Endolymphatic sac tumour in von Hippel-Lindau disease: management strategies
نویسندگان
چکیده
منابع مشابه
Endolymphatic sac tumour in von Hippel-Lindau disease: management strategies
Endolymphatic sac tumour (ELST) is infrequent, as emerges from small series reported in the literature. It is a slow-growing malignancy with local aggressiveness and a low risk of distant metastases. It is often misdiagnosed because of the late onset of symptoms and difficulty in obtaining a biopsy. Its frequency is higher in von Hippel-Lindau (VHL) disease (a genetic systemic syndrome involvin...
متن کاملTumors of the endolymphatic sac in von Hippel-Lindau disease.
CONTEXT Endolymphatic sac tumors (ELSTs) are associated with von Hippel-Lindau disease and cause irreversible sensorineural hearing loss (SNHL) and vestibulopathy. The underlying mechanisms of audiovestibular morbidity remain unclear and optimal timing of treatment is not known. OBJECTIVE To define the mechanisms underlying audiovestibular pathophysiology associated with ELSTs. DESIGN, SETT...
متن کاملCharacterization of endolymphatic sac tumors and von Hippel-Lindau disease in the International Endolymphatic Sac Tumor Registry.
BACKGROUND Endolymphatic sac tumors (ELSTs) are, with a prevalence of up to 16%, a component of von Hippel-Lindau (VHL) disease. Data from international registries regarding heritable fraction and characteristics, germline VHL mutation frequency, and prevalence are lacking. METHODS Systematic registration of ELSTs from international centers of otorhinolaryngology and from multidisciplinary VH...
متن کاملEndolymphatic sac tumor and von Hippel-Lindau disease in a single family.
Serviços de Radiologia, Otorrinolaringologia e Genética Clínica do Hospital Universitário de Brasília, Universidade de Brasília (UnB), Brasília DF, Brazil: Médica Residente de Radiologia e Diagnóstico por Imagem, Hospital Universitário de Brasília (HUB), Universidade de Brasília (UnB); Médico Neurorradiologista, Doutorando do Programa de Pós-graduação em Ciências Médicas da Faculdade de Medicin...
متن کاملSurgical resection of endolymphatic sac tumors in von Hippel-Lindau disease: findings, results, and indications.
OBJECTIVES/HYPOTHESIS To define the surgical treatment and outcomes of von Hippel-Lindau (VHL) disease-associated endolymphatic sac tumors (ELSTs), we analyzed consecutive VHL patients who underwent ELST resection. STUDY DESIGN Retrospective investigation of consecutive VHL patients who underwent resection of ELSTs at a clinical research center between 1999 and 2010. METHODS Analysis of ser...
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ژورنال
عنوان ژورنال: Acta Otorhinolaryngologica Italica
سال: 2017
ISSN: 0392-100X,1827-675X
DOI: 10.14639/0392-100x-1402