dura mater is outside of the blood–brain barrier.

Introduction

Primary CNS lymphoma is an extranodal non-Hodgkin lymphoma that arises from the brain parenchyma, eyes, meninges, or spinal cord in the absence of systemic disease.[4] The most common type of PCNSL presents as a space-occupying lesion in the brain parenchyma. Lymphoma arising primarily from the meninges without brain or systemic involvement is rare. Primary leptomeningeal lymphoma arises within the leptomeningeal space and usually presents with headache, meningeal signs, and cranial nerve involvement.[25] Primary dural lymphoma arises from the dura mater and differs biologically from other CNS lymphomas.[9,24] It is usually a low-grade B-cell MZL, whereas other types of PCNSL are usually high-grade, diffuse, large B-cell lymphomas. Marginal zone lymphoma is an indolent non-Hodgkin lymphoma that occurs more frequently in the gastrointestinal tract, where it has been called the "mucosa-associated lymphoid tissue (MALT) lymphoma."[17] This specific pathological subtype of the non-Hodgkin lymphoma has important therapeutic and prognostic implications, making PDL a distinct subtype of PCNSL.

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