Rosai-Dorfman Disease of Rare Isolated Spinal Involvement: Report of 4 Cases and Literature Review

Background Rosai-Dorfman disease (RDD) is a rare histioproliferative disorder that only occasionally involves the central nervous system (CNS). Case Description The diagnosis and treatment of 4 patients with isolated spinal RDD are discussed. All 4 patients were treated by total or subtotal surgical...

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Bibliographic Details
Published in:World neurosurgery 2016, Vol.85, p.367.e11-367.e16
Main Authors: Huang, Bo Yuan, Zhang, Hua, Zong, Wen Jing, Sun, Yan Hui
Format: Article
Language:English
Subjects:
Adolescent
Adult
Cervical Vertebrae
Diagnosis, Differential
Dura Mater
Female
Histiocytes - chemistry
Histiocytosis, Sinus - diagnosis
Histiocytosis, Sinus - pathology
Histiocytosis, Sinus - surgery
Humans
Isolated spinal involvement
Lumbar Vertebrae
Male
Meningeal Neoplasms - diagnosis
Meningioma - diagnosis
Middle Aged
Neurosurgery
Neurosurgical Procedures - methods
Rosai-Dorfman disease
S100 Proteins - analysis
Spinal Cord - pathology
Spinal Cord - surgery
Thoracic Vertebrae
Treatment Outcome
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Summary:Background Rosai-Dorfman disease (RDD) is a rare histioproliferative disorder that only occasionally involves the central nervous system (CNS). Case Description The diagnosis and treatment of 4 patients with isolated spinal RDD are discussed. All 4 patients were treated by total or subtotal surgical resection and none of them experienced recurrence. Histopathologic examination showed a characteristic emperipolesis, and the lymphocytes were engulfed in the S-100-protein-positive histiocytes with no expression of CD1a. Conclusions 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 therapy, and chemotherapy have not shown reliable therapeutic efficiency.
ISSN:1878-8750
1878-8769
DOI:10.1016/j.wneu.2015.09.097