title: Neuroendocrine carcinoma of the esophagus: Clinicopathological and immunohistochemical features of 14 cases.
abstract: BACKGROUND
  Neuroendocrine 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.
  
  
  METHODS
  Fourteen 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.
  
  
  RESULTS
  Eleven 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.
  
  
  CONCLUSIONS
  Consistent 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.
authors.affiliation: Department of Gastroenterological Surgery, National Kyushu Cancer Center, Fukuoka, Japan.
authors.forename: Akinori
authors.initials: A
authors.lastname: Egashira
authors.affiliation: Department of Gastroenterological Surgery, National Kyushu Cancer Center, Fukuoka, Japan.
authors.forename: Masaru
authors.initials: M
authors.lastname: Morita
authors.affiliation: Department of Cancer Pathology, National Kyushu Cancer Center, Fukuoka, Japan.
authors.forename: Reiko
authors.initials: R
authors.lastname: Kumagai
authors.affiliation: Department of Cancer Pathology, National Kyushu Cancer Center, Fukuoka, Japan.
authors.forename: Ken-Ichi
authors.initials: KI
authors.lastname: Taguchi
authors.affiliation: Department of Radiology, National Kyushu Cancer Center, Fukuoka, Japan.
authors.forename: Masanobu
authors.initials: M
authors.lastname: Ueda
authors.affiliation: Department of Gastroenterological Surgery, National Kyushu Cancer Center, Fukuoka, Japan.
authors.forename: Shohei
authors.initials: S
authors.lastname: Yamaguchi
authors.affiliation: Department of Gastroenterological Surgery, National Kyushu Cancer Center, Fukuoka, Japan.
authors.forename: Manabu
authors.initials: M
authors.lastname: Yamamoto
authors.affiliation: Department of Gastroenterological Surgery, National Kyushu Cancer Center, Fukuoka, Japan.
authors.forename: Kazuhito
authors.initials: K
authors.lastname: Minami
authors.affiliation: Department of Gastroenterological Surgery, National Kyushu Cancer Center, Fukuoka, Japan.
authors.forename: Yasuharu
authors.initials: Y
authors.lastname: Ikeda
authors.affiliation: Department of Gastroenterological Surgery, National Kyushu Cancer Center, Fukuoka, Japan.
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Toh
mesh_terms: D000368:Aged; D018278:Carcinoma, Neuroendocrine / Q000473:pathology*; D004938:Esophageal Neoplasms / Q000473:pathology*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged