Oligometastatic Mixed Neuroendocrine Adenocarcinoma of the Esophago-Gastric Junction: A Case of Successful Multidisciplinary Management, the Lessons Learnt and Review of the Literature

TL;DR

A rare case of mixed neuroendocrine adenocarcinoma at the esophago-gastric junction was successfully treated with a combination of chemotherapy, surgery, and immunotherapy, leading to long-term remission.

Contribution

This case highlights the effectiveness of a multidisciplinary approach in managing oligometastatic mixed neuroendocrine tumors of the esophago-gastric junction.

Findings

A patient with mixed neuroendocrine adenocarcinoma achieved disease-free status after multimodal treatment including surgery and chemotherapy.

Literature review showed that systemic chemotherapy for neuroendocrine components can lead to significant responses and improved survival when followed by conversion surgery.

Most mixed neuroendocrine tumors are diagnosed after surgical resection, indicating the importance of thorough preoperative staging.

Abstract

Background: Mixed neuroendocrine–non-neuroendocrine neoplasms (MiNENs) of the esophago-gastric junction (EGJ) are rare aggressive malignant neoplasms, with, currently, limited evidence regarding the appropriate therapeutic approach. Methods: Herein, we report multimodal treatment management of a patient with oligometastatic MiNEN of EGJ (Siewert III), discuss the lessons learnt, and provide a review of the literature. Results: A 69-year-old female was diagnosed with a locally advanced EGJ tumor and three liver metastases (cT4, cN+, M1). Although the initial histology from biopsy revealed adenocarcinoma, the histopathology of a lymph node biopsy from staging laparoscopy revealed infiltration of neuroendocrine carcinoma cells. Thus, the diagnosis of a mixed neuroendocrine adenocarcinoma was set, and systemic chemotherapy with etoposide and cisplatin was initiated. A major clinical response led to conversion surgical resection of the primary tumor and metastases, followed by adjuvant therapy with immunotherapy. The patient is free of disease at the 3-year follow-up. A review of the literature on similar cases of EGJ or gastric MiNENs revealed a limited number of cases. Out of the 39 patients, 20 of them (51.3%) suffered from advanced-stage disease. The MiNEN diagnosis typically occurred after surgical resection. Systemic chemotherapy against the neuroendocrine component demonstrated significant response rates, while in cases in which conversion surgery was offered, prolongation of survival was demonstrated. Conclusions: Our case and the existing literature on MiNENs of EGJ underline the need for a personalized treatment approach following thorough interpretation of comprehensive pretherapeutic staging. Conversion radical surgery with curative intent could be considered in cases of major or complete clinical response to induction chemotherapy with potentially favorable outcomes.

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