Adrenocortical carcinoma in children.

Adrenocortical carcinoma in childhood is a rare, potentially fatal disease. Despite its often-dramatic presentation, there typically has been a distressingly long delay between the onset of symptoms and the time of diagnosis, which undoubtedly has contributed to the poor prognosis in these children by permitting the disease to reach an advanced stage before treatment is started. It is therefore imperative that the physician recognize the endocrine manifestations of these tumors. Although biochemical evaluation is helpful, it often cannot differentiate benign from malignant neoplasms and therefore should not unduly delay intervention. Aggressive surgical resection continues to be the mainstay of treatment as the role of adjuvant therapy continues to evolve. It is hoped that increased familiarity with this unusual tumor will result in earlier detection, prompt intervention, and improved survival for children with adrenocortical carcinoma.