title: [Prophylactic pancreas surgery].
abstract: The goal of prophylactic surgery is to prevent malignant growth in patients with hereditary tumor predisposition. The pancreas presents as particularly challenging, due to the difficulty of operation and comparatively high risk of morbidity and even mortality. In addition, partial operative procedures and, more significantly, total resection lead to exocrine pancreas insufficiency and secondary diabetes, with grave consequences for the patient. Hereditary tumor predisposition syndromes that can result in pancreaticoduodenal endocrine tumors (PET) include multiple endocrine neoplasia type 1 syndrome and von Hippel-Lindau syndrome. As penetrance is maximally 70-80% and the 10-year survival rate over 80%, prophylactic pancreatic resection without evidence of a tumor is not indicated. However, prophylactic extension of a resection would be advised, should a PET be diagnosed. Patients predisposed to developing ductal pancreatic carcinoma (PC) are at risk of familial pancreatic cancer syndrome (FPC), hereditary pancreatitis, and other hereditary tumor predisposition syndromes such as Peutz-Jeghers syndrome and familial atypical multiple mole-melanoma syndrome. As the gene defect responsible for FPC has yet to be identified and the penetrance of PC in the other tumor predisposition syndromes is low or unknown, a prophylactic pancreatectomy based on today's knowledge is not indicated. Prophylactic extension of the resection is advisable should PC or high-grade PanIN lesions be diagnosed, as these patients often present with multifocal dysplasia and even carcinoma.
authors.affiliation: Klinik für Visceral-, Thorax- und Gefässchirurgie der Philipps-Universität Marburg.
authors.forename: P
authors.initials: P
authors.lastname: Langer
authors.forename: M
authors.initials: M
authors.lastname: Rothmund
authors.forename: D K
authors.initials: DK
authors.lastname: Bartsch
mesh_terms: D004379:Duodenal Neoplasms / Q000235:genetics / Q000401:mortality / Q000473:pathology / Q000517:prevention & control*; D020022:Genetic Predisposition to Disease / Q000235:genetics; D006801:Humans; D018761:Multiple Endocrine Neoplasia Type 1 / Q000235:genetics* / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D010179:Pancreas / Q000473:pathology; D010180:Pancreatectomy; D010190:Pancreatic Neoplasms / Q000235:genetics* / Q000401:mortality / Q000473:pathology / Q000517:prevention & control*; D010375:Pedigree; D011379:Prognosis; D018570:Risk Assessment; D016019:Survival Analysis; D015996:Survival Rate