Adrenocortical tumors and hyperplasias in childhood--etiology, genetics, clinical presentation and therapy.

ABSTRACT: Adrenocortical tumors are rare in children and are associated with a poor prognosis when malignant. The fund of knowledge regarding etiology, presentation and clinical outcomes remains limited. Evaluation of genetic disorders associated with the development of adrenocortical disorders has allowed researchers to identify a number of mutations that may be involved in tumorigenesis, including alterations in the GNAS1, PRKAR1A, TP53 and IGF2 genes. Clinical presentation in children is associated most commonly with young age, female gender and symptoms of virilization. Most children have localized disease at presentation which may be associated with a better prognosis when compared to adults. Surgical resection remains the only potentially curative treatment and mitotane, the most frequently us

SUBMITTER: Sutter JA 

PROVIDER: S-EPMC1907361 | biostudies-literature | 2006 Sep

REPOSITORIES: biostudies-literature