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Appendiceal Carcinoids in Children-Prevalence, Treatment and Outcome in a Large Nationwide Pediatric Cohort
Johannes Wolfgang Duess1, Ansgar Lange2, Jan Zeidler2
1Department of Pediatric Surgery, University of Leipzig, 04103 Leipzig, Germany.
Insights
Appendiceal carcinoids, rare pediatric neuroendocrine tumors, were found in 0.11% of appendectomies. Most children received only appendectomy, but treatment varied, necessitating further study.
Area of Science:
- Pediatric Oncology
- Gastrointestinal Surgery
- Pathology
Background:
- Appendiceal carcinoids are rare neuroendocrine tumors often discovered incidentally after appendectomy.
- Prevalence, treatment, and outcomes in pediatric cases are not well-established.
Purpose of the Study:
- To determine the prevalence of appendiceal carcinoid tumors in children.
- To analyze treatment modalities and outcomes in pediatric patients.
Main Methods:
- Observational cohort study analyzing 8 years of data from a large German health insurance database.
- Included patient demographics, surgical techniques, departmental data, diagnostic work-up, and postoperative morbidity.
Main Results:
- Appendiceal carcinoids were identified in 44 out of 40,499 pediatric appendectomy cases (0.11% prevalence).
- The mean age was 14.7 years; 91% underwent laparoscopic appendectomy.
- Only 18% required right-sided hemicolectomy, and 11% had additional imaging (CT/MRI).
Conclusions:
- Appendiceal neuroendocrine tumors are rare in children, with a prevalence of 0.11%.
- Appendectomy alone was the primary treatment for most pediatric patients.
- Treatment approaches remain variable, highlighting the need for longer follow-up and guideline evaluation for limited surgical strategies.
Abstract:
Background and Objectives: Appendiceal carcinoids are rare neuroendocrine tumors and mainly found incidentally during histopathological examination following appendectomy. This observational cohort study was performed to determine the prevalence, treatment modalities and outcomes in children diagnosed with an appendiceal carcinoid tumor. Materials and Methods: Data from the largest German statutory health insurance "Techniker Krankenkasse" were analyzed within an 8-year period: January 2010 to December 2012 and January 2016 to December 2020. Patient characteristics, surgical technique, type of surgical department, diagnostic management, and postoperative morbidity were analyzed. Results: Out of 40.499 patients following appendectomy, appendiceal carcinoids were found in 44 children, resulting in a prevalence of 0.11%. Mean age at appendectomy was 14.7 (±2.6) years. Laparoscopic approach was performed in 40 (91%) cases. Right-sided hemicolectomy was performed in 8 (18%) patients. Additional diagnostic work-up (CT and MRI) was recorded in 5 (11%) children. Conclusions: This large nationwide pediatric study shows that 1 in 1000 patients was found to have a neuroendocrine tumor of the appendix (prevalence 0.11%), emphasizing its low prevalence in the pediatric age group. The majority of patients were treated with appendectomy only. However, treatment modalities are still variable. Longer follow-up analyses are needed to evaluate published guidelines and recommendations to aim for a limited surgical approach.
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