• These tumors are rare; 1-2 cases per million persons in the United States
• Less than 0.05% of newly diagnosed cancers per year
• Bimodal occurrence, with tumors developing in children < 5 years of age and in adults in their fifth through seventh decade of life
• Male:female ratio is 2:1, with functional tumors being more common in women
• Left adrenal involved slightly more often than the right (53% vs 47%); bilateral tumors are rare (2%)
• 50-60% of patients have symptoms related to hypersecretion of hormones (most commonly Cushing syndrome and virilization)
• Feminizing and purely aldosterone-secreting carcinomas are rare
• 50% of patients have metastases at the time of diagnosis
• Symptoms of specific hormone excess (cortisol excess, virilization, feminization)
• Palpable abdominal mass
• Abdominal pain
• Fatigue, weight loss, fever, hematuria
• All laboratory abnormalities depend on hormonal status of tumor
• Elevated urinary free cortisol or steroid precursors
• Loss of normal circadian rhythm for serum cortisol
• Low serum adrenocorticotropic hormone (ACTH)
• Abnormal dexamethasone suppression test
• Elevated serum testosterone, estradiol, or aldosterone levels
• Evaluation of adrenal glands with CT or MRI (adrenocortical carcinomas are typically isodense to liver on T1-weighted MRI, and hyperdense relative to liver on T2-weighted MRI images)
• MRI more accurately gauges the extent of any intracaval tumor thrombus
• History and physical exam may reveal evidence of hormonal function, particularly the development of cushingoid, masculinizing, or feminizing features
• Imaging should include detailed anatomic imaging of both adrenal glands (CT or MRI) and potential sites of intra-abdominal metastasis (especially liver)
• Plasma metanephrines to rule out medullary tumor (pheochromocytoma)
• ACTH, serum cortisol urine free cortisol, aldosterone, and sulfate derivative of dehydroepiandrosterone (DHEAS) in all patients
• Testosterone or estrogen in patients with suggestive symptoms or signs
• Surgery is the only treatment that can cure or prolong survival
• Laparoscopic surgery not recommended because of spread of tumor, fragility of tumor, and the possible need to resect adjacent involved organs
• For local recurrent disease, reoperation is the only effective therapy and may prolong life
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