Current and emerging therapies for PNETs in patients with or without MEN1

摘要: Pancreatic neuroendocrine tumours (PNETs) might occur as a non-familial isolated endocrinopathy or part of complex hereditary syndrome, such multiple endocrine neoplasia type 1 (MEN1). MEN1 is an autosomal dominant disorder characterized by the combined occurrence PNETs with parathyroids and anterior pituitary. Treatments for primary include surgery. non-resectable metastases biotherapy (for example, somatostatin analogues, inhibitors receptors monoclonal antibodies), chemotherapy radiological therapy. All these treatments are effective in patients without MEN1; however, there scarcity clinical trials reporting efficacy same MEN1. Treatment challenging owing to concomitant development other tumours, which have metastasized. In recent years, preclinical studies identified potential new therapeutic targets treating MEN1-associated (including PNETs), epigenetic modification, β-catenin-wingless (WNT) pathway, Hedgehog signalling, gene replacement This Review discusses advances.