Bo Yuan Huang1, Hai Long Liu1, Chun Jiang Yu2. 1. Department of Neurosurgery, San Bo Brain Hospital, Capital Medical University, Beijing, China. 2. Department of Neurosurgery, San Bo Brain Hospital, Capital Medical University, Beijing, China. Electronic address: 13436561446@163.com.
Abstract
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.
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.
Authors: Yi Li; Emily Sloan; Andrew Bollen; David Solomon; Philip Theodosopoulos; Soonmee Cha Journal: Int J Hematol Oncol Stem Cell Res Date: 2021-10-01