Rosai-Dorfman disease presenting as a thoracic intradural extramedullary spinal tumor but without extraspinal manifestations

摘要

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, was first reported by Destombes in 1965 as adenitis with lipid excess. Four years later, it was more fully described by Rosai and Dorfman. It is traditionally characterized by massive lymphadenopathy, lymphocytosis, constitutional symptoms, and varying organ system involvement [1], including the skin, paranasal sinuses, soft tissues, bone, salivary glands, oral cavity, kidney, lower respiratory tract, larynx, and very rarely other locations. Central nervous system (CNS) involvement is extremely rare; to date, only 16 cases of spinal cord involvement have been described.