Long segment Rosai-Dorfman disease-causing spinal cord compression: A case report.

INTRODUCTION AND IMPORTANCE

Rosai-Dorfman disease is a rare, histiocytic lymphoproliferative disease of unknown etiology. It manifests mainly as painless cervical lymphadenopathy, with very few cases reported extranodal involvement in the central nervous system. Isolated spinal Rosai-Dorfman disease is sporadic.

CASE PRESENTATION

This case report documents a rare instance of an isolated long-segment spinal Rosai-Dorfman disease (C4-D6) along with the review of relevant literature. A 33-year male presented with progressive quadriparesis and urinary retention. A magnetic resonance scan (MRI) revealed a long segment epidural lesion from C4-D6 levels that led to the displacement of the cord. A core biopsy of the spinal tumor revealed characteristic histiocytic emperipolesis and confirmational immunocytohistochemistry markers, confirming the diagnosis. Surgical resection and laminoplasty were performed.

CLINICAL DISCUSSION

The following histopathology and immunocytohistochemistry findings showed the presence of histiocytes positive for S100 and CD68 positive. Therefore, it was diagnosed to be a case of Rosai-Dorfman disease. The patient had a smooth postoperative recovery and displayed marked motor improvement in the ensuing days. This is a rare case that posed an intriguing challenge to approach.

CONCLUSION

To our knowledge, we have encountered one of the most prolonged segmental lesions in isolated spinal Rosai-Dorfman diseases, where surgical management (surgical resection and laminoplasty) has proven to bring about remarkable improvement.