Isolated cerebellar Rosai-Dorfman granuloma mimicking Lhermitte-Duclos disease

Case report

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Rosai-Dorfman disease (RDD) is an idiopathic histioproliferative disorder that rarely involves the CNS. Rosai-Dorfman disease is exceedingly rare in the pediatric population and has never been observed in the cerebellum of a child. The authors present the case of a 14-year-old male with a cerebellar lesion having radiographic characteristics of Lhermitte-Duclos disease. After a period of observation with a presumptive diagnosis of Lhermitte-Duclos disease, the child underwent suboccipital craniotomy and resection of the lesion due to continuous suboccipital headaches. Histological examination of the tissue demonstrated RDD. The published literature on RDD is reviewed with an emphasis on differential diagnosis.

Abbreviations used in this paper: LDD = Lhermitte-Duclos disease; RDD = Rosai-Dorfman disease.
Article Information

Contributor Notes

Address correspondence to: Tae Sung Park, M.D., Department of Neurosurgery, St. Louis Children's Hospital, One Children's Place, Suite 4S20, St. Louis, Missouri 63110. email: park@nsurg.wustl.edu.

© AANS, except where prohibited by US copyright law.

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