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. 2017 Apr;58(4):882-888.
doi: 10.1080/10428194.2016.1218006. Epub 2016 Sep 21.

Marginal zone dural lymphoma: the Memorial Sloan Kettering Cancer Center and University of Miami experiences

Macarena I de la Fuente  1 , Aya Haggiagi  2 , Adrienne Moul  3   4 , Robert J Young  5 , Charif Sidani  6 , Arnold Markoe  7 , Francisco Vega  3 , Lisa M DeAngelis  2 , Izidore S Lossos  4   8
Affiliations

Marginal zone dural lymphoma: the Memorial Sloan Kettering Cancer Center and University of Miami experiences

Macarena I de la Fuente et al. Leuk Lymphoma. 2017 Apr.

Abstract

Dural lymphoma (DL) is a rare type of primary CNS lymphoma arising from the dura mater. The optimal treatment is uncertain. A retrospective review was performed on 26 DL patients. Seventeen patients underwent resection and nine had a biopsy. Twenty three patients could be assessed for a response to treatment after surgery. Thirteen received focal radiotherapy (RT), six whole brain RT (WBRT), three chemotherapy alone and one chemotherapy followed by WBRT. Twenty two achieved complete response (CR) and one a partial response (PR). Four patients relapsed (two local and two systemic). Median follow up was 64 months, with median progression free survival (PFS) and OS not reached. Three year PFS was 89% (95% CI 0.64-0.97). All patients are alive at last follow-up, demonstrating that DL is an indolent tumor with long survival. CR is achievable with focal therapy in the majority of cases, but there is a risk for relapses and long-term follow-up is recommended.

Keywords: Dural lymphoma; focal radiotherapy; marginal zone lymphoma; primary central nervous system lymphoma.

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Conflict of interest statement

Conflict of interest disclosure statement: The authors have nothing to disclose.

Figures

FIGURE 1
FIGURE 1. Axial post-gadolinium T1-weighted imaging of dural masses
(a) Brain MRI image showing a meningioma with avid enhancement including dural tail and characteristic hyperostosis of the overlying calvarium. (b) Brain MRI image showing a DL with lesser enhancement than the dural tail; it also shows an irregular medial border of DL which is atypical of meningioma. White arrows denote dural tails.
FIGURE 2
FIGURE 2. Marginal zone dural lymphoma, pathologic features
(a) Low power view of a dura biopsy demonstrating extensive hyalinization and fibrosis (H&E, 4×). (b) Atypical, monocytoid cells surrounding and infiltrating meningeal tissue (H&E, 20×) (c) Tumor abuts the brain parenchyma with a pushing border (large arrow). The blood vessel demonstrates prominent lymphocytic infiltrate within the Virchow-Robin space (thin arrow). (d) Higher power demonstrating that the neoplastic cells have a plasmacytic differentiation. (e) Atypical monocytoid cells with intervening blood vessels demonstrating extensive hyalinization (H&E, 10×). (f) Higher magnification of neoplastic monocytoid cells with surrounding blood vessels demonstrating extensive hyalinization (H&E, 40×).
FIGURE 3
FIGURE 3. Marginal zone dural lymphoma with plasmacytic differentiation, immunohistochemistry profile
(a) Brain parenchyma with vessel demonstrating prominent lymphocytic infiltrate within the Virchow-Robin space. (b) CD20 demonstrates few cells with expression. (c) CD5 highlights numerous T cells within Virchow-Robin space. (d) Lambda by in situ hybridization demonstrates clonal plasma cell differentiation. (e) Kappa by in situ hybridization is negative. (f) Ki67 proliferation index is less than 5%.
FIGURE 4
FIGURE 4. Progression free survival and overall survival for marginal zone dural lymphoma patients
(a) Progression free survival. (b) Overall survival. N: 23.
See this image and copyright information in PMC

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