International consensus on mitotane treatment in pediatric patients with adrenal cortical tumors: indications, therapy, and management of adverse effects

Mitotane treatment for advanced pACC with stages III and IV and with incomplete resection/tumor spillage (clear consensus)

For stage II patients, mitotane is not generally indicated

Timing of initiating mitotane depends on the patient's clinical condition and the setting of the planned therapy

Starting dose 50 mg/kg/d (1500 mg/m²/d), which can be increased up to 4000 mg/m²/d

Blood levels should range between 14 and 20 mg/L

Duration of mitotane treatment depends on the clinical risk profile and tolerability

Mitotane causes adrenal insufficiency in virtually all patients, requiring glucocorticoid replacement shortly after beginning

Frequent clinical and neurological examinations (every 2-4 weeks), with evaluation of laboratory values, are required because adverse effects are wide-ranging and can be life-threatening