Gastroenteropancreatic neuroendocrine carcinoma in children and adolescents: a population-based study.

Abstract

Gastroenteropancreatic Neuroendocrine Carcinoma (GEP-NEC) in children is an exceptionally rare and aggressive form of cancer. We aimed toconducta population-based cohort study to predict overall survival (OS) in pediatric patients with GEP-NEC. The Surveillance, Epidemiology, and End Results (SEER) database was employedto identify all pediatric patients with GEP-NECdiagnosed between 2000 and 2019.To create survival curves based on variouscriteria, Kaplane-Meier estimations were utilized. The log-rank test was used to compare survival curves.The variables associated with OSwere determined using Cox proportional-hazards regression. Furthermore, we developeda nomogram to predict overall survival in pediatric GEP-NECpatients. 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.