A Rare Case of Composite Dural Extranodal Marginal Zone Lymphoma and Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma.

Mark Bustoros,Cynthia Liu,Benjamin Liechty,Deborah Gruber,Timothy Shepherd,Bruce Raphael,David Zagzag,Dimitris G Placantonakis

doi:10.3389/fneur.2018.00267

Abstract

Primary extranodal marginal zone lymphoma (MZL) of the dura is a rare neoplastic entity in the central nervous system (CNS). We used literature searches to identify previously reported cases of primary dural MZL. We also reviewed clinical, pathologic, and radiographic data of an adult patient with concurrent dural MZL and chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL). We identified 104 cases of dural MZL in the literature. None of them presented concurrently with another type of non-Hodgkin lymphoma. This is the first report of composite lymphoma consisting of dural MZL and CLL/SLL in the bone marrow and lymph nodes. Primary dural MZL is a rare, indolent low-grade CNS lymphoma, with a relatively good prognosis. Its treatment is multidisciplinary and often requires surgical intervention due to brain compression, along with low to moderate doses of radiotherapy and/or systemic chemotherapy.

Highlights

Primary central nervous system (CNS) lymphomas account for 3–4% of all CNS malignancies [1,2,3]
We review the literature on dural Marginal zone lymphoma (MZL) and present clinical, pathologic, and radiographic data of an adult male with newly diagnosed primary dural MZL and simultaneous chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL)
The following immunohistochemical stains were performed on the brain specimen: CD3, CD20, CD21, CD10, CD79a, CD138, Bcl2, Bcl6, MUM-1, Ki-67, in situ hybridization studies for kappa, lambda light chains, and EBER; Immunohistochemistry on the bone marrow biopsy included staining for CD3, CD20, CD5, CD23 and CyclinD1

Summary

Introduction

Primary central nervous system (CNS) lymphomas account for 3–4% of all CNS malignancies [1,2,3]. Marginal zone lymphoma (MZL) is a low-grade non-Hodgkin’s lymphoma (NHL), which, according to the WHO classification, is subdivided into three types: extranodal ( known as mucosa-associated lymphoid tissue MZL), nodal, and splenic [4]. Extranodal MZL is the most common variant. It constitutes around 8% of NHL [5], an increase of 3% compared with prior estimates [4]. Extranodal MZL of the gastrointestinal tract was first described by Isaacson and Wright as an indolent low-grade lymphoma [6]. Primary extranodal marginal zone lymphoma (MZL) of the dura is a rare neoplastic entity in the central nervous system (CNS)

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Conclusion