BACKGROUND AND OBJECTIVE: Carcinoid tumors of the appendix are rare in childhood and usually have a benign clinical course. Their incidence in appendectomy specimens ranges from 0.1% to 0.9%. The aim of the study was to report the frequency, clinical presentation, tumor characteristics, and outcome of children with appendiceal carcinoid treated in a pediatric oncology department.PATIENTS AND METHODS: All of the cases referred during a 19-year period (1990-2008) were studied retrospectively. Demographics, clinical presentation, tumor characteristics, and follow-up results were recorded.RESULTS: Among 839 admissions, 19 patients (9 boys) with appendiceal carcinoid were identified during the study period. Their median age was 10.5 years (range 4.5-13.2 years). In all of the cases, diagnosis was established after appendectomy. The mean tumor diameter was 4.55 (± 3.45) mm (range 1-15 mm). Concomitant appendicitis was diagnosed in 12 patients. In 18 children tumor size was ≤ 10 mm and did not infiltrate surrounding tissues. In 1 patient the size was 15 mm and a microscopic rupture of the appendix with infiltration of the surrounding fat was present. All of the tumors were located at the tip of the appendix and were of the classic histological type. Staging and follow-up consisted of abdominal ultrasound, chest and abdominal computed tomography scans, Tc bone scan, urine 5-hydroxylindoloacetic acid levels, and 111In octreotide scan. No patient had metastases requiring further therapeutic interventions. No relapses or other neoplasms occurred during a median follow-up period of 45 months (range 6-118 months).CONCLUSIONS: Carcinoid tumors of the appendix in children are rare. Long-term follow-up revealed that a good prognosis is possible provided they are diagnosed and surgically removed at an early stage.