Esophageal Large-cell Neuroendocrine Carcinoma With Pagetoid Spread: A Case Report and Literature Review

BACKGROUND/AIM: Esophageal neuroendocrine carcinoma (NEC) is known for its aggressive behavior and poor prognosis. However, the characteristics of this disease remain unclear owing to its rarity. CASE REPORT: A 59-year-old male with an esophageal submucosal tumor-like lesion was referred to our department. Esophagogastroduodenoscopy revealed a protruding lesion in the lower esophagus and a wide range of patchy staining in the middle to lower esophagus after iodine spraying. Biopsies yielded a diagnosis of neuroendocrine carcinoma with suspicion of peripheral extension. Robot-assisted subtotal esophagectomy was performed, and histopathological examination confirmed a diagnosis of large-cell neuroendocrine carcinoma (LCNEC) accompanied by pagetoid spread of 13 cm in length. No recurrence has been observed during 24 months of follow-up. To the best of our knowledge, this is the first report of esophageal NEC with pagetoid spread. We conducted a literature review of NEC with pagetoid spread in various organs and analyzed 16 cases, including the present case. CONCLUSION: Pagetoid spread is a distinctive characteristic of NECs, especially LCNEC, arising in various organs, including the esophagus. Attention to this characteristic is warranted because it results in undetected cancer spread, which may lead to complications of incomplete therapies or tumor relapses.