Carcinoid tumor of the appendix in childhood: the experience of two Italian institutions

OBJECTIVES: Although rare, carcinoid tumor of the appendix is the most common neoplasm of the gastrointestinal tract in children and adolescents. It is usually an incidental finding after a laparotomy for appendectomy, with a frequency of 2 to 5 cases per 1000 appendectomies. The experience with 14 cases of carcinoid reported in the appendix is described. METHODS AND RESULTS: In six patients the tumor measured 1 cm or less; only in one patient did it measure 2 cm. In three patients the tumor measured between 1 and 2 cm and in four the size was not known. In five cases the lesion had invaded the mesoappendix and periappendiceal fat. All tumors were discovered by chance, and three patients underwent further surgery as a result of suspected involvement of the margins. All the patients were alive with no evidence of disease at 24 to 214 months from diagnosis. CONCLUSIONS: Traditionally, local invasiveness and size have been considered prognostic factors. In our experience, both patients with local invasiveness and the patient with a tumor larger than 2 cm had good outcomes. Ileocolectomy performed in the patient with a 2-cm tumor and in another two patients with smaller tumors did not demonstrate residual disease. Although the need for right hemicolectomy still remains controversial for tumors measuring more than 2 cm, the approach may be nonaggressive in case of tumors invading the serosa and the periappendiceal fat. Nonaggressive treatment has been suggested by some authors in cases of tumors larger than 2 cm; however, larger series need to be evaluated.