Endocrine Abstracts

Background: von Hippel-Lindau syndrome (VHL) is a rare autosomal dominant disease caused by alterations of the vhl tumor-suppressor gene. Patients with VHL are at risk for development of retinal, central nervous system and spine hemangioblastomas, clear-cell renal cell carcinomas, pheochromocytomas, endolymphatic sac tumors and cysts; and pancreatic islet cell tumors. Based on the presence or absence of pheochromocytoma as a phenotypic marker, VHL can be divided into di...