Article
Endolymphatic sac tumor with von Hippel-Lindau disease: report of a case with analysis of von Hippel-Lindau gene and review.
Annals of diagnostic pathology - 1 Oct 2010
Rao Qiu, Zhou Jing, Wang Jian-dong, Jin Xing-zao, Ma Heng-hui, Lu Zhen-feng, Zhou Xiao-jun
Abstract excerpt
Endolymphatic sac tumors (ELSTs) are very rare and locally aggressive low-grade neoplasm of endolymphatic system origin, which are associated with von Hippel-Lindau (VHL) disease. Evidence suggests that the specific VHL alteration influences the phenotype. Because of the rarity of ELSTs, only a small number of cases have been subjected to molecular genetic analysis. The correlation between ELSTs and the genotype...
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