J Res Clin Med. 2020;8(1): 18.
doi: 10.34172/jrcm.2020.18
  Abstract View: 253
  PDF Download: 149

Case Report

Isolated intracranial Rosai-Dorfman disease in an adult man: Report of a rare case

Somaye Rezaei 1 ORCID logo, Fariba Zarzanalivan 1, Pouya Pirouti 2, Mohammad Reza Amiri Nikpour 1, Abdolreza Javadi 3*, Shahram Torkamandi 4,5* ORCID logo

1 Department of Neurology, Imam Khomeini Hospital, Urmia University of Medical Sciences, Urmia, Iran
2 Islamic Azad University of Urmia, Urmia, Iran
3 Department of Pathology, Imam Hossein Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
4 Neurophysiology Research Center, Urmia University of Medical Sciences, Urmia, Iran
5 Department of Immunology and Medical Genetics, Faculty of Medicine, Urmia University of Medical Sciences, Urmia, Iran
Email: torkamandi.shahram@yahoo.co.uk torkamandi.shahram@yahoo.co.uk


Background: Isolated intracranial Rosai-Dorfman disease (RDD) is an extremely rare, idiopathic histo-proliferative disorder. RDD is associated with the proliferation of histiocytes and emperipolesis.

Case Presentation: we report a case with isolated intracranial RDD. A 47-year- old man presented with a dizziness, falling, and then secondary generalized seizure, hemiparesis and right hemisensory deficit. This case preoperatively was misdiagnosed with meningioma. Histopathological examination revealed pale histiocytes displaying emperipolesis which were positive for S-100 and CD68 proteins and negative for CD1a marker. BRAF V600E mutation was negative.

Conclusion: In this case, total resection was performed and clinical symptoms were regressed completely.

Keywords: Rosai-Dorfman Disease, Intracranial, Emperipolesis, Menangioma, Histo-proliferative
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Submitted: 04 Apr 2020
Accepted: 28 Apr 2020
ePublished: 16 May 2020
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