Adrenocortical Carcinoma PDQ®

In brief

NCI's evidence-based clinical reference for healthcare professionals treating adrenocortical carcinoma in pediatric patients. Covers diagnostic evaluation, staging, treatment approaches, and prognostic factors for this rare endocrine malignancy in children.

• Adrenocortical carcinoma is a rare pediatric malignancy requiring specialized multidisciplinary management.
• Complete surgical resection remains the primary curative treatment for localized adrenocortical carcinoma.
• Hormonal syndromes (virilization, Cushing syndrome) are common presenting features in pediatric cases.
• Genetic predisposition (Li-Fraumeni syndrome, Beckwith-Wiedemann) should be evaluated in all pediatric patients.
• Prognosis depends heavily on stage at diagnosis and completeness of surgical resection.

Written by the GCMD Library team from the guideline.