The clinicopathologic features and prognosis of esophageal neuroendocrine carcinomas: a single-center study of 53 resection cases

Lei Ye,Hui Shi,Hui Min Wu,Lei Zhang,De Min Li,Fang Yu Wang,Lin Wu,Pin Tu,Heng Lu

doi:10.1186/s12885-019-6420-8

Abstract

BackgroundEsophageal neuroendocrine carcinomas (NECs) are exceedingly rare and poorly understood. The aims of the retrospective study were to delineate the clinicopathologic features and prognosis of patients with the disease.MethodsWe performed a retrospective study containing 53 patients of esophageal NECs in our center from 2002 through 2018. Patients were assigned to the pure esophageal NECs group and the esophageal NECs mixed with squamous carcinoma and/or esophageal adenocarcinoma (MiNECs) group. Demographic, clinical, pathologic and prognostic factors were recorded and analyzed.ResultsOf the 53 patients, elderly male patients were predominant. Dysphagia was the most common symptom (45/53, 84.9%). Most tumors were centered in the middle esophagus (36/53,67.9%).Ulcerated appearance was frequently seen in the pure NECs (56.8%), and the tumors in the MiNECs group mostly represented elevated types (57.9%). Synaptophysin (38/45, 84.4%), chromogranin A (21/38, 55.3%) and CD56(23/27, 85.2%) have been proven to be positive markers for NECs. Most patients (46/53, 86.8%) received surgery combined with chemotherapy. Though the pathologic stages were alike (P = 0.129), the median survival time was 3.53 years for the pure NECs group and 7 years for the MiNECs group. In multivariate analysis, pathologic stage (RR = 1.938, P = 0.045) and age (RR = 2.410, P = 0.028) were independent prognostic factors for patients with MiNECs. The prognosis of patients with pure NECs was independent from any factors.ConclusionsCareful endoscopic examination could help distinguish pure NECs from MiNECs. NECs were aggressive, but a relative better prognosis for patients with MiNECs. Surgery should be performed if applicable, and chemotherapy might be helpful.

Highlights

Esophageal neuroendocrine carcinomas (NECs) are exceedingly rare and poorly understood
Exclusion criteria were: 1) clinical data were not available even though the pathologic diagnosis was an esophageal NEC; 2) the patient had a history of neuroendocrine carcinoma in other organs; 3) the final pathological diagnosis was made on the biopsy sample; 4) the tumors located in the gastroesophageal junction (GEJ) were carefully evaluated, and only carcinomas in which the epicenter was present within 2 cm to the GEJ were included [5]
To observe whether there exhibited some differences between patients with pure NECs or patients with Mixed neuroendocrine-nonneuroendocrine carcinomas (MiNECs), we summarized them separately

Summary

Introduction

Esophageal neuroendocrine carcinomas (NECs) are exceedingly rare and poorly understood. NECs in the esophagus are extremely rare, and data on the clinical features, pathological descriptions and prognosis is limited. Because of the paucity of large surveys regarding experience with esophageal NECs, the biological features and optimum therapy far from being established up. We described the clinical and pathological features, immunohistochemical findings, endoscopic characteristics and prognosis of primary esophageal NECs. Coexistence of squamous cell carcinoma and/or adenocarcinoma is often observed [4]. To better understand the behavior of the NECs, we categorized patients into groups with and without synchronous esophageal tumors. To our knowledge, this is the largest clinic-pathological study investigating the entity to date

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