Adrenocortical carcinoma in children - Review and recent innovations

Pediatric adrenocortical carcinoma is a rare, potentially fatal disease. The majority of pediatric adrenocortical carcinomas are functional, but despite the dramatic clinical presentation, most patients have a delay in diagnosis. Although biochemical and histological diagnosis has remained largely unchanged, radiographic evaluation has improved and new molecular data have accrued. Unfortunately, despite these advances, treatment of the disease has largely remained unchanged. New surgical techniques and preoperative management have diminished the morbidity and mortality, however. This review will give the reader increased familiarity with this unusual tumor and hopefully, lead to earlier recognition, prompt intervention, and improved survival of these patients.