Intracranial manifestations of adult Rosai-Dorfman disease: a systematic review and IPD meta-analysis of 327 cases - Takeaways - MDSpire

Intracranial manifestations of adult Rosai-Dorfman disease: a systematic review and IPD meta-analysis of 327 cases

  • By

  • Daniela A. Perez-Chadid

  • Aafreen Azmi

  • Jeremiah H. Wijaya

  • Temitope Oshinowo

  • Juan P. Avila-Madrigal

  • Aditi S. Gorthy

  • Sri Sai Lakshman Akkineni

  • Andrew Egladyous

  • Nemanja Novakovic

  • Morana Vojnic

  • Jonathan H. Sherman

  • Anil Nanda

  • December 6, 2025

  • 0 min

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  • 1

    Rosai-Dorfman Disease (RDD) is a rare histiocytic disorder with an estimated prevalence of 1:200,000, affecting adults and presenting with CNS manifestations in 5% of cases.

  • 2

    Intracranial RDD typically appears as a dural-based mass, often misdiagnosed as meningioma, with symptoms including headaches, seizures, and focal neurologic deficits.

  • 3

    Diagnosis of intracranial RDD relies on histopathology, revealing large histiocytes that are S100 and CD68 positive, with characteristic emperipolesis.

  • 4

    Management of intracranial RDD is not standardized; surgical resection is common, but adjuvant therapies like corticosteroids and radiotherapy may be used for multifocal cases.

  • 5

    This systematic review aims to clarify the understanding of adult intracranial RDD by synthesizing data on epidemiology, imaging, treatment strategies, and clinical outcomes.

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