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 is a benign lymphohistiocytosis that often involve lymph nodes and present as massive lymphadenopathy with sinus histiocytosis. The disease is rarely associated with intracranial involvement. Herein, we report a 33-years-old man with recent onset of unconsciousness. According to his past medical history, he was suffering from frontal headache, ataxia and dizziness with no sensory or motor defect since August 2010. At initial work up, MRI showed infiltrating mass in the left parietal region. Microscopically, the mass consisted of infiltration of abundant lymphoplasma cells, neutrophils and some histiocytes scattered in fibrotic background. Emperipolesis (lymphocytophagocytosis) of histiocytic cells made the diagnosis of Rosai-Dorfman disease. Rosai-Dorfman disease should be added in the list of differential diagnosis for a dural mass mimicking meningioma or cerebral mass mimicking glioma, therefore, immunohistochemical staining for EMA, S100 and CD1a should be performed to rule out the differential diagnosis.
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PMID:Intracranial ROSAI-DORFMAN Disease. 2326 85

Chordoid glioma (CG), which often originated from the third ventricle, was a type of rare and slowly growing low grade glioma with chordoid appearance. So far, concurrent occurrence of third ventricule chordoid gliomas with Rosai-Dorfman disease (RDD) has never been observed. In this study, the clinical features, pathological characteristics as well as their prognosis of two CG with RDD component cases were discussed. Herein, we reported two cases of CG with RDD component from a 45-year-old female and a 38-year-old male respectively. Radiologically, the two lesions were located on the region of suprasellar-third ventricular with homogenous contrast enhancement. They underwent gross total resection and remained free of disease progress till now. Microscopically, both lesions showed the classic morphology of CG and RDD component. A morphologic curiosity is presented in tumors manifested by CG with RDD component in the suprasellar-third ventricular region. To the best of our knowledge, this is the first presentation of two collision tumors consisting of CG and RDD on the suprasellar-third ventricular region. Awareness of this entity is important to distinguish it from other CGs. More examples and a timely follow-up are required for understanding the biological features of these cases.
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PMID:Concurrence of chordoid gliomas with Rosai-Dorfman component: report of two rare cases. 3196 79