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)

We present a case of an 81-year-old man with a history of worsening dysphonia of six months duration. A year before a sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) was diagnosed on a submandibular lymph node biopsy. On presentation the patient showed a mass in the left subglottic area with a modest reduction in the airway space. The patient underwent an endoscopic CO2 laser excision of the mass, without post-operative complications. Histopathological examination and electron microscope images confirmed the previous diagnosis of Rosai-Dorfman disease. The clinical and pathological features of this entity are discussed.
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PMID:Rosai-Dorfman disease of the larynx. 1099 48

Rosai-Dorfman disease is a rare histiocyte disorder that is typically characterized by massive cervical lymphadenopathy. Isolated extranodal involvement is uncommon, and isolated laryngeal involvement is extremely rare. We report an unusual case of Rosai-Dorfman disease with isolated laryngeal involvement that led to recurrent dysphonia and airway obstruction. We discuss the challenges we faced in reaching a correct pathologic diagnosis and in deciding on an appropriate treatment regimen. Based on our experience, we believe that Rosai-Dorfman disease should be considered as a differential diagnosis in patients who present with a recurrent inflammatory (histiocytic) mass lesion of the larynx.
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PMID:Rosai-Dorfman disease with isolated laryngeal involvement. 2394 Aug 76