Neuroendocrine cancer articles from across Nature Portfolio

Phaeochromocytomas and paragangliomas (PPGL) are rare endocrine tumours that can affect paediatric patients as well as adults. In this first international Consensus Statement on PPGL in paediatric patients, the authors discuss the diagnosis, management and long-term surveillance of these tumours in children and adolescents.

Immune checkpoint inhibitors have been incorporated as the standard of care for some high-grade neuroendocrine neoplasms (NEN), but their efficacy in digestive NEN is still unclear. Here the authors report the results of a phase II trial of nivolumab (anti-PD1) in combination with platinum-doublet chemotherapy (carboplatin-etoposide) as first-line treatment in patients with unresectable or metastatic advanced grade 3 NENs of gastro-enteropancreatic or unknown origin.

Enhancer of zeste homolog 2 (EZH2) has been implicated as a driver of disease progression and resistance to hormonal therapies. Here, the authors focus on EZH2 in two subtypes of advanced prostate cancer and report how it modulates the bivalent genes thereby leading to forward differentiation after being targeted in neuroendocrine prostate cancer.

Here the authors perform a high-throughput screen to identify compounds that stimulate or repress the splicing of neuronal microexons. One of these compounds rescues the splicing of several microexons in the brains of mice haploinsufficient for Srrm4.

The NETTER-2 trial provides the first phase III evidence that ¹⁷⁷Lu-dotatate is active as first-line therapy in patients with gastroenteropancreatic neuroendocrine tumours with a Ki-67 proliferative index of 10–55%. This approach is an important addition to the first-line therapeutic armamentarium, although treatment selection based on patient-specific and tumour-specific characteristics remains appropriate.

In this Journal Club, Taboada and Riechelmann discuss the importance of a study outlining a novel neuroendocrine neoplasm classification system.