Gastroenteropancreatic neuroendocrine carcinoma in children and adolescents: a population-based study,Journal of Cancer Research and Clinical Oncology

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Gastroenteropancreatic neuroendocrine carcinoma in children and adolescents: a population-based study
Journal of Cancer Research and Clinical Oncology ( IF 2.7 ) Pub Date : 2024-01-02 , DOI: 10.1007/s00432-023-05568-3
Weilong Lin ₁ , Zhitao Zhu ₂ , Yuping Shang ₃

Affiliation

Purpose

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.

Methods

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.

Results

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.

Conclusions

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.

中文翻译：

儿童和青少年胃肠胰腺神经内分泌癌：一项基于人群的研究

目的

儿童胃肠胰腺神经内分泌癌（GEP-NEC）是一种极其罕见且具有侵袭性的癌症。我们的目的是进行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)。

方法

利用监测、流行病学和最终结果 (SEER) 数据库来识别 2000 年至 2019 年间诊断的所有 GEP-NEC 儿科患者。为了根据各种标准创建生存曲线，使用了 Kaplane-Meier 估计。对数秩检验用于比较生存曲线。使用 Cox 比例风险回归确定与 OS 相关的变量。此外，我们开发了列线图来预测儿科 GEP-NEC 患者的总生存率。

结果

总共确定了 103 名儿童 GEP-NEC 患者。肿瘤主要影响女性（62.2%）。大多数 GEP-NEC 发现于阑尾（63.1%），其次是胰腺（23.3%）和肠道（13.6%）。阑尾起源的 NEC 的局部分期率 (76.9%) 和手术率 (98.5%) 最高。然而，胰腺起源的远处病变比例最大（66.7%），但手术比例最低（37.5%）。所有患者的总体 1 年、3 年和 5 年生存率分别为 94.4%、85.4% 和 85.4%。与阑尾和肠道肿瘤相比，胰腺来源的肿瘤的存活率最差。 Cox 比例风险回归表明，只有位点是生存的重要独立预测因子。