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. 2023 Jul 8;42(3):1021–1054. doi: 10.1007/s10555-023-10121-2

Table 6.

Clinical trials of immune checkpoint inhibitors in NEC

Trials Target Agent Line Phase Treatment Primary sites Key outcomes Ref
NCT03278405 PD-L1 Avelumab (Avel) 1st II Mono GEP or lung Among 10 GEP and pulmonary NECs in NET001 cohort, 9 were GEP-NECs. In all patients, ORR, 0%; mPFS, 2.0 months; mOS, 5.7 months 210
NCT03352934 2nd II Mono Any Among 29 patients with G3 NENs, 16 and 19 were NECs and GEP-G3 NENs, respectively. In an interim analysis, the DCR after 8 weeks was 32% (PR of two); mOS, 4.2 months 213
NCT02939651 PD-1 Pembrolizumab (Pembro) 2nd II Mono Any Among 29 patients with G3 NEN, 24 and 19 were GEP-NENs and NECs, respectively. In all patients, ORR was 3.4%; mPFS, 8.9 weeks; mOS, 20.4 weeks. Only one patient with a large cell esophageal NEC had an objective PR that was ongoing for 13 months 209
NCT03136055 2nd II Mono Any Among 14 NECs after failure of previous therapy, 6 were GEP-NECs. In all patients, ORR, 7%; mPFS, 58 days. Because more than 2 of 14 patients did not respond by week 18 in the stage-1 part, the stage-2 part for additional patient enrollment was terminated 211
NCT03136055 2nd II Pembro + chemo (CPT-11 or PTX) Any Among 22 NECs after failure of previous therapy, 16 were GEP-NECs. Chemotherapy: 17 CPT-11 and 5 PTX. In all patients, ORR, 9%; mPFS, 2 months; mOS, 4 months 244
NCT03167853 Toripalimab (Tori) 2nd I Mono Any Among 40 NENs (Ki-67 ≥ 10%) after failure of previous therapy, 32 were NECs, including 25 GEP-NECs. ORR, 18.7% in NEC and 25.0% in NET. In all patients, the mPFS and mOS were 2.5 and 7.8 months, respectively 214
NCT03879057 2nd I Tori + surufatinib Any Among 22 patients with NENs, 14 were NECs. In 13 evaluable NECs, ORR, 23.1%; mPFS, 4.0 months; mOS, 7.5 months 246
NCT04169672 2nd II Tori + surufatinib Any In 20 evaluable NECs, ORR, 20%; DCR, 70%; mPFS, 3.94 months 247
NCT02955069 Spartalizumab (Spa) 2nd II mono GEP Among 116 NENs after failure of previous therapy, 21 were GEP-NECs. ORR, 4.8% in GEP-NEC, and 7.4% in NET. In GEP-NEC, mPFS and mOS were 1.8 and 6.8 months, respectively 212
NCT03980925 Nivolumab (Nivo) 1st II Nivo + CBDCA + ETP GEP or UK Among 38 patients with G3 GEP or unknown NENs in the first-line setting, 26 and 31 were NECs and GEP-NENs, respectively. In all patients, ORR was 54.1%; mPFS, 5.7 months; mOS, 13.9 months. The 12-month OS rate was 58.3% and 54.7% in NETs and NECs, respectively 243
NCT03728361 1st II Nivo + TEM Any Among 28 NENs, 14 and 8 were GEP-NENs and NECs, respectively. ORR, 38% in NEC and 35% in NET. In the NECs, mPFS and mOS were 6.9 and 32.3 months, respectively 239
NCT03591731 2nd II (randomized parallel arms) mono GEP or lung Among 91 refractory pulmonary LCNECs or GEP-NECs after failure of previous therapy, 46 were GEP-NECs. ORR at 8 weeks, 7.1% in GEP-NEC, and 7.3% in pulmonary LCNEC. In all patients, the mPFS and mOS were 1.8 and 7.2 months, respectively. The primary endpoint, ORR at 8 weeks, was not met 208
PD-1/CTLA-4 Nivolumab (Nivo) + ipilimumab (Ipi) 2nd Nivo + Ipi Among 94 refractory pulmonary (large-cell only) or GEP-NECs after failure of previous therapy, 46 were GEP-NECs. ORR at 8 weeks, 11.6% in GEP-NEC, and 18.2% in pulmonary LCNEC. In all patients, the mPFS and mOS were 1.9 and 5.8 months, respectively. The primary endpoint, ORR at 8 weeks, was met 208
NCT02834013 2nd II (basket trial, multiple cohorts) Nivo + Ipi Any Among 32 patients with non-pancreatic NEN, 15 and 18 were GEP-NENs and NECs, respectively. ORR, 44% in NEC and 0% in NET. In all patients, mPFS and mOS were 4 and 11 months, respectively. 6-month PFS rate of 44% in NEC and 14% in NET 234
2nd Nivo + Ipi Among 19 patients with G3 NEN, 9 and 11 were GEP-NENs and NECs, respectively. In all patients, ORR, 26%; mPFS, 2.0 months; mOS, 8.9 months 235
NCT02923934 1st II Nivo + Ipi Any Among 29 patients with NENs, 6 and 4 were GEP-NETs and GEP-NECs, respectively. ORR, 50% in GEP-NEC and 33% in GEP-NET. In all patients, ORR, 24%; mPFS, 4.8 months; mOS, 14.8 months 236
NCT03095274 PD-L1/CTLA-4 Durvalumab (Durva) + tremelimumab (Treme) 2nd II (multiple cohorts) Durva + Treme GEP Among 33 patients with GEP-NENs after first line platinum-based chemotherapy, 18 and 15 were GEP-NECs and GEP-G3 NETs, respectively. In all patients, ORR was 9.1%; mPFS, 2.4 months; mOS, 5.9 months. The primary endpoint, the 9-month OS rate, was met 217

Abbreviations: NCT number, ClinicalTrials.gov Identifier; GEP, gastro-entero-pancreatic; NEC, neuroendocrine carcinoma; NEN, neuroendocrine neoplasm; NET, neuroendocrine tumor; UK, unknown; ORR, overall response rate; mPFS, median progression-free survival; mOS, median overall survival; DCR, disease control rate; Ref, reference; Mono, monotherapy