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  • • Variety of clinical symptoms through excess production of adrenal hormones

    • Complete surgical removal of the primary lesion and any resectable metastatic sites has been the mainstay of treatment

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Epidemiology

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  • • 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

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Symptoms and Signs

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  • • Symptoms of specific hormone excess (cortisol excess, virilization, feminization)

    • Palpable abdominal mass

    • Abdominal pain

    • Fatigue, weight loss, fever, hematuria

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Laboratory Findings

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  • • 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

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Imaging Findings

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  • • 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

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  • • Mean diameter of adrenal carcinoma at diagnosis is 12 cm

    • Radiographic evaluation of suspected metastatic sites for purposes of staging should be undertaken prior to thought of any surgery

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Rule Out

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  • • Pheochromocytoma

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  • • 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

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  • • 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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Surgery

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Indications

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  • • Disease localized to the adrenal, or local spread

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Contraindications

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  • • Widely metastatic disease

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Medications

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  • • Mitotane (an adrenolytic agent) can be used as adjuvant therapy; controls ...

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