53例肺外小细胞癌的临床分析

Extrapulmonary Small Cell Carcinoma in 52 Patients

背景与目的:小细胞癌主要发生于肺内,肺外小细胞癌(extrapulmonarysmallcellcarcinoma,ESCC)被认为是一类与肺小细胞癌不同的临床病理类型。本研究目的在于探讨ESCC的临床特征、治疗及预后。方法:回顾性分析1985年1月至2005年12月中山大学肿瘤防治中心收治的经病理证实的53例ESCC患者的临床资料,分析本组患者的发病情况,发病部位,病理诊断,肿瘤分期,治疗方法及预后。结果:53例ESCC患者中,男性39例,女性14例。中位年龄53岁(27～76岁)。53例ESCC中食管33例(62.3%),子宫颈5例,喉4例,鼻咽3例,上颌窦2例,直肠2例,舌下腺、甲状腺、胸膜和肝脏各1例。局限期患者40例(75.5%),广泛期患者13例(24.5%)。对于广泛期患者多给予含铂方案化疗,总有效率为69.2%;对于局限期患者采取不同的治疗模式,即手术+化疗+放疗者7例,手术+放疗者3例,手术+化疗者18例,放疗+化疗者6例,单独放疗者4例,单独化疗者2例。全组患者中位生存期(mediansurvivaltime,MST)为20个月,1年和3年生存率(overallsurvival,OS)分别为41.3%和31.4%。局限期患者和广泛期患者的MST分别为26个月和15个月,1年OS分别为51.1%和14.4%,3年OS分别为42.5%和0(P=0.017)。结论:ESCC可发生于人体不同部位,以食管ESCC最多见。局限期ESCC常采用综合治疗模式,化疗仍是广泛期ESCC的主要治疗手段。总体上,局限期ESCC预后明显较广泛期ESCC好。

BACKGROUND ＆ OBJECTIVE： The majority of small cell carcinoma occurs in the lung. Extrapulmonary small cell carcinoma （ESCC） has been recognized as a clinicopathologic entity distinct from small cell carcinoma of the lung. The study was to investigate the clinical characteristics, therapy, and prognosis of ESCC. METHODS： The medical records, from Jan. 1985 to Dec, 2005, of 53 patients with pathologically proved ESCC were analyzed retrospectively. RESULTS： Of the 53 patients, 39 were men and 14 were women, with the median age of 53 years （range, 27-76 years）. Of the 53 cases of ESCC, 33 （62,3%） were detected in the esophagus, 5 in the cervix, 4 in the larynx, 3 in the pharynx, 2 in the upper sinus, 2 in the rectum and sublingual gland, 1 in the thyroid gland, 1 in the pleura, and 1 in the liver. Forty patients （75.5%） had limited disease （LD） and 13 （24.5%） had extensive disease （ED）. Patients with ED mostly received platinum-based chemotherapy, for which the response rate was 69.2%. Patients with LD were treated with a variety of therapeutic modalities： 7 were treated with surgery plus radiochemotherapy, 3 with surgery plus radiotherapy, 18 with surgery plus chemotherapy, 6 with radiotherapy plus chemotherapy, 4 with radiotherapy alone, and 2 with chemotherapy alone. The median survival time （MST） was 20 months for all patients, and the 1- and 3- year survival rates were 41.3% and 31.4%. MST for patients with ED and LD were 15 months and 26 months, respectively, and 1- and 3-year survival rates were 51.1% vs. 14.4%, and 42.5% vs. 0% （P=0.017）. CONCLUSIONS： ESCC is identified in various sites, with the most common primary site being the esophagus. Multimodality therapy has become increasingly used for the majority of patients with LD-ESCC. Combination chemotherapy has been a major treatment for patients with ED-ESCC. Generally, the prognosis of LD-ESCC is significantly superior to ED-ESCC.