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 (RDD) is a rare histiocytic disorder of unknown cause. RDD most commonly involves the cervical lymph nodes, but extranodal involvement has been described. We report the case of a patient with extranodal RDD that presented as a retroperitoneal mass obstructing the left ureter. The patient underwent surgical resection of the mass, followed by a 5-month course of vinblastine. There was no evidence of progressive disease 22 months after surgery.
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PMID:Extranodal Rosai-Dorfman disease as a pelvic mass. 1651 11

Rosai-Dorfman disease is a rare condition with poorly understood pathogenesis at this time. Although it often involves the lymph nodes, it can present nearly anywhere at extranodal sites. Patients are frequently asymptomatic, but surgical debulking is currently the only method of treatment that has shown benefit for patients requiring intervention. This case report discusses a unique presentation of Rosai-Dorfman disease involving the ureter of a 49-year-old woman with known history of sarcoidosis, discovered incidentally on routine CT scan.
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PMID:Rosai dorfman disease: A rare presentation of primary ureteral involvement with concurrent sarcoidosis. 3250 34