von Hippel-Lindau Disease Associated Pancreatic Neuroendocrine Tumors – Molecular Genetics and Clinical Aspects - Abstract
Pancreatic neuroendocrine tumors (PNETs) occur in about 10% of patients with von Hippel-Lindau disease (VHL). Females are more frequently affected than men. VHL associated PNETs are virtually always endocrine inactive. PNETs are mostly detected in patients with already known VHL disease. VHL associated PNETs occur as single or multiple tumors. Magnetic resonance imaging (MRI) is the method of choice to detect PNETs. Imaging in the early arterial phase is of utmost importance. Nuclear medicine imaging with newly introduced and promising agents such as [68Ga]-SST receptor analogs or [18F]-DOPA-PET CT, is recommended preoperatively to confirm the diagnosis, to exclude multifocal tumors and to identify potential metastases. Surgery should be performed for PNETs measuring 3 or more cm in diameter. PNETs of the tail or body of the pancreas can be resected by endoscopic technique. Treatment options for malignant tumors include tumor debulking, nuclear radiation by [90Y] or [177Lu]-labelled DOTA-TATE or DOTA-TOC, somatostatin analogs, and tyrosine kinase inhibitors. Regular follow-up investigations with MRI of the abdomen are recommended for all VHL patients in order to detect and remove these tumors before reaching 3 cm in diameter. Once PNETs are excluded in VHL patients, controls every 2-3 years are adequate.