Pancreatic neuroendocrine tumors associated with vonHippel Lindau disease:Diagnostic and management recommendations

Background. von Hippel Lindau disease (VHL) is an inherited syndrome charac terized by tumors of the kidney, adrenal, central nervous system and pancre as. The incidence and natural history of pancreatic neuroendocrine tumors o ccurring in VHL are not known. Methods. From December 1988 through November 1997, 256 patients with VHL we re screened with imaging studies, and these data were reviewed from a prosp ective database. Results. Thirty (12 %) of 256 patients had solid pancreatic lesions consist ent with neu neuroendocrine tumors. Fourteen patients underwent resection, and 4 with metastases on imaging studies underwent biopsy only. Of the 14 p atients who underwent resection, 11 remain free of disease, 2 have experien ced recurrence, and 1 has died of unrelated causes (mean follow-up, 25 mont hs; range, 3 to 73 months). The size of the primary tumor (median, 5 cm; ra nge, 3 to 8 cm) in patients with liver metastases was significantly larger than the size of the primary tumor (median, 2 cm; range, 1 to 5 cm) in pati ents without liver metastases (P = .0013). Conclusions. Solid pancreatic lesions were detected in 12 % of patients wit h VHL. Larger primary tumors were associated with liver metastases. Pancrea tic imaging to identify neuroendocrine tumors and resection when they reach 2 to 3 cm may prevent the development of hepatic metastases.