Table 1.

Summary table for Rosai-Dorfman disease, both for extra-CNS disease and for isolated spinal disease.

	Extra-CNS Disease	Isolated Spinal Disease
Etiology	Idiopathic	Idiopathic
Incidence	100 cases per year in US	15 reported cases (6 epidural, 7 intradural, 2 intramedullary)
Gender Ratio (M:F)	1.4:1	1.5:2
Age Predilection	Mean age 21 years	Mean age 40 years
Risk Factors	Unknown	Unknown
Clinical Symptoms	Variable depending on location of involvement Constitutional symptoms common	Bilateral limb paraparesis, sensory deficits, urinary and bowel incontinence, back pain Constitutional symptoms rare
Laboratory Findings	Anemia, leukocytosis, elevated ESR, and/or polyclonal hypergammaglobulinemia reported in most patients	Anemia, leukocytosis, elevated ESR, polyclonal hypergammaglobulinemia occur variably
Imaging Findings	Variable CT and MRI findings depending on location	General: Well circumscribed mass in dural or epidural space CT: Hyper-dense; homogenous enhancement MRI: T1WI iso-intensity; T2WI hypo- or iso-intensity with possible heterogeneity; homogenous enhancement post-contrast
Treatment	Surgical resection If recurrent, persistent, or multifocal disease, radiotherapy, steroids, or chemotherapy are variably effective	Surgical resection If recurrent, persistent, or multifocal disease: radiotherapy, steroids, or chemotherapy
Prognosis	Good	Good