title: Primary small cell carcinoma of the esophagus.
abstract: BACKGROUND
  Primary small cell esophageal carcinoma (SCEC) is a rare and aggressive disease for which there is no recommended standard treatment at this time.
  
  
  METHODS
  A total of 126 patients with SCEC, diagnosed histologically between May 1985 and June 2005 at our institution, were analyzed retrospectively. All were staged according to the Veterans' Administration Lung Study Group staging system. The TNM system for esophageal carcinoma (6th edition, American Joint Committee on Cancer) was also used for those who underwent esophagectomies. SPSS (10.0) software was used for statistical analysis. Cox's hazard regression model was performed to identify prognostic factors. The Kaplan-Meier and log-rank methods were used to estimate and compare survival rates. The chi2 test was performed to examine frequencies between different groups.
  
  
  RESULTS
  Through a median follow-up of 13 months, 108 patients died, 10 were alive, and 8 were lost to follow-up. Of the entire study population, the overall median survival time (MST) and 1-, 3-, and 5-year overall survival rates were 12.5 months and 52.2%, 15.9%, and 12.2%, respectively. For limited disease, the MST and 1-, 2-, and 3-year overall survival rates were 14.0 months and 62.1%, 30.8%, and 22.4%, respectively; for extensive disease, the respective values were 7.0 months and 29.3%, 13.6%, and 2.7% (p = 0.0001). The MST of 14.5 months for cases who received chemotherapy was superior to that of 5.2 months for cases who did not (p = 0.0001). Tumor stage, length of the primary lesion, and chemotherapy, but not surgery were independent prognostic factors in a multivariate analysis.
  
  
  CONCLUSIONS
  SCEC is systemic disease. Tumor stage and chemotherapy were independent prognostic factors. Systemic therapy, based on chemotherapy with radiotherapy, is recommended.
authors.affiliation: Department of Radiation Oncology, Cancer Hospital (Institute), Peking Union Medical College, Chinese Academy of Medical Sciences, China.
authors.forename: Jima
authors.initials: J
authors.lastname: Lv
authors.forename: Jun
authors.initials: J
authors.lastname: Liang
authors.forename: Jinwan
authors.initials: J
authors.lastname: Wang
authors.forename: Luhua
authors.initials: L
authors.lastname: Wang
authors.forename: Jie
authors.initials: J
authors.lastname: He
authors.forename: Zefen
authors.initials: Z
authors.lastname: Xiao
authors.forename: Weibo
authors.initials: W
authors.lastname: Yin
mesh_terms: D000368:Aged; D000970:Antineoplastic Agents / Q000627:therapeutic use; D018288:Carcinoma, Small Cell / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D003131:Combined Modality Therapy; D004938:Esophageal Neoplasms / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D016629:Esophagectomy; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D011379:Prognosis; D011879:Radiotherapy Dosage; D012189:Retrospective Studies; D015996:Survival Rate
chemical_list: D000970:Antineoplastic Agents