pNETs frequently contain mutations in MEN1, ATRX, DAXX, and the PI3K/AKT/mTOR pathway. However, more data are needed to determine whether this information can predict response to standard treatments, such as CAPTEM. At NANETS 2022, Hendifar et al. presented retrospective data on 25 patients with well-differentiated grade 1 and 2 pNETs who had received CAPTEM as first- or second-line treatment and whose tumors had been molecularly characterized through next-generation sequencing (NGS).

Chemotherapy is currently the SoC first-line treatment for high-grade neuroendocrine neoplasms (HG-NENs), even though it only provides modest benefits in OS and PFS. Given the lack of therapeutic options for metastatic NEN patients and the promising antitumor activity of immunotherapy demonstrated across several solid ­cancer types, the efficacy of pembrolizumab monotherapy was investigated in an open-label, nonrandomized phase II study in patients with metastatic extra-pulmonary HG-NEN (Ki67 >20 %).