Neuroendocrine carcinoma of the esophagus: Clinicopathological and immunohistochemical features of 14 cases

Abstract

BackgroundNeuroendocrine carcinoma (NEC) of the esophagus is a rare and highly aggressive disease but the biological features are poorly understood. The objective of this study was to analyze the clinicopathological and immunohistochemical features of NEC of the esophagus.MethodsFourteen patients diagnosed with NEC of the esophagus from 1998 to 2013 were included in this study. Clinicopathologic features, therapeutic outcomes and immunohistochemical results were analyzed.ResultsEleven out of 14 cases showed protruding or localized type with or without ulceration. Only three patients were negative for both lymph node and organ metastasis and seven cases were positive for metastases to distant organs and/or distant lymph nodes. Of the six patients with limited disease (LD), three patients were treated by surgery. Three patients with LD and seven patients with extensive disease (ED) were initially treated with chemotherapy, except for one who underwent concurrent chemo-radiotherapy due to passage disturbance. The median survival of patients with LD was 8.5 months, whereas that of patients with LD was 17 months. Among the 14 cases, 12 cases (83.3%), 13 cases (91.7%) and 12 cases (83.3%) showed positive immunostaining for choromogranin A, synaptophysin and CD56, respectively. Nine of 14 cases (64.2%) presented positive staining for c-kit and most (8/9, 88%) simultaneously showed p53 protein abnormality. Two cases were negative for c-kit and p53, and positive for CK20.ConclusionConsistent with previous reports, the prognosis of NEC of esophagus is dismal. From the results of immunohistochemical study, NEC of esophagus might be divided into two categories due to the staining positivity of c-kit and p53. This study provides new insight into the biology of NEC of the esophagus.

A case of mixed adenoneuroendocrine carcinoma (MANEC) arising in Barrett’s esophagus: literature and review

“…Regarding the origin of esophageal NETs, Merkel cells and stem cells are reported candidates [ 22 , 23 ]. Egashira et al showed positive staining for CK20, a marker of Merkel cells, in 14.3% of esophageal MANECs and suggested c-kit and p53 to be potential markers of the origin of NEC cells [ 22 ]. On the other hand, it was suggested that gastric NECs arise from adenocarcinomas rather than from non-neoplastic neuroendocrine cells [ 24 ].…”

Esophageal neuroendocrine carcinoma presenting as a rare cause of dysphagia

“…Esophageal NECs are a very rare cause of dysphagia and represent only 0.4%-2.0% of all esophageal malignancies. [2] Additional uncommon locations are the jejunum, ileum, bladder, cervix, and prostate. [2,4] At the time of diagnosis, the prognosis is considered poor, with median survivals of 34, 14, and 5 months for those with localized, regional, and distant disease respectively.…”

“…[2] Additional uncommon locations are the jejunum, ileum, bladder, cervix, and prostate. [2,4] At the time of diagnosis, the prognosis is considered poor, with median survivals of 34, 14, and 5 months for those with localized, regional, and distant disease respectively. [5] Treatment is subsequently guided based on the classification of limited disease (LD) versus extended disease (ED).…”

Histopathology of Neuroendocrine Neoplasms of the Gastrointestinal System

“…NENs pažeráka sú pomerne vzácne a zriedkavé nádory, s incidenciou 0,05-7,6 % zo všetkých malignít pažeráka [48]. Tieto ná-dory rastú prevažne v dolnej tretine pažeráka, často sú asociované s Baretovým ezofágom [49].…”