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

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

JOURNAL=Frontiers in Neurology

VOLUME=9

YEAR=2018

URL=https://www.frontiersin.org/journals/neurology/articles/10.3389/fneur.2018.00267

DOI=10.3389/fneur.2018.00267

ISSN=1664-2295

ABSTRACT=<sec id="ST1"><title>Background</title><p>Primary extranodal marginal zone lymphoma (MZL) of the dura is a rare neoplastic entity in the central nervous system (CNS).</p></sec><sec id="ST2"><title>Methods</title><p>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).</p></sec><sec id="ST3"><title>Results</title><p>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.</p></sec><sec id="ST4"><title>Conclusion</title><p>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.</p></sec>