Isolate vertigo crisis revealing an endolymphatic sac tumor

Endolymphatic sac tumors are rare entities that have a destructive potential on the temporal bone. They are aggressive tumors presenting as low-grade papillary adenocarcinoma, but there are no reports of metastasis in the literature. The Von Hippel-Lindau disease is a hereditary condition caused by germinal mutations of the tumor-suppressor VHL-gene. We present the case of an endolymphatic sac tumor associated with the Von Hippel-Lindau disease at a 46-year-old patient revealed by an isolate vertigo crisis, discussing the management of the tumor and the clinical, imaging, genetic and histopathologic features of it.

Endolymphatic sac tumors have recently been described as part of the Von Hippel-Lindau disease, a genetic disorder involving the development of hypervascular tumors. The treatment depends on the size of the tumor, however surgical approach is the most successful choice and can be associated or not with radiotherapy.