Surgical management of pancreatic neuroendocrine tumors in von Hippel-Lindau disease

von Hippel-Lindau (VHL) disease is a hereditary autosomal dominant syndrome characterized by the development of several different neoplasms. Although central nervous system hemangioblastomas and renal cell carcinomas are the most significant source of morbidity and mortality associated with VHL disease, approximately two-thirds of patients with VHL will have pancreatic lesions identified throughout their lifetime. Cystic pancreatic lesions are benign; however solid lesions most commonly represent pancreatic neuroendocrine tumors (PanNETs), which have the ability to metastasize. VHL-associated PanNETs are almost uniformly nonfunctional with variable metastatic potential. Surgical treatment depends on the size of the lesion, tumor genotype, the location within the pancreas as well as patient-related factors. This chapter will outline the treatment options for patients with VHL disease and PanNETs.