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Treatment strategies for neuroendocrine carcinoma of the upper digestive tract



Neuroendocrine carcinoma (NEC) of the esophagus and the stomach is aggressive. The purpose of this study was to determine the optimal therapeutic strategy.


Both clinicopathological factors and treatment results were examined in 34 patients with immunohistochemically diagnosed NEC of the upper gastrointestinal tract (esophagus 22; stomach 12).


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.


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.

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We thank Brian Quinn for assisting with the preparation of the manuscript.


This work was supported in part by a Grant-in-Aid from the Ministry of Education, Culture, Sport, Science and Technology of Japan (Grant No. 18K08638) and the Fujii Setsuro Memorial Foundation.

Author information

MM: conceptualizing and designing the study, and writing the manuscript. KT: pathological diagnosis and advice. MK: collecting the data. TN: collecting the data. HU: collecting the data. MS: analyzing the data. MO: contributing intellectual inputs mainly for chemotherapy. MI: contributing intellectual inputs mainly for surgical therapy. KS: contributing intellectual inputs mainly for surgical therapy. TE: contributing intellectual inputs mainly for chemotherapy. YT: supervising the entire study.

Correspondence to Masaru Morita.

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The authors declare that they have no conflict of interest in association with the present study.

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All procedures performed in this study involving human participants were in accordance with ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical statements.

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Informed consent was obtained from individual participants in the study.

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Morita, M., Taguchi, K., Kagawa, M. et al. Treatment strategies for neuroendocrine carcinoma of the upper digestive tract. Int J Clin Oncol (2020).

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  • Neuroendocrine carcinoma
  • Esophagus
  • Stomach