2020 Year in Review - Neuroendocrine Tumors

Results of the CLARINET FORTE trial showed that lanreotide autogel (LAN) 120 mg at escalating dosing frequency (every 14 days) was associated with promising progression-free survival (PFS) benefit and no new safety issues in patients with progressive pancreatic or midgut neuroendocrine tumors (NETs).
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.
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