Yuan Zhong-Yu, Guan Zhong-Zhen, Zhou Zhong-Mei, Xia Yi, Huang Wan-Zhong, Yang Xiu-Li
State Key Laboratory of Oncology in South China, Guangzhou, Guangdong, 510060, P. R. China.
Ai Zheng. 2006 Sep;25(9):1131-3.
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.
The medical records, from Jan. 1985 to Dec. 2005, of 53 patients with pathologically proved ESCC were analyzed retrospectively.
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 ).
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.
大多数小细胞癌发生于肺部。肺外小细胞癌（ESCC）已被确认为一种有别于肺小细胞癌的临床病理实体。本研究旨在探讨ESCC的临床特征、治疗方法及预后情况。
回顾性分析1985年1月至2005年12月期间53例经病理证实为ESCC患者的病历资料。
53例患者中，男性39例，女性14例，中位年龄53岁（范围27 - 76岁）。53例ESCC中，33例（62.3%）发生于食管，5例发生于宫颈，4例发生于喉，3例发生于咽，2例发生于上颌窦，2例发生于直肠和舌下腺，1例发生于甲状腺，1例发生于胸膜，1例发生于肝脏。40例（75.5%）为局限性疾病（LD），13例（24.5%）为广泛性疾病（ED）。ED患者大多接受以铂类为基础的化疗，其有效率为69.2%。LD患者接受多种治疗方式：7例接受手术加放化疗，3例接受手术加放疗，18例接受手术加化疗，6例接受放疗加化疗，4例仅接受放疗，2例仅接受化疗。所有患者的中位生存时间（MST）为20个月，1年和3年生存率分别为41.3%和31.4%。ED和LD患者的MST分别为15个月和26个月，1年和3年生存率分别为51.1%对14.4%，42.5%对0%（P = 0.017）。
ESCC可发生于多个部位，最常见的原发部位是食管。多模式治疗越来越多地应用于大多数LD - ESCC患者。联合化疗一直是ED - ESCC患者的主要治疗方法。总体而言，LD - ESCC的预后明显优于ED - ESCC。
