Multimodal Treatment With Nivolumab Contributes to Long-Term Survival in a Case of Unresectable Esophagogastric Junction Neuroendocrine Carcinoma

Advanced neuroendocrine carcinoma (NEC) has an extremely poor prognosis, partly explained by the rarity and diagnostic difficulty, for which the most appropriate treatment strategy has not been established. In this report, we discuss a case of unresectable advanced esophagogastric junction NEC, which was difficult to diagnose, that has achieved relatively long-term survival with multidisciplinary treatment centered on nivolumab. A man in his 60s was initially diagnosed with an advanced esophagogastric junction squamous cell carcinoma (SCC). The lymph node metastasis was detected in the regional lymph nodes and para-aortic region. We diagnosed the patient with T3, N3, M1 (Lym), stage IVB, and administered systemic chemotherapy. Due to the failure of first-line, fluorouracil, and cisplatin therapy, we administered nivolumab as the second-line therapy. This therapy demonstrated partial response, so we performed conversion surgery, however the postoperative diagnosis was NEC. Three years after treatment initiation, a single lymph node metastasis has recurred, which is under control with nivolumab and radiation therapy. However, 4.5 years after the start of treatment, with the advent of immune-related adverse events (irAE), nivolumab was discontinued and the patient was placed on surveillance. Six months after that, metastasis to the hilar lymph node and adrenal gland was observed. Both times that recurrence/metastasis appeared, they occurred while nivolumab was being discontinued, suggesting its significant systemic anti-cancer effect. Therefore, nivolumab in particular may be an effective treatment for advanced esophageal NEC, and this case suggests that it may contribute to prolonged progression-free survival.