Navigating the treatment landscape in gastroenteropancreatic neuroendocrine neoplasms

Gastroenteropancreatic neuroendocrine neoplasms are a large and very diverse group of neoplasms. They are becoming a burning clinical problem because of increasing frequency and diagnosis in the advanced state. The treatment landscape has been changed over the last years. Treatment choice depends on many factors such as the tumor’s type, location, aggressiveness, and hormone-producing capabilities. The main goals of treatment are long-term symptomatic control, antitumor effect, and improvement of the quality of life. The results of the PROMID and CLARINET trials have augmented fundamental position of somatostatin analogs. Our understanding of the biology, genetics of the neoplasms has improved considerably in the last several decades and the spectrum of available therapeutic options is rapidly expanded. The current evidence-based treatment options include everolimus, sunitinib, peptide receptor radionuclide therapy, and chemotherapy. Treatment practice changed as a result of high-quality phase 3 clinical trials which shaped current guidelines; multiple retrospective studies which raised new questions and attempted to fill some of the data gaps. Here we review the treatment options for gastroenteropancreatic neuroendocrine neoplasms, discussing important diagnosis and biomarker-related factors, safety of therapy with special insight into cardiac safety, as well we looked at promising investigative therapies.