Background: Carcinoid tumor (CT) is an unusual neoplasm observed in several locations and associated with the production of vasoactive substances and occasionally with carcinoid syndrome (flushing, diarrhea, wheezing).
Patients and methods: A retrospective review of the medial records of all children with a diagnosis of CT treated in our service between 1966 and 2003 was performed.
Results: Ten patients (5 boys and 5 girls with a mean age of 8 years) were diagnosed with CT. Eight had CT of the appendix, of which 4 showed the typical clinical presentation of acute appendicitis. Seven of these tumors were localized at the tip of the appendix and measured 2 cm or less. In one patient, the tumor was located at the cecum and measured 3.5 cm. In this patient, reoperation with ileocecal resection was performed. Two patients (4 and 6 years old) had bronchial CT associated with chronic respiratory manifestations. An accurate diagnosis was made after a 1-year follow-up. Pneumonectomy and tracheobronchial sleeve resection were carried out. None of the patients showed symptoms of carcinoid syndrome. All the patients underwent biochemical and radiological studies and fiberoptic bronchoscopy during follow-up. All the patients are currently disease-free.
Conclusions: Typical symptoms of acute appendicitis were not observed in half of patients with CT of the appendix. CT associated with carcinoid syndrome is exceptional in pediatric patients. In most CT of the appendix, simple appendicectomy was associated with an excellent prognosis. Diagnosis of bronchial TC tends to be delayed and consequently CT should be considered in the differential diagnosis of children with respiratory symptoms unresponsive to standard medical treatment.