Characterization of endolymphatic sac tumors and von Hippel–Lindau disease in the International Endolymphatic Sac Tumor Registry

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.

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