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

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.

Prognostic value of pretreatment contrast-enhanced computed tomography in esophageal neuroendocrine carcinoma: A multi-center follow-up study

“…Previous studies reported that TNM stage[ 1 , 3 , 6 , 19 ] and adjuvant therapy[ 8 , 12 , 13 ] of esophageal NEC were closely related to the risk of death in patients with esophageal NEC, which is partly consistent with our findings. Studies also confirmed that earlier clinical stage tends to yield better survival outcomes for esophageal NEC[ 8 , 12 , 13 ].…”

“…Esophageal NEC is a rare malignant tumor of the esophagus, which exists independently or mixed with squamous cell carcinoma and adenocarcinoma[ 1 - 3 ]. The principal clinical manifestations are non-specific dysphagia, including choking sensation, chest pain, emaciation, hematemesis, and black stool.…”

“…The principal clinical manifestations are non-specific dysphagia, including choking sensation, chest pain, emaciation, hematemesis, and black stool. Ectopic hormone secretion may occur, such as antidiuretic hormone, with corresponding clinical symptoms[ 1 - 5 ]. Esophageal NEC is radically different from squamous cell carcinoma and adenocarcinoma in biological behavior, tumor response, and prognosis[ 9 ].…”

“…Neuroendocrine tumors are heterogeneous tumors that secrete a variety of peptide substances and/or hormones and other bioactive substances[ 1 ]. Neuroendocrine cells are located throughout the body; thus, neuroendocrine tumors occur throughout the entire body as well.…”

“…There are currently no specific criteria to evaluate patients with esophageal NEC in the American Joint Committee on Cancer (AJCC) staging system. Owing to its rarity, only a few cases of esophageal NEC have been reported in the literature, with most focusing on the clinicopathological characteristics[ 1 , 3 , 6 , 12 ] and treatment[ 8 , 12 , 13 ]. Recent retrospective clinical esophageal NEC analyses have shown better postoperative survival rates with a combination of radical resection[ 8 , 13 ], radical lymph node dissection[ 1 , 3 , 6 ], and adjuvant therapy[ 13 ].…”