title: Adrenal cortical neoplasms in children: why so many carcinomas and yet so many survivors?
abstract: Adrenal cortical neoplasms in children are represented by a disproportionate number of cases that have been diagnosed pathologically as adrenocortical carcinomas (ACCs)-as many as 90% of all cortical tumors in some pediatric series. Like other solid malignancies of childhood, over half of ACCs present in the first 4 years of life in over 50% of cases. Most are sporadically occurring neoplasms, but ACCs are a manifestation of Beckwith-Wiedemann and Li-Fraumeni syndromes. Despite the fact that the microscopic features are often quite atypical and identical in many respects to ACCs in adults, the clinical outcome is favorable in 70% or more of cases. Tumor weight is seemingly a significant determinant in prognosis at a threshold of greater than 400 g. A risk assessment system is proposed that incorporates tumor weight, localization of tumor to the gland without invasion into the surrounding tissues or organs, and absence of metastasis.
authors.affiliation: Lauren V. Ackerman Laboratory of Surgical Pathology, Barnes-Jewish and St. Louis Children's Hospitals, Washington University Medical Center, St. Louis, MO 63110, USA. dehner@path.wustl.edu
authors.forename: Louis P
authors.initials: LP
authors.lastname: Dehner
authors.forename: D Ashley
authors.initials: DA
authors.lastname: Hill