Primary XXXX of the esophagus.

@entity1 @entity3975 @entity5 @entity75 @entity189 @entity1721 @entity418 @entity3 @entity395
INTRODUCTION: Primary @entity3975 ( @entity3975 ) of the esophagus is an uncommon @entity5 characterized by a diffuse mixture of squamous and mucus-secreting @entity189 cells. Its biological behavior and response to therapies have not been well studied. Surgical resection is still the primary treatment, but the prognosis is poor. This retrospective study was designed to investigate the clinical characteristics, diagnosis, treatment, and prognosis of primary esophageal @entity3975 . METHODS: Clinical data were retrospectively analyzed from 36 @entity1 with pathologically confirmed primary esophageal @entity3975 who underwent transthoracic esophagectomy with lymphadenectomy between January 1991 and June 2010 at the @entity5 Hospital of Shantou University Medical College. There were 27 @entity1 and 9 @entity1 ranging in age from 40 to 78 years (median, 58 years). Twenty-six of the 36 @entity1 were treated with surgery alone. The other 10 were treated with surgery plus postoperative radiotherapy. The Kaplan-Meier and log-rank methods were used to estimate and compare survival rates. Cox's hazard regression model was used to identify prognostic factors, with entry factors of gender, age ( <=60 years versus >60 years), length of the @entity75 ( <=5 cm versus >5 cm), location of the @entity75 , macroscopic @entity5 type, @entity5 cell differentiation, pT, pN, pTNM stage, operation type (radical/palliative), and radiotherapy (yes/no). RESULTS: The clinical symptoms, radiological and endoscopic features of primary esophageal @entity3975 were similar to those of @entity1721 ( @entity1721 ). Of the 20 cases who underwent preoperative endoscopic biopsy, 18 cases were misdiagnosed as @entity1721 and 2 were misdiagnosed as @entity418 . The mean follow-up duration of this series was 38.8 months (range, 3-142 months). Twenty-two @entity1 had died, 12 were still alive, and 2 were lost to follow-up. The median survival time of the 36 @entity1 was 29.0 months (95% confidence interval = 20.0-38.0), and the 1-, 2-, 3-, and 5-year overall survival rates were 80.6%, 57.1%, 34.4%, and 25.8%, respectively. The 5-year survival rate of 25.8% was lower than 5-year absolute survival rate of 39.2% for @entity1721 @entity1 who had undergone surgical resection during the same period at our center. For @entity1 who underwent a radical operation, the 5-year survival rate of 32.0% for @entity3975 @entity1 was also lower than that of 41.7% (908/2175) for @entity1721 @entity1 . In univariate analysis, pN (pN0/pN1-3) (p = 0.003) and operation type (radical/palliative) (p = 0.006) significantly influenced the median survival time of @entity3975 @entity1 . In multivariate analysis, pN (pN0/pN1-3) (p = 0.002) and operation type (radical/palliative) (p = 0.004) were independent prognostic factors. CONCLUSIONS: Primary esophageal @entity3975 is a @entity395 and prone to be misdiagnosed. Lymph node @entity3 and operation are independent prognostic factors. Surgical resection is the primary treatment, but the prognosis is poor.
