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Gastroenteropancreatic neuroendocrine carcinoma in children and adolescents: a population-based study
Weilong Lin1, Zhitao Zhu2, Yuping Shang3
1Department of Pediatric Surgery & Vascular Anomalies, Xi'an International Medical Center Hospital, Xi'an, 710100, Shaanxi, China.
Insights
This study found that the primary tumor site is the most crucial factor predicting overall survival in pediatric Gastroenteropancreatic Neuroendocrine Carcinoma (GEP-NEC). Understanding this helps tailor treatment for this rare childhood cancer.
Area of Science:
- Pediatric Oncology
- Cancer Research
- Epidemiology
Background:
- Gastroenteropancreatic Neuroendocrine Carcinoma (GEP-NEC) is an exceptionally rare and aggressive pediatric cancer.
- Predicting overall survival (OS) in affected children is critical for treatment planning.
Purpose of the Study:
- To conduct a population-based cohort study to predict OS in pediatric GEP-NEC patients.
- To identify key predictors of survival in this rare pediatric malignancy.
Main Methods:
- Utilized the Surveillance, Epidemiology, and End Results (SEER) database (2000-2019) to identify pediatric GEP-NEC cases.
- Employed Kaplan-Meier estimations and log-rank tests for survival curve analysis.
- Applied Cox proportional-hazards regression and developed a nomogram for survival prediction.
Main Results:
- Identified 103 pediatric GEP-NEC patients; females constituted 62.2%.
- Appendix was the most common primary site (63.1%), followed by pancreas (23.3%).
- Pancreatic origin tumors showed poorer survival rates and lower surgical intervention compared to appendiceal or intestinal origins; primary site was the sole independent predictor of OS.
Conclusions:
- The primary tumor site is the most significant predictor of overall survival in pediatric GEP-NEC.
- Multidisciplinary team collaboration is essential for optimal treatment planning in pediatric GEP-NEC.
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.
