RESUMO
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.