Adrenocortical carcinoma (ACC) is a cancer that arises from the adrenal cortex. It represents 0.2% of childhood malignancies and has an international incidence of 1 in 2,000,000. It is more frequent in girls than in boys (ratio 1.5:1). Adrenocortical carcinoma occurs in childhood with a peak incidence at 3.5 years of age. Most ACCs in children and adolescents are hormone-secreting and the clinical presentation reflects the pattern of adrenocortical hormones secreted by the tumor. Signs and symptoms of virilisation are present in over 90% of cases, hypertension may also be present. Hirsutism, acne and deepening of the voice may be apparent in both sexes. Girls may also present with cliteromegaly and facial hair, while boys present with phallomegaly and early virilisation. Cushing's syndrome (see this term) occurs in a third of cases with the most common signs being a moon-like facies, centripetal fat distribution and plethora. There is an increased incidence of ACC in patients with isolated hemihypertrophy, Wiedemann-Beckwith syndrome, congenital adrenal hyperplasia (CAH) and Li-Fraumeni syndrome (LFS) (see these terms). Diagnosis is based on the results of urine and blood tests, dexamethasone suppression tests, abdominal ultrasound, CT and MRI scans. Differential diagnosis should include phaeochromocytoma and neuroblastoma (see these terms). At least 50 % of ACCs have an inherited basis and hence referral to a cancer genetics service is recommended. Complete, radical surgical resection is the treatment of choice and may be curative, especially for small tumors. In patients with incomplete resection or metastatic spread, treatment options include chemotherapy and/or mitotane, which obtained EU marketing authorisation in 2004 as an Orphan drug for symptomatic treatment of advanced (unresectable, metastatic or relapsed) adrenocortical carcinoma. ACC has a poor prognosis (5-year actuarial survival is 36%) except in case of small tumours that are completely resected.
Last update: June 2007