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Published on: August 24, 2019
Management of appendiceal carcinoid tumors in children
Sara C Fallon1, M John Hicks2, Jennifer L Carpenter1
1Division of Pediatric Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas.
Insights
Appendiceal carcinoid tumors are rare in children, found incidentally in 0.2% of appendectomies. Most are small with low risk of spread, but further study is needed for optimal management guidelines.
Area of Science:
- Pediatric Oncology
- Surgical Pathology
- Neuroendocrine Tumors
Background:
- Appendiceal carcinoid tumors, or well-differentiated neuroendocrine neoplasms, are rare pediatric lesions.
- Often incidentally discovered post-appendectomy, with limited data on natural history and management guidelines.
- Lack of established protocols for family counseling, further surgery, or follow-up imaging in pediatric cases.
Purpose of the Study:
- To review institutional experience with appendiceal carcinoid tumors in children.
- To provide data to inform and improve the management of these rare pediatric neoplasms.
- To address the need for better understanding of natural history and treatment strategies.
Main Methods:
- Retrospective chart review of pediatric patients diagnosed with appendiceal carcinoid tumors (2002-2014).
- Data collection included demographics, pathology, postoperative management, and follow-up.
- Descriptive statistical analyses were performed on collected data.
Main Results:
- Twenty-eight pediatric cases identified, representing 0.2% of appendectomies.
- Mean age 13.8 years; 54% female. Mean tumor size 0.73 cm.
- 18% (5/28) required further surgery (ileocecectomy/hemicolectomy) due to mesoappendix invasion, lymphovascular invasion, or subserosal extension; no recurrences at 1.8-year mean follow-up.
Conclusions:
- Appendiceal carcinoid tumors are incidentally found in ~0.2% of pediatric appendectomies.
- Tumors are typically small, with <20% showing lymphovascular invasion or mesenteric extension.
- Prospective, multicenter studies are essential to define ileocecectomy indications and follow-up imaging protocols.
Background:
Appendiceal carcinoid tumors, also know as well-differentiated neuroendocrine neoplasms, are rare lesions detected incidentally after appendectomy in children. There are limited data about the natural history of these tumors, and guidelines regarding family counseling and need for additional surgery or follow-up imaging are not established in the pediatric age group. The purpose of this study was to review our institutional experience with appendiceal carcinoid tumors to provide data that might improve management.
Methods:
After institutional review board approval, the charts of all patients treated at our institution for an appendiceal carcinoid tumor between 2002 and 2014 were reviewed. Data collected included patient demographics, pathologic details, postoperative management, and follow-up information. Descriptive analyses were performed.
Results:
Twenty-eight patients were identified, which represents an incidence of 0.2% of children undergoing appendectomy during that time interval. The mean age at surgery was 13.8 ± 2.1 y; 54% were females. Two patients had symptoms suspicious for carcinoid syndrome at presentation, though none had evidence of metastatic disease. The mean tumor size was 0.73 ± 0.4 cm. Five patients (18%) underwent subsequent ileocecectomy or right hemicolectomy because of pathologic findings of invasion of the mesoappendix (n = 4) or lymphovascular invasion and subserosal extension (n = 1), two of whom had residual disease in the resected specimen (one in a lymph node). No recurrences have been detected at mean follow-up of 1.8 y.
Conclusions:
Appendiceal carcinoid tumors are discovered incidentally in about 0.2% of children undergoing appendectomy. Based on findings from a large contemporary series, we can conclude that these tumors are generally small and demonstrate lymphovascular invasion or mesenteric extension in fewer than 20% of cases. Prospective, multicenter studies are necessary to better define the indication for ileocecectomy and follow-up imaging protocols.
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