Carcinoid tumors in children and adolescents: risk for second malignancies

Carcinoid tumors (CTs) are rare in the pediatric population and are generally noted as an incidental finding on histopathologic examination. Cure is usually achieved with wide surgical excision. Second primary malignancies (SPM) of the gastrointestinal tract after CTs have been reported in 13% to 33% of affected adults. The risk of SPM appears highest after small bowel or appendiceal CTs and usually presents within 7 years from diagnosis.

The purpose of this study was to investigate the natural history of CTs in pediatric patients treated at a major children's hospital and to determine whether children and adolescents with primary CTs developed SPM during routine long-term follow-up.

We conducted a retrospective review of the medical records of children and adolescents with CTs diagnosed at Nationwide Children's Hospital, Columbus, Ohio between 1945 and 2012.

Thirty-two patients with CT were identified, representing 0.48% of all malignancies diagnosed at Nationwide Children's Hospital. Mean age at presentation was 13 years (range, 8 to 20 y). The majority were appendiceal (87.5%) followed by bronchial (9.4%). Most of the appendiceal tumors presented with clinical appendicitis (25/28). Three had incidental appendectomies at the time of a planned abdominal surgery for other reasons. Four patients with appendiceal CTs had invasive features. All patients with appendiceal and bronchial CTs were successfully treated by complete surgical excision and were free of disease at an average of 7 years from diagnosis. None of our patients developed SPM during the period of observation (median 84 mo; range, 12 to 156 mo).

In this single-institution retrospective review, survival of children with CT was excellent. No SPMs were observed over the period of follow-up differing from previously reported adult CT series.