Okamoto K, Fujisawa K, Kono K, Ogawa Y, Shimoyama H, Haruta S, Takazawa Y, Ueno M, Udagawa H. Long-term survival with multimodal treatment including conversion surgery for locally advanced esophageal neuroendocrine carcinoma: A case report. World J Gastrointest Surg 2025; 17(6): 107086 [DOI: 10.4240/wjgs.v17.i6.107086]
Corresponding Author of This Article
Kentoku Fujisawa, MD, Department of Gastroenterological Surgery, Toranomon Hospital, 2-2-2 Toranomon, Minato-ku, Tokyo 105-8470, Tōkyō, Japan. fkentoku@gmail.com
Research Domain of This Article
Surgery
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Kazuya Okamoto, Kentoku Fujisawa, Yusuke Ogawa, Hayato Shimoyama, Shusuke Haruta, Masaki Ueno, Harushi Udagawa, Department of Gastroenterological Surgery, Toranomon Hospital, Tokyo 105-8470, Tōkyō, Japan
Kei Kono, Yutaka Takazawa, Department of Pathology, Toranomon Hospital, Tokyo 105-8470, Tōkyō, Japan
Author contributions: Okamoto K and Fujisawa K designed the case report and drafted the manuscript; Kono K and Takazawa Y acquired data; Okamoto K, Fujisawa K, Ogawa Y, Shimoyama H, Haruta S, Ueno M, and Udagawa H managed the patient’s care and acquired data; Fujisawa K, Ueno M, and Udagawa H supervised the work; and all authors have reviewed the manuscript and read and approved the final manuscript.
Informed consent statement: All study participants, or their legal guardian, provided informed written consent prior to study enrollment.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Kentoku Fujisawa, MD, Department of Gastroenterological Surgery, Toranomon Hospital, 2-2-2 Toranomon, Minato-ku, Tokyo 105-8470, Tōkyō, Japan. fkentoku@gmail.com
Received: March 18, 2025 Revised: April 4, 2025 Accepted: May 14, 2025 Published online: June 27, 2025 Processing time: 73 Days and 11 Hours
Abstract
BACKGROUND
Esophageal neuroendocrine carcinoma (NEC), a rare and aggressive malignancy with a poor prognosis, is often diagnosed at an advanced stage. The optimal treatment strategy for locally advanced and recurrent esophageal NEC remains unclear, and conversion surgery has only been reported for a few cases. Herein, we present the case of a 66-year-old male with locally advanced esophageal NEC initially diagnosed as squamous cell carcinoma.
CASE SUMMARY
The patient underwent induction chemotherapy with docetaxel, cisplatin, and 5-fluorouracil, followed by conversion surgery, including subtotal esophagectomy, three-field lymph node dissection, and distal pancreatectomy with splenectomy, due to infiltration of the pancreas by the No. 11p lymph node. Postoperative pathological findings revealed a large cell-type NEC without a squamous cell carcinoma component, suspected to be a mixed neuroendocrine/non-neuroendocrine neoplasm. Hepatic metastasis was diagnosed within one month of surgery. Despite the administration of four courses of irinotecan + cisplatin chemotherapy, the treatment effect was considered a ‘progressive disease’. After a multidisciplinary discussion, the patient underwent partial liver resection, followed by second-line chemotherapy with amrubicin. The patient achieved three-year survival with no new recurrence.
CONCLUSION
This case highlights the potential of multimodal treatment for long-term survival in advanced esophageal NEC.
Core Tip: We used a multimodal treatment approach for locally advanced unresectable esophageal neuroendocrine carcinoma (NEC). Two aspects of this case are noteworthy. First, because NEC was finally diagnosed through postoperative pathological examination, the chemotherapy was administered based on the initial presumption of squamous cell carcinoma. This facilitated complete resolution of the squamous cell carcinoma component, allowing for subsequent targeting of the remaining NEC component. Second, following chemotherapy, no new distant metastases developed, with effective tumor control for the primary lesion and metastatic lymph nodes. Thus, surgery can be effective if resection is feasible, even for locally advanced NEC.
