Adrenal tumors are extremely rare in childhood, except in Brazil, so that their clinical manifestations remain poorly known by pediatricians. In children, the most common manifestation of adrenal tumors is the appearance of pubic hair and other signs of virilization. Isolated androgen secretion is present in 40% of children's tumors vs 9% in adults. These tumors also have no reliable set of criteria for malignancy. Therefore, all suspected adrenal tumors must be managed as adrenocortical carcinomas (ACCs) until the final diagnosis is made. All children should be treated in endocrine oncologic centers with experienced endocrine surgeons in a highly interdisciplinary setting.
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