Preventive Medicine and Management of von Hippel-Lindau Disease-associated Pancreatic Neuroendocrine Tumors.

IRINA BANCOS; MASSIMILIANO FERRARA; TOBIAS KRAUSS, MD,; THERA P. LINKS; ULRICH WELLNER; ANDREY KVACHENYUK; KARINA VILLAR GÓMEZ DE LAS HERAS; MARINA Y. YUKINA; ROMAN PETROV; GARRETT BULLIVANT; LAURA VON DUECKER; SWATI JADHAV; URSULA PLOECKINGER; STAFFAN WELIN; CAMILLA SCHALIN-JÄNTTI; OLIVER GIMM; MARIJA PFEIFER; JOANNE NGEOW; KORNELIA HASSE-LAZAR; GABRIELA SANSÓ, PHD

ENDOCRINE - RELATED CANCER

BIOSCIENTIFICA LTD

Lugar: Bristol; Año: 2018

1351-0088

Question: Does operative removal of pancreatic neuroendocrine tumors (PanNETs) in patients with von Hippel-Lindau disease (VHL) prolong survival?Findings: In a worldwide registry of 2,256 patients with VHL, 259 (11%) had a total of 460 PanNETs. The 10-year survival was significantly longer in operated vs non-operated patients, in particular for PanNETs 2,000 entries) to assess diagnostic and treatment data for PanNETs and other VHL-associated tumors. We searched for predictors for malignancy and assessed long-term outcomes in operated and non-operated patients.Main outcomes and measuresMeasures and outcomes included clinical and germline mutation data; imaging-based tumor size measurement on CT or MRI; calculation of tumor growth rates; comorbidities; mode of surgery; and survival.