Isolated intradural Rosai-Dorfman disease of the spine: report of two cases

Abstract

Rosai-Dorfman disease (RDD) is an idiopathic histiocytic proliferative disorder characterized by sinus histiocytosis and massive lymphadenopathy. Extranodal and central nervous system (CNS) involvement is unusual. We describe two distinctly rare cases of isolated intradural RDD of the spine without cervical lymphadenopathy. We report our experience in the management of two patients with RDD. The first patient had two ventral intradural lesions, radiologically in favour of multiple spinal meningiomas, and the second patient presented an intradural extramedually lesion, extending into the right foramina, suggestive of a sacral perineural cyst. Treatments consisted of total excision and subtotal excision without steroids and radiotherapy, respectively. The definitive diagnosis was confirmed by histopathology and immunochemistry. RDD should be considered in the rare differential diagnosis of a spinal disease. Resection of the lesions is an effective treatment choice to relieve spinal compression symptoms. The efficacy of the adjuvant therapy to control the disease is unknown.