Isolated Intramedullary Spinal Rosai-Dorfman Disease: A Case Report and Literature Review.

Isolated Intramedullary Spinal Rosai-Dorfman Disease: A Case Report and Literature Review.

World Neurosurg. 2015 Dec 31;

Authors: Huang BY, Liu HL, Yu CJ

Abstract
Rosai-Dorfman disease (RDD) is a rare histioproliferative disorder that only occasionally involves the central nervous system (CNS). We presented the diagnosis and treatment of an exceedingly rare case of isolated intramedullary spinal RDD which was previously reported for only 3 times. Moreover, it was for the first time that intramedullary spinal RDD was described in child. The patient was treated by total surgical resection and experienced no recurrence during the 12-months follow-up. Histopathological examination showed a characteristic emperipolesis, the lymphocytes were engulfed in the S-100-protein-positive histiocytes with negative expression of CD1a. Preoperative diagnosis of spinal RDD is still challenging because the lesion is usually a dura-based lesion that mimics a meningioma. Surgical resection is an effective treatment and radiotherapy, steroid and chemotherapy has not demonstrated reliable therapeutic efficiency.

PMID: 26748178 [PubMed - as supplied by publisher]

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