BACKGROUND Neuroendocrine carcinoma (NEC) of the esophagus and the stomach is aggressive. The purpose of this study was to determine the optimal therapeutic strategy. METHODS Both clinicopathological factors and treatment results were examined in 34 patients with immunohistochemically diagnosed NEC of the upper gastrointestinal tract (esophagus 22; stomach 12). RESULTS Twenty-nine tumors showed protruding and localized type, like submucosal tumor. Esophagectomy and gastrectomy were performed in six and eight patients, respectively. Among the six patients with esophageal NEC, three with node metastasis developed recurrence within seven months, while the other three (pT1bN0) had no recurrence. Regarding gastric NEC, three patients with pT3N1 or 2 tumor received adjuvant chemotherapy and achieved a 5-year survival. However, the other five experienced recurrence after gastrectomy. Systemic chemotherapy was performed as the main treatment for 18 patients with advanced NEC. The median survival was 10 months after initial chemotherapy. No marked differences in the response were recognized between the 14 cases with esophageal NEC and the 4 with gastric NEC. The median survival was 14.3 and 5.3 months for the 11 effective and 7 non-effective patients, respectively. CONCLUSIONS A macroscopically unique appearance, like submucosal tumor, suggests the possibility of NEC. Esophagectomy is an effective treatment option for limited-stage NEC without node metastasis, while gastrectomy followed by adjuvant chemotherapy may be effective for NEC even with node metastasis when R0 resection can be achieved. Systemic chemotherapy is relatively effective for advanced NEC, although early progression frequently develops.

中文翻译：

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上消化道神经内分泌癌的治疗策略。

背景技术食道和胃的神经内分泌癌（NEC）是侵袭性的。这项研究的目的是确定最佳的治疗策略。方法对34例经免疫组织化学诊断为上消化道NEC（食道22；胃12）的患者的临床病理因素和治疗结果进行了检查。结果29例肿瘤表现为突出和局限型，如粘膜下肿瘤。分别在六名和八名患者中进行了食管切除术和胃切除术。在6例食管NEC患者中，有3例发生淋巴结转移，在7个月内复发，而其他3例（pT1bN0）没有复发。对于胃NEC，三名患有pT3N1或2肿瘤的患者接受了辅助化疗，并获得了5年生存率。然而，其余五个在胃切除术后复发。全身化疗是18例晚期NEC患者的主要治疗方法。中位生存期为初始化疗后10个月。在14例食管NEC患者和4例胃NEC患者之间，反应没有明显差异。11名有效和7名无效患者的中位生存期分别为14.3个月和5.3个月。结论肉眼可见的独特外观，如粘膜下肿瘤，提示NEC的可能性。食管切除术是有限期无结节转移的NEC的有效治疗选择，而胃切除术后辅以化学疗法即使达到R0切除的结节转移也可能对NEC有效。全身化疗对于晚期NEC相对有效，