宫颈神经内分泌癌17例临床分析

Clinical analysis of 17 cases of cervical neuroendocrine cancer

目的:探讨宫颈神经内分泌癌(NECC)的临床病理特征、治疗及预后特点。方法:回顾收集2008年5月至2018年11月北京大学人民医院收治的17例NECC患者的临床病例资料,采用Kaplan-Meier法及Log-rank检验进行生存分析。结果:患者年龄32~64岁,平均(50.3±8.0)岁,中位年龄49岁。肿瘤直径0.2~9cm,平均(2.9±2.2)cm。单纯神经内分泌癌8例,鳞状细胞癌伴神经内分泌分化2例,腺癌伴神经内分泌分化3例,腺鳞癌伴神经内分泌分化2例,宫颈高级别鳞状上皮内病变伴神经内分泌癌2例。16例行根治性手术者,其中5例接受新辅助化疗,5例术后辅助化疗,7例术后接受放疗;余1例未手术者接受放化疗。15例患者获得最终随访,随访时间2~119个月。16例手术患者中,12例无复发及转移;1例发生远处转移(肝腰椎转移)但仍存活;3例死于多发转移所致的多器官功能衰竭(2例肝、腰椎转移,1例腹腔、心膈角多发淋巴结转移);1例未手术患者(IVb期)随访22个月因多器官功能衰竭死亡。单因素分析显示,年龄(≥40岁)、肿瘤直径(≥4cm)、盆腔淋巴结转移、淋巴脉管间隙浸润、术后放疗、辅助化疗、新辅助化疗、FIGO分期(≥IIa期)均不是影响患者预后的危险因素。结论:NECC发病率低,恶性程度高,确诊依赖于病理,病因及影响预后因素需进一步探讨。广泛子宫切除+双附件+盆腔和(或)腹主动脉旁淋巴结切除是早期患者的主要治疗方案,辅助化疗具有重要意义。

Objective:To discuss the clinicopathological features,treatment and prognosis of neuroendocrine carcinoma of the cervix(NECC).Methods:Clinical case datas of 17 NECC patients admitted to Peking University People's Hospital from May 2008 to Nov.2018 were retrospectively collected.Survival analysis was performed by Kaplan-meier method and Log-rank test.Results:The patients were 32 to 64 years old,with an average age of(50.3±8.0)years old and a median age of 49 years old.The tumor diameter was 0.2~9cm,with an average of(2.9±2.2)cm.There were 8 cases of pure neuroendocrine carcinoma,2 cases of squamous cell carcinoma with neuroendocrine differentiation,3 cases of adenocarcinoma with neuroendocrine differentiation,2 cases of adenocarcinoma with neuroendocrine differentiation,and 2 cases of high-grade squamous intraepithelial lesion of the cervix with neuroendocrine carcinoma.Among the 16 patients undergoing radical surgery,5 received neoadjuvant chemotherapy,5 received postoperative adjuvant chemotherapy,7 received postoperative radiotherapy,and the remaining 1 received radiochemotherapy.Fifteen patients were finally followed up for 2~119 months.Among the 16 patients,12 had no recurrence or metastasis.1 patient survived with distant metastasis(hepatic and lumbar metastasis).Three patients died of multiple organ failure caused by multiple metastases(2 patients with liver and lumbar metastases,1 patient with multiple lymph node metastases in abdominal cavity and diaphragmatic Angle).One unoperated patient(IVb stage)died of multiple organ failure after 22 months of follow-up.Univariate analysis showed that age(≥40 years old),tumor diameter(≥4cm),pelvic lymph node metastasis,lymphatic and vascular gap infiltration,postoperative radiotherapy,adjuvant chemotherapy,neoadjuvant chemotherapy,FIGO stage(≥IIa stage)were not risk factors affecting the prognosis of patients.Conclusion:The incidence of NECC is low,the degree of malignancy is high,the diagnosis depends on pathology,and the etiology and prognostic factors of patients need more medical history analysis to further explore,extensive hysterectomy+bilateral adnexa+pelvic and/or para-aortic lymph node resection is the main treatment plan for early patients,and adjuvant chemotherapy is of great significance.