Extra-pulmonary poorly differentiated neuroendocrine carcinoma is rare, and evidence for treatment has been limited. In this article, the evidence behind the cytotoxic chemotherapy choices used for metastatic or unresectable EP-PD-NEC is reviewed. In the first-line setting, etoposide and platinum chemotherapy or irinotecan and platinum have been demonstrated to be equivalent in a large phase III trial. Questions remain regarding the optimal number of cycles, mode of delivery, and the precise definition of platinum resistance in this setting. In the second-line setting, FOLFIRI has emerged as an option, with randomized phase 2 trials demonstrating modest, but significant, response rates. Beyond this, data are extremely limited, and several regimens have been used. Heterogeneity in biological behaviour is a major barrier to optimal EP-PD-NEC management. Available data support the potential role of the Ki-67 index as a predictive biomarker for chemotherapy response. A more personalised approach to management in future studies will be essential, and comprehensive multi-omic approaches are required to understand tumour somatic genetic changes in relation to their effects on the surrounding microenvironment.
肺外低分化神经内分泌癌较为罕见，其治疗证据有限。本文综述了转移性或不可切除性肺外低分化神经内分泌癌细胞毒性化疗方案选择的循证依据。在一线治疗中，一项大型III期临床试验证实依托泊苷联合铂类化疗与伊立替康联合铂类化疗具有等效性。关于最佳治疗周期数、给药方式以及铂类耐药的确切定义等问题仍有待明确。在二线治疗中，FOLFIRI方案已成为一种选择，随机II期试验显示其具有适度但显著的有效率。除此之外，相关数据极为有限，临床中已尝试使用多种治疗方案。生物学行为的异质性是优化肺外低分化神经内分泌癌治疗的主要障碍。现有数据支持Ki-67指数作为化疗反应预测性生物标志物的潜在价值。未来研究中采用更个体化的治疗策略至关重要，需要通过全面的多组学方法来理解肿瘤体细胞遗传学改变及其对周围微环境的影响。
Selection of Chemotherapy in Advanced Poorly Differentiated Extra-Pulmonary Neuroendocrine Carcinoma
肿瘤(癌症)患者之家