Division of Gastroenterology, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan; Department of Medicine, Faculty of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan.
Department of Gastroenterology and Hepatology, Linkou Chang Gung Memorial Hospital and Chang Gung University, Taoyuan, Taiwan; Department of Gastroenterology and Hepatology, New Taipei Municipal TuCheng Hospital and Chang Gung University, New Taipei City, Taiwan.
J Formos Med Assoc. 2021 Jan;120(1 Pt 2):508-514. doi: 10.1016/j.jfma.2020.06.024. Epub 2020 Jun 27.
BACKGROUND & AIMS: Esophageal neuroendocrine tumors (NET) are very rare and mostly carcinomic, carrying poor prognosis. There is still no guideline or consensus on the treatment for esophageal NET.
Patients with histologically-proven esophageal neuroendocrine tumor were recruited from 9 hospitals in Taiwan between 2002 and 2017. Clinical, laboratory, radiological, endoscopic, pathological data, treatment strategies, follow-up periods, and survivals were collected retrospectively.
In total, 39 esophageal NET were analyzed and 38 were neuroendocrine carcinoma (NEC). Sixteen (41%) patients had mixed components with either adenocarcinoma (N = 9, 23%) or squamous-cell carcinoma (SCC) (N = 7, 18%). 64.1% of the patients experienced dysphagia and ulcerative mass was the most comment endoscopic finding. There was a higher proportion of drinkers (54.1%), betel chewers (21.6%) and smokers (64.9%) among the patients than in the general population in Taiwan. Five patients (12.8%) had been diagnosed with other cancers. Definite chemoradiotherapy (N = 14, 35.9%) and surgery (N = 7, 17.9%) were the major treatment. Patients with Ki-67% above the median level (50%) in the tumors tended to have worse survival (P = 0.06). However, presence of mixed component was not a significant survival predictor in our study (P = 0.56).
Mixed component of an esophageal NET is commonly observed. Staged workup and the principle of treatment can follow that for the common cancer type of esophagus. The risk factors and behaviors of esophageal NEC in Taiwan seem to be similar to that of esophageal SCC.
食管神经内分泌肿瘤（NET）非常罕见，大多为癌性，预后较差。目前对于食管 NET 的治疗尚无指南或共识。
我们从 2002 年至 2017 年在台湾的 9 家医院招募了经组织学证实的食管神经内分泌肿瘤患者。回顾性收集了临床、实验室、影像学、内镜、病理学数据、治疗策略、随访时间和生存情况。
共分析了 39 例食管 NET，其中 38 例为神经内分泌癌（NEC）。16 例（41%）患者存在混合成分，包括腺癌（9 例，23%）或鳞状细胞癌（7 例，18%）。64.1%的患者出现吞咽困难，溃疡性肿块是最常见的内镜表现。与台湾一般人群相比，患者中饮酒者（54.1%）、嚼槟榔者（21.6%）和吸烟者（64.9%）的比例更高。5 例（12.8%）患者被诊断出患有其他癌症。明确的放化疗（N=14，35.9%）和手术（N=7，17.9%）是主要治疗方法。肿瘤 Ki-67%高于中位数（50%）的患者生存状况较差（P=0.06）。然而，在本研究中，混合成分并不是生存的显著预测因素（P=0.56）。
食管 NET 常存在混合成分。分期检查和治疗原则可遵循食管癌的常规治疗方法。台湾食管 NEC 的危险因素和行为似乎与食管 SCC 相似。
