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