Neuroendocrine neoplasms (NENs) are biologically diverse tumors. This article is a critical review of recent evidence focusing on systemic therapies (through mid-2025). We summarize what is most practice-relevant and where gaps remain. In diagnosis, somatostatin-receptor PET/CT has largely replaced older scintigraphy, and adding FDG PET can flag more aggressive disease. Blood-based tests and selected tissue markers (e.g., MGMT, DAXX/ATRX/ALT) show promise but require cautious interpretation in routine care. In treatment, radioligand therapy (PRRT) is used earlier in appropriate receptor-positive disease; cabozantinib improves progression-free survival after prior therapy; and belzutifan offers a biomarker-guided option for malignant pheochromocytoma/paraganglioma. Immunotherapy remains limited to defined subsets, including high-grade neoplasms. We appraise strengths and limitations of key trials, note issues of access and toxicity, and highlight active areas in development (SSTR antagonists, alpha emitters, and dose-guided PRRT). Our goal is to provide a concise, evidence-based map of the field to support informed clinical judgment and future research priorities.
神经内分泌肿瘤（NENs）是一类生物学特性多样的肿瘤。本文对截至2025年中期的全身性治疗最新证据进行了批判性评述，重点总结最具临床实践价值的内容及现存研究空白。在诊断方面，生长抑素受体PET/CT已基本取代传统闪烁显像技术，联合FDG PET可有效识别更具侵袭性的病变。血液检测及特定组织标志物（如MGMT、DAXX/ATRX/ALT）虽展现应用前景，但在常规诊疗中需审慎解读。治疗领域进展包括：放射性配体疗法（PRRT）在适宜受体阳性患者中应用时机前移；卡博替尼可改善经治患者的无进展生存期；贝组替凡为恶性嗜铬细胞瘤/副神经节瘤提供了生物标志物指导的治疗选择。免疫治疗目前仍限于特定亚群，包括高级别神经内分泌肿瘤。本文评估了关键临床试验的优势与局限，关注治疗可及性与毒性问题，并着重指出当前重点研发方向（SSTR拮抗剂、α粒子疗法及剂量引导的PRRT）。旨在通过简明扼要的循证医学图谱，为临床决策制定与未来研究方向提供参考依据。
Emerging Diagnostics and Therapies in Neuroendocrine Neoplasms: A Critical Review
肿瘤(癌症)患者之家