Oncological management of gastro-entero-pancreatic neuroendocrine neoplasias

Petrányi Ágota (1), Uhlyarik Andrea (2), Rácz Károly (3), Bodoky György (1)
– (1) Egyesített Szent István és Szent László Kórház, Onkológiai Osztály, Budapest
– (2) Magyar Honvédség Egészségügyi Központ, Budapest
– (3) Semmelweis Egyetem, II. Sz. Belgyógyászati Klinika, Budapest

Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are unusual and relatively rare neoplasms. They characteristically synthetize, store and secrete a variety of peptides and neuroamines, which can lead to development of disctinct clinical syndromes. Clinical symptoms and presentations vary depending on the location and hormones produced by the tumor. The diagnosis of NETs is established by histological examination and the immunohistochemical detection of general neuroendocrine markers, such as chromogranin A (CgA) and synaptophysin. An update of the WHO classifi cation has resulted in a new classification dividing neuroendocrine neoplasms into neuroendocrine tumors (NETs) including G1 (Ki67 index ≤2%) and G2 (Ki67 index 3–20%) tumors and neuroendocrine carcinomas (NECs) with Ki67 index >20%, G3. The different available therapeutic approaches, including surgery, liver-directed ablative therapies, peptide receptor radionuclide therapy, and systemic hormonal, cytotoxic or targeted therapy, are discussed in this overview.