Appendiceal carcinoids in children-management and outcomes

Background: Appendiceal carcinoid tumors are very rare in children, and management has been guided by adult presentations and outcomes. Here, we present our experience with pediatric appendiceal carcinoid tumors. Methods: We undertook a retrospective review of all cases of appendiceal carcinoids in children over a 20-y period. Data regarding clinical presentation, diagnosis, pathology, follow-up, and outcomes were collected and analyzed. Results: We identified 13 cases of appendiceal carcinoids. All cases were diagnosed after appendectomy for presumed appendicitis (nine acute and four interval;), with no patient having carcinoid syndrome. Mean age at diagnosis was 13.7 y, and all but one case was female. Tumor size ranged from microscopic foci of tumor cells to 2.1 cm (mean, 0.93 cm). Seven cases had invasion of the mesoappendix, three of which underwent a subsequent right hemicolectomy. The patient with the largest tumor (2.1 cm) had evidence of lymphatic invasion with three nodes positive for tumor after right colectomy. No patient had elevation of 5-hydroxyindoleacetic acid or serum chromogranin A, and surveillance computed tomographic scans did not reveal any liver metastases. Conclusions: This is a relatively large series of carcinoids of the appendix in children. We found no evidence of carcinoid syndrome or metastatic disease in these cases other than lymphatic. The need for a secondary colectomy is perhaps questionable.