Emergency Pancreatoduodenectomy for Pancreatic Metastasis from Renal Cell Carcinoma in a Patient with von Hipple-Lindau Disease: A Case Report

Excerpt

Von Hippel-Lindau (VHL) disease is an autosomal-dominant neoplasia syndrome, with an estimated prevalence of 1 per 36,000 live births [1]. The types of tumor seen in patients with VHL disease include hemangioblastoma of the central nervous system and retina, renal cell carcinoma (RCC), neuroendocrine tumor of the pancreas, and pheochromocytoma of the adrenal gland. The mechanism responsible for carcinogenesis in this disease complex is a loss of tumor suppressor function resulting from a causative mutation in the VHL gene. Unusually, a patient with VHL disease inherits heterozygous mutation in the VHL gene. In such individuals, a tumor arises when the wild-type allele is inactivated by somatic mutation in a susceptible organ (Knudson's two-hit hypothesis) [1, 2]. Because of the wide variety of tumor types, treatment for each lesion should be carefully stratified and coordinated with multispecialty care [1, 2]. …