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.
Abstract

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