Related Experiment Video
Updated: Jun 20, 2025
Co-culture of Glutamatergic Neurons and Pediatric High-Grade Glioma Cells Into Microfluidic Devices to Assess Electrical Interactions
Published on: November 17, 2021
Gastroenteropancreatic neuroendocrine tumors in children and adolescents
Ülkü Miray Yıldırım1, Dilşad Koca1, Rejin Kebudi1
1Division of Pediatric Hematology Oncology, Institute of Oncology, İstanbul University, İstanbul, Türkiye.
Background:
Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are rare in children and adolescents. Standard management of these tumors has not been well established due to their rarity in this age group. We aimed to report the clinical and pathological characteristics of patients with this rare disease followed and treated between the years 1993-2022.
Materials And Methods:
The medical records of patients with GEP-NETs were reviewed.
Results:
Fourteen patients (11 girls, 3 boys) were diagnosed with GEP-NET. The median age was 13 (9-18) years. Tumor localization was the appendix in 12, stomach in one and pancreas in one patient. Mesoappendix invasion was detected in four patients two of whom underwent right hemicolectomy (RHC) and lymph node dissection (LND). Of those, one patient had lymph node involvement. The other two had not further operations. Somatostatin was used in one with pancreatic metastatic disease and the other with gastric disease after surgery. No additional treatment was given in other patients. All patients are under follow-up without evidence of disease at a median follow-up of 85 months (7-226 months).
Conclusion:
GEP-NETs should be considered in the differential diagnosis of acute appendicitis and in cases with persistent abdominal pain. In children, there is invariably a favorable prognosis, and additional surgical interventions other than simple appendectomies generally do not provide benefits. Mesoappendix invasion may not necessitate RHC and LND.
Insights
Pediatric gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are rare, but typically have a favorable prognosis. Simple appendectomies are often sufficient, even with mesoappendix invasion, avoiding extensive surgery.
Area of Science:
- Pediatric Oncology
- Gastroenterology
- Endocrinology
Background:
- Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are exceptionally rare in pediatric populations.
- Established management protocols for pediatric GEP-NETs are lacking due to their infrequency.
- This study details the clinical and pathological features of pediatric GEP-NET patients treated between 1993-2022.
Purpose of the Study:
- To characterize the clinical and pathological aspects of rare pediatric GEP-NETs.
- To evaluate treatment outcomes and prognostic factors in young patients with GEP-NETs.
Main Methods:
- Retrospective review of medical records for patients diagnosed with GEP-NET.
- Analysis of tumor location, surgical interventions, adjuvant therapies, and follow-up data.
Main Results:
- Fourteen pediatric GEP-NET cases were identified (11 female, 3 male), with a median age of 13 years.
- The appendix was the most common tumor site (12/14 cases).
- Mesoappendix invasion occurred in four patients; two underwent right hemicolectomy (RHC) and lymph node dissection (LND), with one showing lymph node involvement. Other patients received conservative management or somatostatin therapy. All patients remain disease-free.
Conclusions:
- GEP-NETs should be considered in pediatric differential diagnoses for acute appendicitis and persistent abdominal pain.
- Pediatric GEP-NETs exhibit a favorable prognosis, often not requiring extensive surgical intervention beyond appendectomy.
- Mesoappendix invasion may not mandate RHC and LND in pediatric cases.
