Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019625 (Rosai-Dorfman disease)
 763 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Rosai-Dorfman disease, first described in 1969, is a rare idiopathic histioproliferative disease affecting the lymph nodes. Typical clinical features include bilateral painless lymphadenopathy, fever and polyclonal hypergammaglobulinemia. In approximately 43% of cases, extranodal sites may be involved and occasionally represent the initial or sole manifestation of the disease. Central nervous system manifestations are exceedingly rare, and only 17 cases have been recorded, among which there are merely seven isolated intracranial cases without concurrent nodal or other extranodal involvement. Herein, we report a 38-year-old male presenting with generalized tonic-clonic seizure and radiological findings indicative of meningioma. Complete physical examinations and laboratory surveys demonstrate the absence of involvement at other body sites. Microscopically, the lesion consists of proliferative histiocytes exhibiting emperipolesis coupled with the characteristic cytoplasmic staining against S-100 protein. The differential diagnosis of polymorphic inflammatory meningioma-mimicking masses is discussed, and a review of previously reported intracranial Rosai-Dorfman disease is presented.
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PMID:Isolated intracranial Rosai-Dorfman disease: case report and literature review. 970 47

A 9-year-old boy presented with a 12-month history of headaches and recent grand mal seizures. Computed tomography and magnetic resonance imaging of the brain revealed an enhancing right frontal convexity lesion. The preoperative diagnosis was meningioma. However, histological examination was diagnostic of Rosai-Dorfman disease. Rosai-Dorfman disease confined to the intracranial compartment is very rare, of 34 reported cases only one presented in the first decade. Optimal treatment has not been established, but complete surgical resection alone seems effective and allows histological distinction from meningioma.
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PMID:Isolated intracranial Rosai-Dorfman disease mimicking meningioma in a child. 1532 36

A 40-year-old male presented with a single generalized tonic-clonic seizure. MRI revealed an enhancing, dural-based, left lateral sphenoid wing lesion suggestive of a meningioma. At microsurgical excision, the lesion was firm and relatively avascular. The histopathological report revealed S-100 positive histiocytic proliferation with lymphophagocytosis (emperipolesis) characteristic of the Rosai-Dorfman disease. The case and its management are discussed.
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PMID:Rosai-Dorfman disease mimicking a sphenoid wing meningioma. 1580 69