Primary esophagogastric neuroendocrine carcinoma: a retrospective study from the Nottingham Upper Gastrointestinal Cancer Center

Primary neuroendocrine carcinomas (NECs) of the esophagus and stomach are rare with an incidence ranging from 0.01 to 0.08 cases per 100,000 persons per year [1, 2]. The prevalence of esophageal NEC ranges between 0.01 and 1.4% and gastric NEC between 2 and 3% of all gastroenteropancreatic neuroendocrine tumors [1, 3, 4, 5, 6, 7]. The esophagus is the most common site for extrapulmonary small cell carcinomas with a prevalence between 18 and 44% [6, 8, 9, 10, 11]. Pathobiology of esophagogastric NEC is largely unknown [12]. Although recent advances in diagnostic techniques have increased awareness of this rare disease, there is a lack of clinical evidence to inform appropriate management of these rare tumors [13]. In the current study, we have explored the clinicopathological characteristics, treatment modalities, and survival of 24 patients with primary esophagogastric NEC who were treated in the Nottingham University Hospitals during a 9-year period (2007–2016)