, Volume 154, Issue 2, pp 367-368
Date: 30 Sep 2011

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

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Dear editor,

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. The disease can have a protracted course lasting months or even years, often accompanied by phases of exacerbation and remission, with an overall mortality of 7% [3]. We describe here, to the best extent of our knowledge, the first case of RDD with a lesion in the thoracic intradural extramedullary space causing acut ...

This work has been carried out at UCL/UCLH which receive funding from the National Institute for Health Research Comprehensive Biomedical Research Centre.