Neuroendocrine carcinoma of the esophagus: clinical characteristics and prognostic evaluation of 49 cases with surgical resection.
BACKGROUND
The clinicopathological features and optimum treatment of esophageal neuroendocrine carcinoma (NEC) are hardly known due to its rarity. Therefore, we conducted a retrospective study to analyze the clinical characteristics and prognosis of patients with surgically resected esophageal NEC.


METHODS
We collected clinicopathological data on consecutive limited disease stage esophageal NEC patients who underwent esophagectomy with regional lymphadenectomy in West China Hospital from January 2007 to December 2013.


RESULTS
A total of forty-nine patients were analyzed retrospectively. The mean age of the patients was 58.4±8.2 years with male predominance. Fifty-five percent of the esophageal NEC were located in the middle thoracic esophagus. Histologically, 28 (57.1%) patients were found to be small cell NECs. Fifty-one percent of the patients were found to have lymph node metastasis. According to the 2009 American Joint Committee on Cancer (AJCC) staging system for esophageal squamous cell carcinoma, 9 patients were at stage I, 21 patients stage II, and 19 patients stage III. Twenty-six patients (53.1%) received adjuvant therapy. After a median follow-up of 44.8 months [95% confidence interval (CI), 35.2-50.4 months], the median survival time of the patients was 22.4 months (95% CI, 14.0-30.8 months). The 1-year and 3-year survival rates for the whole cohort patients were 74.9% and 35.3%, respectively. In univariate analysis, TNM staging, lymph node metastasis and adjutant therapy significantly influenced survival time. In multivariate analysis, TNM staging was the only independent prognostic factor.


CONCLUSIONS
Esophageal NEC has a poor prognosis. The 2009 AJCC TNM staging system for esophageal squamous cell carcinoma may also fit for esophageal NEC. Surgery combined with adjuvant therapy may be a good option for treating limited disease stage esophageal NEC. Further prospective studies defining the optimum therapeutic regimen for esophageal NEC are needed.
Department of Thoracic Surgery, West China Hospital of Sichuan University, Chengdu 610041, China.
Han-Yu
HY
Deng
Department of Thoracic Surgery, West China Hospital of Sichuan University, Chengdu 610041, China.
Peng-Zhi
PZ
Ni
Department of Thoracic Surgery, West China Hospital of Sichuan University, Chengdu 610041, China.
Yun-Cang
YC
Wang
Department of Thoracic Surgery, West China Hospital of Sichuan University, Chengdu 610041, China.
Wen-Ping
WP
Wang
Department of Thoracic Surgery, West China Hospital of Sichuan University, Chengdu 610041, China.
Long-Qi
LQ
Chen
Neuroendocrine carcinoma (NEC); esophagus; prognosis; surgery