宫颈神经内分泌癌治疗的新进展

New Progress in Treatment of Neuroendocrine Carcinoma of the Cervix

宫颈神经内分泌癌(neuroendocrine carcinoma of the cervix,NECC)是一种罕见的恶性程度极高的妇科肿瘤。临床上对NECC的组织来源、发病机制并不明确。NECC没有标准的治疗方案,多是结合宫颈鳞状细胞癌、腺癌和小细胞肺癌的治疗经验制定治疗方案。近年来大样本队列基因组研究显示,NECC与宫颈外神经内分泌癌具有不同的基因组学特征,对NECC与宫颈外神经内分泌癌之间的生物学和治疗相关性提出了质疑。由于NECC的罕见性,获得足够数量的患者进行疗效试验的可能性很低,这阻碍了NECC标准治疗方案的制定。基因检测能为NECC靶向药物的个体化治疗提供策略。多项研究结果显示,NECC具有潜在的可调控的治疗靶点,免疫疗法与放射疗法结合可以延长晚期复发性NECC患者的长期生存。综述NECC的分子特征、靶向药物及免疫治疗的研究进展。

Neuroendocrine carcinoma of the cervix(NECC)is a rare and highly malignant gynecological carcinoma.Clinically,the origin and pathogenesis of NECC are not clear.There is no standard treatment protocol for NECC.Most of the treatments are based on the experience of cervical squamous cell carcinoma,adenocarcinoma and small cell lung cancer.Recent genomic studies in large sample cohorts have shown that different genomic characteristics between NECC and extracervical neuroendocrine carcinoma,raising questions about the biological and therapeutic correlation between NECC and extracervical neuroendocrine carcinoma.Due to the rarity of NECC,it is difficult to obtain sufficient numbers of patients for efficacy trials,making it difficult to develop a standard treatment regimen for NECC.Genetic testing provides a strategy for individualized treatment of NECC with targeted drugs.Multiple studies have shown that NECC has potential modifiable therapeutic targets,and the combination of immunotherapy and radiation therapy can prolong long-term survival in patients with advanced recurrent NECC.This article reviews the molecular characteristics,targeted drugs and immunotherapy of NECC.