背景:食管神经内分泌癌(ENEC)是一种极为罕见的恶性肿瘤。ENEC的临床数据仅限于病例报告和病例系列。需要更多关于其临床特征的信息,管理,和预后。
方法:本研究收集了2010年至2018年病理诊断为ENEC的患者资料。数据包括人口统计信息,临床特征,生存趋势是从监测中获得的,流行病学,和结束结果(SEER)数据库。用STATA/SE15.1、SPSS25.0和GraphPadPrism8进行统计分析。
结果:本研究共纳入283例ENEC患者。ENEC的小细胞和大细胞亚型具有相似的临床特征。食管的下三分之一(58%)是ENEC最常见的位置。在诊断的时候,大多数ENEC患者为AJCC第7期IV期(48.1%).超过一半的ENEC患者发生转移(53.4%),最常见的转移部位是肝脏(37.1%)。与低分化食管鳞状细胞癌(ESCC)相比,由于相似的组织学特征,食管的另一种侵袭性恶性肿瘤有时与ENEC混淆,我们的研究显示了肿瘤位置和转移率的差异,但存活率相似。多因素生存分析显示,ENEC位于食管中段(p=0.013),“脑转移”(p=0.019),和“肝转移”(p<0.001)是预后较差的独立预测因子。“手术”(p=0.003),和“化疗”(p<0.001)与更好的生存率相关。
结论:新诊断的ENEC患者中有很大一部分表现为转移性疾病。生存率低的预测因素包括肿瘤位置,脑转移瘤,和肝转移。ENEC和低分化ESCC具有某些组织学特征,但肿瘤位置和转移率不同。然而,尚未建立标准的治疗策略,但手术和化疗与更好的结局相关.
Esophageal neuroendocrine carcinoma (ENEC) is an extremely rare type of malignancy. Clinical data of ENEC are limited to case reports and case series. More information is needed on its clinical feature, management, and prognosis.
This
study collected information of ENEC patients diagnosed pathologically from 2010 to 2018. Data including demographic information, clinical features, and survival trends were obtained from the Surveillance, Epidemiology, and End Results (SEER) database. Statistical analyses were performed with STATA/SE 15.1, SPSS 25.0, and GraphPad Prism 8.
A total of 283 ENEC patients were included in this
study. The small-cell and large-cell subtypes of ENEC possess similar clinical features. The lower third of the esophagus (58%) was the most common location of ENEC. At the time of diagnosis, most ENEC patients were AJCC 7th stage IV (48.1%). Metastasis occurred in more than half of the ENEC patients (53.4%), and the most common metastatic site was the liver (37.1%). Compared with poorly differentiated
esophageal squamous cell carcinoma (ESCC), another aggressive malignancy of the esophagus sometimes confused with ENEC because of similar histological features, our
study showed differences in tumor location and metastatic rate, but similar poor survival rates. Multivariate survival analysis showed that ENEC located at the middle third of esophagus (p = 0.013), \"Brain metastasis\" (p = 0.019), and \"Liver metastasis\" (p < 0.001) were independent predictors of worse outcomes. \"Surgery\" (p = 0.003), and \"Chemotherapy\" (p < 0.001) were associated with better survival.
A significant proportion of patients with newly diagnosed ENEC presented with metastatic disease. Predictors of poor survival included tumor location, brain metastasis, and liver metastasis. ENEC and poorly differentiated ESCC share certain histological features, but differ in tumor location and metastatic rate. Yet, no standard treatment strategy has been established, but surgery and chemotherapy were related to better outcomes.