Objective: To investigate the clinicopathological characteristics and prognostic factors of neuroendocrine carcinoma (NEC) of the cervix. Methods: Eight-two patients diagnosed as NEC of cervix from 2008 to 2016 at West China Second University Hospital were analyzed retrospectively including HE slide review, immunohistochemistry and HPV genotyping. Survival analysis was performed using Kaplan-Meier and Cox regression model. Results: The age of the patients ranged from 16 to 75 years with mean age of 43 years. According to International Federation of Gynecology and Obstetrics (FIGO) clinical stage, 52 cases were in stageⅠ, 10 cases in stageⅡ, 14 cases in stage Ⅲ and 6 cases in stage Ⅳ. The tumor size ranged from 0.5 to 6.5 cm, with an average of 3.6 cm. Upon histopathologic review, 74 tumors were classified as small cell carcinoma; 7 tumors as large cell NEC, and 1 as atypical carcinoid. Further evaluation showed 52 cases (63.4%) with deep stromal invasion, 73 cases (89.0%) with lymph-vascular invasion, and 28 cases (34.2%) with pelvic and (or) para-aortic lymph nodes involvement. Immunohistochemical staining showed neuroendocrine markers Syn, CD56, NSE, S-100 protein and CgA were positive in 93.9%, 84.2%, 74.4%, 64.6% and 51.2% of cases, respectively. The results of HPV-DNA detection were positive in 72 cases, high-risk HPV types were 70 cases and 49 cases were HPV18 positive. The median follow-up time was 37 months (range, 6-101 months). Twenty-nine cases were found recurrence or metastasis, including 23 cases of death. The univariate analysis demonstrated that the tumor size, lymph node metastasis, infiltration depth, FIGO stage and whether the lesion confined to the uterus were significant prognostic factors(P<0.05). Cox multivariate analysis showed that lymph node metastasis and FIGO stage were independent prognostic factors of NEC(P<0.05). Conclusions: NEC of the cervix is a highly aggressive malignancy with poor prognosis. The tumor is associated with HPV infection, especially type 18. Small cell NEC is the most common type of cervical NEC. Diagnosis is based on histological and immunohistochemical examination. Lymph node metastasis and FIGO stage are the independent factors affecting prognosis.
目的： 探讨宫颈神经内分泌癌(neuroendocrine carcinoma of the cervix，NEC)的临床病理特征及预后影响因素。 方法： 回顾性分析2008至2016年就诊于四川大学华西第二医院的82例宫颈NEC患者临床资料，采用HE染色、免疫组织化学染色及人乳头状瘤病毒(HPV)-DNA分型检测，随访资料采用Kaplan-Meier法和Cox比例风险回归模型进行生存分析。 结果： 本组患者年龄16～75岁，平均年龄43岁。国际妇产科联盟(FIGO)分期：Ⅰ期52例，Ⅱ期10例，Ⅲ期14例，Ⅳ期6例。肿瘤大小0.5～6.5 cm，平均3.6 cm。组织学分类：小细胞NEC 74例，大细胞NEC 7例，不典型类癌仅1例。肿瘤浸润宫颈间质深层52例(63.4%)，癌栓73例(89.0%)，盆腔和/或腹主动脉旁淋巴结转移28例(34.2%)。免疫组织化学染色显示癌细胞表达神经内分泌标志物突触素、CD56、神经元特异性烯醇化酶、S-100蛋白及嗜铬素粒A，阳性率分别为93.9%、84.2%、74.4%、64.6%和51.2%。72例HPV-DNA检测结果阳性，其中高危型70例，49例为HPV18阳性。患者中位随访时间37个月(6～101个月)，29例患者出现肿瘤复发及转移，其中死亡23例。单因素生存分析表明肿瘤大小、浸润深度、是否局限于宫体、是否有淋巴结转移及FIGO分期是患者预后的影响因素(均P<0.05)，Cox比例风险回归分析结果显示淋巴结转移及FIGO分期是影响患者预后的独立因素(均P<0.05)。 结论： 宫颈NEC是一组与HPV感染尤其是高危型HPV相关的高恶性肿瘤，其组织学类型以小细胞癌最常见，明确诊断有赖于形态学及免疫组织化学染色。宫颈NEC预后不良，淋巴结转移及FIGO分期是影响预后的独立因素。.
Keywords: Carcinoma, neuroendocrine; Prognosis; Uterine cervical neoplasms.