ROSAI-DORFMAN DISEASE CAUSING SPINAL CORD COMPRESSION

Abstract

Rosai-Dorfman disease is a rare idiopathic, histiocytic, proliferative disease characterized by massive, painless cervical lymphadenopathy. Extranodal involvement is rare and central nervous system involvement is unusual. We present a patient with Rosai-Dorfman disease with spinal cord compression. Very few cases have been reported in the literature. A 17-year-old man presented with a 1-month history of progressive fatigue of the legs. His medical history was significant for Rosai-Dorfman disease diagnosed 7 months earlier. Clinical examination was consistent with a pyramidal syndrome and proprioceptive disturbances on his lower limbs without sensory level. A magnetic resonance imaging scan revealed an intradural extramedullary space-occupying lesion at the T1-T4 level with dural insertion and spinal cord compression. A T1-T4 laminotomy was performed. Upon opening the dura, a reddish-gray mass was encountered, which encased the dorsal and lateral arachnoidal membrane. The lesion was relatively well circumscribed and was easily dissected from the underlying arachnoid. Pathological examination of the compressive soft tissue was consistent with Rosai-Dorfman disease. Postoperatively, the patient showed substantial improvement in neurological function. He was followed for 18 months with no complaints and no recurrence. Neurosurgeons should consider this rare etiology of spinal cord compression. They must be aware that this lesion can occur in front of an intraspinal lesion, mimic meningiomas, occur in young people, and can potentially be associated with other locations of disease, including intracranial lesions. Surgery is the treatment of choice.