title: Management of pancreatic gastrinoma associated with Von Hippel-Lindau disease: a case report.
abstract: BACKGROUND
  Pancreatic neuroendocrine tumors (PNET) are a heterogeneous group and constitute 1.3% of all pancreatic tumors. Approximately 10% of these occur in the context of hereditary syndromes, such as VHL disease.
  
  
  METHODS
  We report a case of a female patient of 37 years diagnosed VHL and intervened on several occasions by cerebral hemangioblastoma and renal carcinomas. During its follow-up she was diagnosed 2 gastrinomas functioning under 2 cm were enucleated. Later developed new PNET and underwent a total duodenopancreatectomy without pyloric preservation.
  
  
  CONCLUSIONS
  The management of PNET in VHL is difficult due to the association of multiple tumors in different organs and the morbidity and mortality associated with the surgery of the pancreas. Management must be individualized for each patient, based on the ability to produce hormones and present symptoms, the size and location, and in the context of other tumors that usually present in these patients.
authors.affiliation: cirugia digestiva, Hospital Universitario y Politécnico La Fe, españa.
authors.forename: Ángela
authors.initials: Á
authors.lastname: Sala Hernández
authors.affiliation: Cirugía Hepatobiliopancreática, Hospital Universitario y Politécnico La Fe.
authors.forename: Eva María
authors.initials: EM
authors.lastname: Montalvá Orón
authors.affiliation: Cirugía Hepatobiliopancreática, Hospital Universitario y Politécnico La Fe.
authors.forename: Eugenia
authors.initials: E
authors.lastname: Pareja Ibars
authors.affiliation: Cirugía General y del Aparato Digestivo, Hospital Universitario y Politécnico La Fe.
authors.forename: Neus
authors.initials: N
authors.lastname: Ballester Pla
authors.affiliation: Cirugía Hepatobiliopancreática, Hospital Universitario y Politécnico La Fe.
authors.forename: Rafael
authors.initials: R
authors.lastname: López Andújar
mesh_terms: D000328:Adult; D004386:Duodenum / Q000601:surgery; D005260:Female; D015408:Gastrinoma / Q000000981:diagnostic imaging / Q000209:etiology* / Q000601:surgery*; D006801:Humans; D010190:Pancreatic Neoplasms / Q000000981:diagnostic imaging / Q000209:etiology* / Q000601:surgery*; D016577:Pancreaticoduodenectomy; D049268:Positron-Emission Tomography; D006623:von Hippel-Lindau Disease / Q000150:complications* / Q000000981:diagnostic imaging