Ear Nose Throat J. 2013 Apr-May;92(4-5):184-8.
FIRST PARAGRAPH: Endolymphatic sac tumor (ELST) is a papillary epithelial neoplasm arising within the endolymphatic sac/duct that shows a high association with von Hippel-Lindau disease (VHL). There is usually a VHL tumor suppressor gene germline mutation with an autosomal dominant inheritance pattern. Approximately 1 in 35,000 to 40,000 people have VHL, of which approximately 10 to 15% have endolymphatic sac tumors. There is a wide age range at presentation, although most patients are between 30 and 40 years; there is no gender predilection.
PubMed ID: 23599098
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