Treatment outcomes and prognostic factors in neuroendocrine carcinoma of the esophagus in Vietnam: A retrospective cohort study.

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Background: Esophageal neuroendocrine carcinoma (ENEC) is a rare and highly aggressive malignancy for which no standard treatment has been established. Data from Southeast Asia are extremely limited. This study aimed to describe the clinicopathological characteristics and evaluate treatment outcomes of patients with ENEC treated at a tertiary cancer center in Vietnam. Methods: We conducted a retrospective–prospective observational study of patients with histologically confirmed primary esophageal neuroendocrine carcinoma treated at Vietnam National Cancer Hospital between January 2020 and May 2025. Clinical characteristics, pathological features, treatment modalities, response rates, progression-free survival (PFS), overall survival (OS), and treatment-related toxicities were analyzed. Survival outcomes were estimated using the Kaplan–Meier method and compared using the log-rank test. Results: A total of 37 patients were included, with a median age of 60.4 years; 94.6% were male. Small cell neuroendocrine carcinoma was the predominant histological subtype (86.5%). At diagnosis, 54.1% of patients had limited-stage disease, while 45.9% had extensive-stage disease, most commonly with liver metastases (82.4%). In limited-stage disease, definitive chemoradiotherapy with etoposide-platinum regimen was the most frequently used treatment and achieved an objective response rate of 75%, including a complete response rate of 50%. Patients who achieved complete response had significantly longer OS compared with non-complete responders (39.3 vs. 10.4 months, p < 0.001). In extensive-stage disease, platinum–etoposide chemotherapy resulted in a disease control rate of 70.6%, with no significant difference in OS between cisplatin- and carboplatin-based regimens (9.7 vs. 10.1 months, p = 0.497). Poor performance status and low body mass index were associated with worse OS. Conclusions: ENEC is an aggressive malignancy associated with poor survival, particularly in extensive-stage disease. Definitive chemoradiotherapy can provide meaningful disease control and durable survival in selected patients with limited-stage disease, especially those achieving complete response. Performance status and nutritional status are important prognostic factors. These real-world data from a resource-limited setting add valuable evidence to the limited literature and may help inform treatment strategies for this rare cancer. Expansion of our data from other center is underway.

Treatment outcomes by stage in patients with esophageal neuroendocrine carcinoma.