Anlotinib plus camrelizumab achieved long‐term survival in a patient with metastatic esophageal neuroendocrine carcinoma

BACKGROUND: Esophageal neuroendocrine carcinoma (NEC) is a rare cancer with an extremely poor prognosis. The average overall survival of patients with metastatic disease is only 1 year. The efficacy of anti‐angiogenic agents combined with immune checkpoint inhibitors remains unknown. CASE PRESENTATION: A 64‐year‐old man, initially diagnosed with esophageal NEC, underwent neoadjuvant chemotherapy and esophagectomy. Although the patient remained disease‐free for 11 months, eventually the tumor progressed and did not respond to three lines of combined therapy (etoposide plus carboplatin with local radiotherapy, albumin‐bound paclitaxel plus durvalumab, and irinotecan plus nedaplatin). The patient then received anlotinib plus camrelizumab, and a dramatic regression was observed (confirmed by positron emission tomography‐computed tomography). The patient has been disease‐free for over 29 months and has survived for over 4 years since diagnosis. CONCLUSION: Combined therapy with anti‐angiogenic agents and immune checkpoint inhibitors may be a promising strategy for esophageal NEC, although more evidence is warranted to validate its efficacy.