Department of Medical Sciences, Uppsala University, Akademiska Sjukhuset ing 100, 75185, Uppsala, Sweden.
Curr Treat Options Oncol. 2021 Jun 10;22(8):68. doi: 10.1007/s11864-021-00866-9.
Treatment recommendations for advanced gastroenteropancreatic neuroendocrine carcinomas (GEP-NEC) are based on uncontrolled, mainly retrospective data. Chemotherapy can offer palliative relief, but long-lasting complete responses or cures are rare. The European Neuroendocrine Tumour Society (ENETS) and European Society for Medical Oncology (ESMO) recommend platinum-based chemotherapy as first-line treatment. This has been the golden standard since the late 1980s and has been evaluated in mostly retrospective clinical studies. However, progression is inevitable for most patients. Unfortunately, data on effective second-line treatment options are scant, and ENETS and ESMO recommendations propose fluorouracil- or temozolomide-based chemotherapy schedules. As such, there is a huge unmet need for improved care. Improved knowledge on GEP-NEC biology may provide a pathway towards more effective interventions including chemotherapy, targeted gene therapy, peptide receptor radionuclide therapy, as well as immune checkpoint inhibitors. The review summarises this current state of the art as well as the most promising developments for systemic therapy in GEP-NEC patients.
晚期胃肠胰神经内分泌癌（GEP-NEC）的治疗建议基于无对照的、主要是回顾性的数据。化疗可以提供姑息缓解，但长期完全缓解或治愈的情况很少见。欧洲神经内分泌肿瘤学会（ENETS）和欧洲肿瘤内科学会（ESMO）推荐基于铂类的化疗作为一线治疗。自 20 世纪 80 年代末以来，这一直是黄金标准，并在大多数回顾性临床研究中进行了评估。然而，对于大多数患者来说，疾病进展是不可避免的。不幸的是，关于有效二线治疗选择的数据很少，ENETS 和 ESMO 的建议提出了基于氟尿嘧啶或替莫唑胺的化疗方案。因此，存在着巨大的未满足的治疗需求。对 GEP-NEC 生物学的深入了解可能为包括化疗、靶向基因治疗、肽受体放射性核素治疗以及免疫检查点抑制剂在内的更有效的干预措施提供途径。本文综述了 GEP-NEC 患者系统治疗的这一最新进展以及最有前途的进展。
