SEOM-GETNE clinical guidelines for the diagnosis and treatment of gastroenteropancreatic and bronchial neuroendocrine neoplasms (NENs) (2022)

Abstract

Neuroendocrine neoplasms (NENs) are a heterogeneous family of tumors of challenging diagnosis and clinical management. Their incidence and prevalence continue to rise mainly due to an improvement on diagnostic techniques and awareness. Earlier detection, along with steadfast improvements in therapy, has led to better prognosis over time for advanced gastrointestinal and pancreatic neuroendocrine tumors. The aim of this guideline is to update evidence-based recommendations for the diagnosis and treatment of gastroenteropancreatic and lung NENs. Diagnostic procedures, histological classification, and therapeutic options, including surgery, liver-directed therapy, peptide receptor radionuclide therapy, and systemic hormonal, cytotoxic or targeted therapy, are reviewed and discussed, and treatment algorithms to guide therapeutic decisions are provided.

Making progress against rare cancers: A case study on neuroendocrine tumors

“…Fewer than 20,000 people in the United States are diagnosed with a NET each year 9 and there are approximately 175,000 prevalent cases 10 . Several treatments are available for NETs, 11 but questions remain about which treatments work the best and the order in which to use them.…”

Mixed neuroendocrine non-neuroendocrine neoplasms in gastroenteropancreatic tract

“…By analogy with SCLC, the first line in metastatic or unresectable disease should include systemic chemotherapy combining etoposide or irinotecan and a platinum salt[ 75 ]. At the molecular level, there is a greater expression of excision repair 1, a non-catalytic subunit of endonuclease, which participates in DNA repair in the NE component and suggests a better response to the combined treatment of platinum and etoposide[ 76 ], although there are studies showing results that are discordant with this molecular explanation.…”

“…Firstly, somatostatin analogues have been proposed for MiNENs with NET G1 and G2 components, since the expression of somatostatin receptor type 5 is 81.8% and 60% in G1 and G2, respectively[ 47 ]. Radiotherapy with radionuclides (PRRT) is considered at progression, which has demonstrated a survival benefit, and sunitinib or everolimus in successive lines[ 75 ].…”

“…First-line STZ with 5-fuorouracil, or temozolomide and CAPTEM are the preferred treatment options. In subsequent lines, it is possible to use FOLFOX or everolimus or, if possible, PRRT[ 75 ].…”