Redefining the Prevalence of Dural Involvement in Rosai-Dorfman Disease of the Central Nervous System.

Abstract:

BACKGROUND:Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease, is a rare condition, classically characterized by painless, massive cervical lymphadenopathy. Histologically, the pathognomonic findings include a dense, mixed inflammatory infiltrate with areas of emperipolesis. Albeit infrequent, when Rosai-Dorfman disease affects the central nervous system, it typically manifests as an isolated dural lesion, often mimicking a meningioma. A purely intraparenchymal manifestation of Rosai-Dorfman disease of the brain and spine with absent dural involvement is exceedingly rare. CASE DESCRIPTION:In this report, we describe a 59-year-old woman who underwent surgical excision of an intraparenchymal cerebellar lesion. Histologic analysis of the resected specimen diagnosed isolated Rosai-Dorfman disease with absent systemic involvement. We also provide an updated review of the literature of nondural-based Rosai-Dorfman disease in the central nervous system. CONCLUSIONS:With the recent increase of such reported cases, it becomes imperative that Rosai-Dorfman be considered more than as a dural lesion that may mimic meningioma. Diagnostic and therapeutic challenges surrounding this disease entity are also discussed.

journal_name

World Neurosurg

journal_title

World neurosurgery

authors

Hong CS,Starke RM,Hays MA,Mandell JW,Schiff D,Asthagiri AR

doi

10.1016/j.wneu.2016.02.121

subject

Has Abstract

pub_date

2016-06-01 00:00:00

pages

702.e13-702.e20

eissn

1878-8750

issn

1878-8769

pii

S1878-8750(16)00369-7

journal_volume

90

pub_type

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