Long segment Rosai-Dorfman disease-causing spinal cord compression: A case report
Rabiul Karim1, Mohammad Majed Sultan1, Kamal Hossain1
1Neurosurgery Department, Chittagong Medical College Hospital.
International Journal of Surgery Case Reports
|February 8, 2022
Summary
This case report details a rare, extensive spinal Rosai-Dorfman disease (RDD) in a male patient. Surgical intervention led to significant motor improvement, highlighting RDD
Area of Science:
- Neurology
- Oncology
- Pathology
Background:
- Rosai-Dorfman disease (RDD) is a rare histiocytic lymphoproliferative disorder.
- Extranodal central nervous system involvement is exceptionally rare.
- Isolated spinal RDD is sporadic, presenting diagnostic and therapeutic challenges.
Purpose of the Study:
- To report a rare case of extensive isolated spinal Rosai-Dorfman disease.
- To review literature on spinal RDD and its management.
- To highlight the effectiveness of surgical intervention in improving neurological deficits.
Main Methods:
- Case report of a 33-year-old male with progressive quadriparesis.
- Magnetic resonance imaging (MRI) revealed a C4-D6 epidural lesion.
- Diagnosis confirmed via core biopsy, histopathology, and immunocytohistochemistry (S100, CD68).
- Surgical resection and laminoplasty performed.
Main Results:
- Histopathology confirmed Rosai-Dorfman disease with characteristic emperipolesis.
- Patient experienced a smooth postoperative recovery.
- Marked improvement in motor function observed post-surgery.
Conclusions:
- This case represents one of the longest segmental lesions in isolated spinal Rosai-Dorfman disease.
- Surgical management, including resection and laminoplasty, yielded remarkable neurological improvement.
- Spinal RDD, though rare, is treatable with surgical intervention.
