Rosai–Dorfman Disease of the Central Nervous System with a Rare Presentation as an Arachnoid Cyst

Rosai–Dorfman disease (RDD) is an uncommon histiocytic disorder presenting with painless massive lymphadenopathy. Extranodal RDD is seen in up to 30% of cases and commonly involves the skin and bone. Central nervous system (CNS) involvement by RDD is very rare, occurring in <5% of cases. In the C...

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Main Authors: Sunayana Misra, Shashi Dhawan, Seema Rao
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2024-07-01
Series:CHRISMED Journal of Health and Research
Subjects:
Online Access:https://journals.lww.com/10.4103/cjhr.cjhr_72_24
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author Sunayana Misra
Shashi Dhawan
Seema Rao
author_facet Sunayana Misra
Shashi Dhawan
Seema Rao
author_sort Sunayana Misra
collection DOAJ
description Rosai–Dorfman disease (RDD) is an uncommon histiocytic disorder presenting with painless massive lymphadenopathy. Extranodal RDD is seen in up to 30% of cases and commonly involves the skin and bone. Central nervous system (CNS) involvement by RDD is very rare, occurring in <5% of cases. In the CNS, RDD is usually seen as dural-based lesion mimicking meningiomas or as intraparenchymal solid lesions. The presentation of RDD in a posterior fossa arachnoid cyst has not been previously reported. To the best of our knowledge, we report the first case of isolated extranodal RDD in the CNS presenting in the wall of a retrocerebellar arachnoid cyst.
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spelling doaj-art-a43c70cd90c34c2aaa59861ff85697f22025-08-20T02:16:02ZengWolters Kluwer Medknow PublicationsCHRISMED Journal of Health and Research2348-33342348-506X2024-07-0111315916110.4103/cjhr.cjhr_72_24Rosai–Dorfman Disease of the Central Nervous System with a Rare Presentation as an Arachnoid CystSunayana MisraShashi DhawanSeema RaoRosai–Dorfman disease (RDD) is an uncommon histiocytic disorder presenting with painless massive lymphadenopathy. Extranodal RDD is seen in up to 30% of cases and commonly involves the skin and bone. Central nervous system (CNS) involvement by RDD is very rare, occurring in <5% of cases. In the CNS, RDD is usually seen as dural-based lesion mimicking meningiomas or as intraparenchymal solid lesions. The presentation of RDD in a posterior fossa arachnoid cyst has not been previously reported. To the best of our knowledge, we report the first case of isolated extranodal RDD in the CNS presenting in the wall of a retrocerebellar arachnoid cyst.https://journals.lww.com/10.4103/cjhr.cjhr_72_24arachnoid cystcentral nervous systemhistiocytosisrosai–dorfman disease
spellingShingle Sunayana Misra
Shashi Dhawan
Seema Rao
Rosai–Dorfman Disease of the Central Nervous System with a Rare Presentation as an Arachnoid Cyst
CHRISMED Journal of Health and Research
arachnoid cyst
central nervous system
histiocytosis
rosai–dorfman disease
title Rosai–Dorfman Disease of the Central Nervous System with a Rare Presentation as an Arachnoid Cyst
title_full Rosai–Dorfman Disease of the Central Nervous System with a Rare Presentation as an Arachnoid Cyst
title_fullStr Rosai–Dorfman Disease of the Central Nervous System with a Rare Presentation as an Arachnoid Cyst
title_full_unstemmed Rosai–Dorfman Disease of the Central Nervous System with a Rare Presentation as an Arachnoid Cyst
title_short Rosai–Dorfman Disease of the Central Nervous System with a Rare Presentation as an Arachnoid Cyst
title_sort rosai dorfman disease of the central nervous system with a rare presentation as an arachnoid cyst
topic arachnoid cyst
central nervous system
histiocytosis
rosai–dorfman disease
url https://journals.lww.com/10.4103/cjhr.cjhr_72_24
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AT shashidhawan rosaidorfmandiseaseofthecentralnervoussystemwithararepresentationasanarachnoidcyst
AT seemarao rosaidorfmandiseaseofthecentralnervoussystemwithararepresentationasanarachnoidcyst