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2020 Year in Review - Neuroendocrine Tumors

Combined data from two phase 2 trials indicate that avelumab monotherapy had limited antitumor activity in patients with grade 2/3 neuroendocrine neoplasms (NENs).
Results of a retrospective analysis showed that checkpoint inhibitors as monotherapy had limited clinical benefit in patients with grade 3 neuroendocrine tumors (NETs) or neuroendocrine carcinomas (NECs), and modest benefit when combined with another checkpoint inhibitor or chemotherapy.
The ongoing nonrandomized, open-label, single-arm phase 2 NICE-NEC trial is evaluating the safety and efficacy of adding the immune checkpoint inhibitor nivolumab to standard platinum-doublet chemotherapy as first-line therapy in patients with metastatic or unresectable grade 3 neuroendocrine neoplasms (NENs) of gastroenteroenpathic (GEP) or unknown origin.
The randomized phase 3 JCOG1205/1206 trial did not demonstrate superiority of irinotecan + cisplatin versus etoposide + cisplatin in patients with completely resected high-grade neuroendocrine carcinomas (HGNECs) of the lung.
Results of a retrospective real-world data analysis indicate that temozolomide-based regimens are associated with a high disease control rate (DCR) and a manageable toxicity profile in patients with metastatic neuroendocrine neoplasms (NENs).
Findings of a retrospective safety analysis suggest that the risk for severe myelotoxicity or opportunistic infections is rare in patients with advanced neuroendocrine tumors (NETs).
The design of an ongoing phase 2 study (NCT04412629) evaluating the efficacy and safety of the multitarget tyrosine kinase inhibitor cabozantinib in patients with high-grade neuroendocrine neoplasms (NENs) was presented at the 2020 North American Neuroendocrine Tumor Society Annual Symposium.
Results of a retrospective study indicate that the 5-fluorouracil + leucovorin + oxaliplatin (FOLFOX) ± bevacizumab regimen is active in patients with aggressive and heavily pretreated pancreatic neuroendocrine tumors (NETs) who have progressed on prior capecitabine + temozolomide chemotherapy.
Patient-reported outcomes data of the XERMELO Patient Registry indicate that patients with neuroendocrine tumors (NETs) experiencing carcinoid syndrome (CS) reported improvement in CS symptom control and satisfaction with telotristat ethyl (TE) treatment, as well as reduced activity impairment and work productivity losses after 6 months of treatment.
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