目的:宫颈大细胞神经内分泌癌(LCNEC)是一种与不良预后相关的罕见肿瘤。关于致癌作用和治疗选择的知识很少,而新的治疗靶向方法是有限的。
方法:我们从开始到2020年6月对四个电子数据库进行了系统评价。符合条件的研究包括所有涉及LCNEC患者生存结果的报告。
结果:共确定了31个病例研究,包括87例LCNEC患者。患者年龄中位数为41岁(范围:21-81)。大多数女性(76.3%)患有FIGOI-II期疾病。总的来说,72.0%接受过手术,70.1%接受化疗,50.7%接受放疗。在已知HPV状态的13名患者中,15%为HPV阴性。中位总生存期(OS)为24个月(范围:0.5-151),3年和5年OS分别为42%和29%,分别。在多变量分析中,仅手术和淋巴结清扫术与生存率显着相关(手术OS:HR0.14;95%C.I.:0.03-0.71,p=0.018/手术PFS:HR0.23,95%C.I.0.06,0.92,p=0.037/淋巴结清扫术OS:HR0.26,95%C.I.0.07-0.98,p=0.046/淋巴结清扫术:HR0.30,95%C.I.年龄,化疗或放疗对生存率无显著影响,但低分期与生存率提高相关.
结论:宫颈LCNECs总体预后较差,尽管他们的初步介绍相对较早。与化疗和放疗相比,手术和淋巴结清扫术似乎显著影响生存率。这似乎对预后没有显著影响。前瞻性多中心癌症登记是必要的,以改善这种罕见疾病的治疗选择。
OBJECTIVE: Large cell neuroendocrine carcinoma (LCNEC) of the cervix represents a rare tumour entity associated with poor prognosis. Knowledge about carcinogenesis and therapeutic options is scarce, while novel therapeutic targeted approaches are limited.
METHODS: We performed a systematic
review of four electronic databases from inception to June 2020. Eligible studies included all reports that addressed survival outcomes of women with LCNEC.
RESULTS: A total of 31 case studies including 87 LCNEC patients were identified. Median patients\' age was 41 years (range: 21-81). Most women (76.3%) had FIGO stage I-II disease. Overall, 72.0% had surgery, 70.1% received chemotherapy and 50.7% received radiotherapy. Of 13 patients with known HPV-status, 15% were HPV negative. Median overall survival (OS) was 24 months (range: 0.5-151), with 3- and 5-year OS of 42% and 29%, respectively. In multivariate analyses, only surgery and lymphadenectomy significantly associated with survival (Surgery OS: HR 0.14; 95% C.I:0.03-0.71, p = 0.018 / Surgery PFS: HR 0.23, 95% C.I. 0.06, 0.92, p = 0.037 / Lymphadenectomy OS: HR 0.26, 95% C.I. 0.07-0.98, p = 0.046 / Lymphadenectomy PFS: HR 0.30, 95% C.I. 0.09-0.98, p = 0.046). Age, chemotherapy or radiotherapy did not significantly impact survival, but lower stage was associated with improved survival.
CONCLUSIONS: Cervical LCNECs overall have a poor prognosis, despite their relatively early-stage initial presentation. Surgery and lymphadenectomy appear to significantly affect survival in contrast to chemotherapy and radiotherapy, which appear to have no significant effect on prognosis. Prospective multicentre cancer registries are warranted to improve treatment options for this rare disease.