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CC BY-NC-ND 4.0 · Asian J Neurosurg 2024; 19(02): 312-316
DOI: 10.1055/s-0044-1787089
Case Report

Fourth Ventricular Rosai–Dorfman Disease Mimicking Intraventricular Tumor in Young Adult: A Rare Case Report

Meghana V. Chougule 
1   Department of Neuropathology, Shanti Pathology Laboratory Cancer Diagnosis & Research Center, Kolhapur, Maharashtra, India
,
Shankaran Prabhakar
2   Department of Neurosurgery, Gangamai Hospital, Solapur, Maharashtra, India
,
Nikhil Tadwalkar
3   Department of Neuropathology, Gangamai Hospital, Solapur, Maharashtra, India
,
Atul Ranade
1   Department of Neuropathology, Shanti Pathology Laboratory Cancer Diagnosis & Research Center, Kolhapur, Maharashtra, India
› Author Affiliations Funding None.
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Abstract

Rosai–Dorfman disease (RDD) or sinus histiocytosis with massive lymphadenopathy can present with or without systemic disease. It is a benign histioproliferative disorder characterized by generalized lymphadenopathy, weakness, anemia, and rarely extranodal involvement. While RDD most commonly affects lymph nodes, extranodal involvement of multiple organs has been reported, including the central nervous system (CNS). However, CNS involvement in RDD is rare and is not well characterized. Isolated involvement of the fourth ventricle is even rarer. Such lesions may be mistaken for intraventricular tumors such as ependymoma or medulloblastoma. This report highlights the necessity to consider RDD as a differential diagnosis in case of intraventricular space-occupying lesion.

Keywords

Rosai–Dorfman disease - fourth intraventricular tumors - extranodal - lympho-histiocytic diseases - emperipolesis

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The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patient understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.




Publication History

Article published online:
27 May 2024

© 2024. Asian Congress of Neurological Surgeons. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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