Adrenocortical Carcinoma in Children

Adrenocortical carcinoma in childhood is a rare yet potentially fatal disease. We present 5 cases of adrenocortical carcinoma detected and managed in childhood. Of the 5 patients 4 presented with clinical signs of excess corticosteroid production and 1 presented suddenly with abdominal pain. All patients had palpable abdominal masses. All tumors were removed surgically and adjuvant therapy with mitotane was used in 4 children. Two patients are alive and 3 are dead. Our experience demonstrates that mitotane may be used safely in children, and that early diagnosis, aggressive surgical extirpation, and the use of mitotane and chemotherapy may lead to an increased survival of children with adrenocortical carcinoma.