Appendix carcinoids in childhood: long-term experience at a single institution in Western Canada and systematic...

The aim of this study was to describe the management experience of children with appendix carcinoids (AC).

A systematic review of the literature pertaining to AC in the pediatric population was performed. PubMed, EMBASE and SCOPUS and most significant Congress proceedings were reviewed. In addition, charts and files at Stollery Children's Hospital, University of Alberta, Edmonton, Canada, were retrieved and the data compared with the scientific literature review.

Between January 2000 and October 2010, seven patients had AC at the authors' institution. Mean age at diagnosis was 15 years while the male : female ratio was 3:4. Five out of seven patients presented with acute appendicitis. Size ranged up to 1.4 cm. In one case only lymph node metastases were observed. No distant metastases were seen in any patient. Carcinoid was low grade in all seven children. One patient suffered from familial adenomatous polyposis. At a mean follow up of 53.4 months, the outcome was excellent with all patients alive without disease. In the systematic review, 206 studies referring to AC in children were identified. Most series on childhood AC were small single-center series with 2-19 patients.

Older age (>10 years) at diagnosis, female preponderance, and tumor diameter <1.5 cm were the salient observations in the present study. Surgical resection at an early stage allows for excellent prognosis without the need for any additional surgical or adjuvant treatment. Genetic counseling may be appropriate in view of the possible association with colonic adenocarcinoma.