Esophageal endocrine cell carcinoma: a report of eight cases and a review of the literature

Abstract

Esophageal endocrine cell carcinoma is a rare histopathological type thought to be highly malignant and to have a poor prognosis, but its clinical behavior has not been fully analyzed. We retrospectively reviewed our cases among 386 patients with esophageal cancer in our institution. Eight patients (2.1 %) were pathologically diagnosed with endocrine cell carcinoma. Median patient age was 63 years, and the male-to-female ratio was 7:1. Cancer stage according to the Japanese Classification of Esophageal Cancer (JCEC) 10th edition, was stage II in one case, III in three, IVa in one, and IVb in three. Chromogranin-A staining was positive in six cases, synaptophysin in six, cluster of differentiation (CD)56 in seven, and neuron-specific enolase (NSE) in seven. Treatment included surgery in two cases, chemoradiotherapy in four, best-supportive case in one, and rejection in one. Prognoses were 13 months after surgery for stage II, 6–28 months after chemoradiotherapy for stage III, and 0–10 months for stage IV.