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

A case of subglottic narrowing caused by an extranodal deposit of sinus histiocytosis in an eight-year-old child with Rosai-Dorfman disease is presented. The airway obstruction was initially managed with a tracheostomy, and then on confirmation of the diagnosis by lymph node biopsy, by excision of the extranodal deposit with a CO2 bronchoscopic laser.
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PMID:Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): a rare case of subglottic narrowing. 154 96

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

An 18 year-old woman presented with massive cervical lymphadenopathy. She also had bilateral parotid gland enlargement, and later upper airway obstruction from subglottic mass compression. Surgical excision of lymph nodes and bilateral total parotidectomy with facial nerve preservation were selected. Direct laryngoscopy and CO2 laser excision were used to remove the subglottic mass. Airway was secured by tracheostomy and after that it was removed. Histologic finding was Sinus histiocytosis with massive lymphadenopathy (SHML) that involved the subglottic. SHML is very rare, probably less than 1000 cases reported in the literature and this is the 6th case in the literature that involved the subglottic. There are no standard treatment modalities. The treatment is comprised of surgery, steroid, chemotherapy and local control. In the present case the authors chose surgery, steroid and local control of disease in the subglottic by using CO2 laser excision. Now, the disease is under control.
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PMID:Sinus histiocytosis with massive lymphadenopathy and subglottic stenosis: a case report. 1624 Oct 31