Adrenocortical carcinoma in children. Review and recent innovations.

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. This delay undoubtedly has contributed to the historically poor prognosis in these children by permitting the disease to reach an advanced stage before treatment is started. It is imperative that the physician recognizes the endocrine manifestations of these tumors early and has a high index of suspicion. Although biochemical and histologic evaluations are helpful, they often cannot differentiate benign lesions from malignant neoplasms and should not unduly delay intervention. Aggressive complete surgical resection continues to be the mainstay of treatment and is the best prognosticator of overall survival. The role of adjuvant therapy and chemotherapy continues to evolve. Molecular studies have increased understanding of cancer biology and may provide possible novel therapeutic approaches in the future. 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.