Management of the pancreatic gastrinoma associated with Von Hippel-Lindau disease. About a case

Autores de IIS La Fe

• Angela Sala Hernández

  Autor

• Eva María Montalvá Orón

  Autor

• Eugenia Pareja Ibars

  Autor

• Maria Neus Ballester Pla

  Autor

• Rafael López Andújar

  Autor

Grupos

• Hepatología experimental y Trasplante hepático

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. CASE REPORT: 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. DISCUSSION: 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.