Total pancreatectomy for multiple neuroendocrine tumors of the pancreas in a patient with von Hippel-Lindau disease.

Von Hippel-Lindau disease (VHLD) is an autosomal dominant familial syndrome associated with multiple neoplasms. Medical management of pancreatic lesions is still controversial, especially for pancreatic neuroendocrine tumors (NET). We report an experience of total pancreatectomy for multiple pancreatic neuroendocrine tumors in a VHLD patient, and discuss the indication of surgical treatment. The patient was a 33-year-old Japanese female with a medical history of VHLD-associated tumors. At 27 years of age, abdominal computed tomography revealed a number of strongly enhanced round tumors throughout the pancreas. She underwent total pancreatectomy with portal vein resection because of back pain and an increase of tumor size. Pathological examination reconfirmed the diagnosis of multiple pancreatic NET invading the portal vein. She has been well with intensive insulin therapy and has shown no recurrence of NET for more than one year. This is a rare case of total pancreatectomy with portal vein resection for treatment of pancreatic NET in a VHLD patient. Total pancreatectomy is a viable option for treatment of multi-centric or extensive pancreatic NET because of a favorable prognosis of NET after radical surgical treatment.