Hong Qian, Wu Kaiming, Chen Chen, Dang Yan, Zhang Qiuju, Zhang Xue, Wang Liting, Han Rui, Zhao Chenguang, Yi Hang, Li Fang, Zhang Renquan, Mu Juwei, Li Jiagen
Department of Thoracic Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Department of Thoracic Surgery, The First Affiliated Hospital of Anhui Medical University, Hefei, Anhui, China.
Ther Adv Med Oncol. 2024 Dec 7;16:17588359241303066. doi: 10.1177/17588359241303066. eCollection 2024.
The esophageal mixed neuroendocrine-non-neuroendocrine neoplasm (MiNEN) is an extremely rare but poor prognosis tumor.
This retrospective study aimed to analyze the clinical characteristics of MiNEN and to investigate postoperative survival and prognostic factors.
This retrospective study analyzed 69 patients diagnosed with esophageal MiNEN at two major esophageal cancer centers in China from January 2000 to December 2021.
We assessed demographic data, tumor characteristics, treatment modalities, and survival outcomes. Statistical analyses included Kaplan-Meier survival curves and Cox regression models to evaluate prognostic factors.
The most common histological types were combinations of small-cell carcinoma and squamous carcinoma (91.3%). The correct diagnostic rate of preoperative pathologic biopsy was only 4.3%. The median overall survival (OS) was 24.0 months, and disease-free survival (DFS) was 16.6 months. The 1-, 3-, and 5-year survival rates were 84.1%, 34.8%, and 25.3%, respectively. A peak period of recurrence or metastasis occurs in the first year after surgery, and regional lymph node recurrence is the main route of postoperative recurrence or metastasis. Tumor size, T-stage, N-stage, and tumor, lymph node, metastasis (TNM) stage were significant prognostic factors. Subgroup analyses showed that in patients with limited-stage MiNEN in stages I-III, the postoperative adjuvant treatment modality failed to improve OS and DFS compared with surgery alone. Postoperative adjuvant therapy also failed to prolong OS and DFS in patients with lymph node-positive MiNEN. No significant survival benefits were observed with different surgical techniques or adjuvant chemotherapy regimens.
Esophageal MiNEN has aggressive behavior and a poor prognosis. In China, the pathologic type of esophageal MiNEN may be dominated by a combination of small-cell carcinoma and squamous carcinoma. Early-stage disease significantly correlated with improved survival outcomes. Current treatment protocols, similar to those for other esophageal cancers, show limited efficacy in improving patient survival.
食管混合性神经内分泌-非神经内分泌肿瘤（MiNEN）是一种极其罕见但预后较差的肿瘤。
本回顾性研究旨在分析MiNEN的临床特征，并探讨术后生存情况及预后因素。
本回顾性研究分析了2000年1月至2021年12月在中国两家主要食管癌中心诊断为食管MiNEN的69例患者。
我们评估了人口统计学数据、肿瘤特征、治疗方式和生存结果。统计分析包括Kaplan-Meier生存曲线和Cox回归模型，以评估预后因素。
最常见的组织学类型是小细胞癌和鳞状细胞癌的组合（91.3%）。术前病理活检的正确诊断率仅为4.3%。中位总生存期（OS）为24.0个月，无病生存期（DFS）为16.6个月。1年、3年和5年生存率分别为84.1%、34.8%和25.3%。复发或转移的高峰期出现在术后第一年，区域淋巴结复发是术后复发或转移的主要途径。肿瘤大小、T分期、N分期和肿瘤、淋巴结、转移（TNM）分期是显著的预后因素。亚组分析显示，在I-III期局限性MiNEN患者中，与单纯手术相比，术后辅助治疗方式未能改善OS和DFS。淋巴结阳性MiNEN患者术后辅助治疗也未能延长OS和DFS。不同的手术技术或辅助化疗方案未观察到显著的生存获益。
食管MiNEN具有侵袭性，预后较差。在中国，食管MiNEN的病理类型可能以小细胞癌和鳞状细胞癌的组合为主。早期疾病与改善生存结果显著相关。目前的治疗方案与其他食管癌的治疗方案相似，在改善患者生存方面疗效有限。
