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

Christopher S. Hong, Robert M. Starke, Michael A. Hays, James W. Mandell, David Schiff, Ashok R. Asthagiri

Research output: Contribution to journalArticlepeer-review

12 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)702.e13-702.E20
JournalWorld neurosurgery
Volume90
DOIs
StatePublished - Jun 1 2016
Externally publishedYes

Keywords

  • Cerebral
  • Inflammatory sinus histiocytosis
  • Intracranial
  • Rosai-Dorfman

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

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