Lin Weilong, Zhu Zhitao, Shang Yuping
Department of Pediatric Surgery & Vascular Anomalies, Xi'an International Medical Center Hospital, Xi'an, 710100, Shaanxi, China.
Department of Medical Imaging, The Second People's Hospital of Lianyungang (The Oncology Hospital of Lianyungang), Lianyungang, 222023, Jiangsu, China.
J Cancer Res Clin Oncol. 2024 Jan 2;150(1):4. doi: 10.1007/s00432-023-05568-3.
Gastroenteropancreatic Neuroendocrine Carcinoma (GEP-NEC) in children is an exceptionally rare and aggressive form of cancer. We aimed to conduct a population-based cohort study to predict overall survival (OS) in pediatric patients with GEP-NEC.
The Surveillance, Epidemiology, and End Results (SEER) database was employed to identify all pediatric patients with GEP-NEC diagnosed between 2000 and 2019. To create survival curves based on various criteria, Kaplane-Meier estimations were utilized. The log-rank test was used to compare survival curves. The variables associated with OS were determined using Cox proportional-hazards regression. Furthermore, we developed a nomogram to predict overall survival in pediatric GEP-NEC patients.
A total of 103 pediatric GEP-NEC patients were identified. The tumors primarily affected females (62.2%). The majority of GEP-NEC was found in the appendix (63.1%), followed by the pancreas (23.3%) and the intestinal tract (13.6%). The highest rates of localized stage (76.9%) and surgery (98.5%) were found in the NEC of appendix origin. However, pancreatic origins had the largest proportion of distant disease (66.7%) but the lowest percentage of surgery (37.5%). Overall 1-year, 3-year, and 5-year survival rates for all patients were 94.4%, 85.4%, and 85.4%, respectively. Tumors of pancreatic origin had the worst survival compared with those of the appendix and intestinal tract. The Cox proportional hazard regression revealed that only site was an important independent predictor of survival.
Our study revealed that only the primary site was found to be the most important predictor of the OS in pediatric GEP-NEC. It's important to work closely with a multidisciplinary team, including oncologists, surgeons, and other specialists, to determine the most appropriate treatment plan for pediatric GEP-NEC.
儿童胃肠胰神经内分泌癌（GEP-NEC）是一种极其罕见且侵袭性强的癌症形式。我们旨在开展一项基于人群的队列研究，以预测GEP-NEC儿科患者的总生存期（OS）。
利用监测、流行病学和最终结果（SEER）数据库来识别2000年至2019年间诊断出的所有GEP-NEC儿科患者。为基于各种标准创建生存曲线，采用了Kaplane-Meier估计法。使用对数秩检验来比较生存曲线。使用Cox比例风险回归确定与总生存期相关的变量。此外，我们开发了一种列线图来预测儿科GEP-NEC患者的总生存期。
共识别出103例儿科GEP-NEC患者。肿瘤主要影响女性（62.2%）。大多数GEP-NEC位于阑尾（63.1%），其次是胰腺（23.3%）和肠道（13.6%）。阑尾起源的神经内分泌癌局部分期（76.9%）和手术率（98.5%）最高。然而，胰腺起源的远处疾病比例最大（66.7%），但手术比例最低（37.5%）。所有患者的1年、3年和5年总生存率分别为94.4%、85.4%和85.4%。与阑尾和肠道起源的肿瘤相比，胰腺起源的肿瘤生存率最差。Cox比例风险回归显示，只有肿瘤部位是生存的重要独立预测因素。
我们的研究表明，在儿科GEP-NEC中，仅原发部位是总生存期的最重要预测因素。与包括肿瘤学家、外科医生和其他专家在内的多学科团队密切合作，为儿科GEP-NEC确定最合适的治疗方案非常重要。
