Adrenocortical Carcinoma

• • 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

Epidemiology

• • 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 and Signs

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

  • Palpable abdominal mass

  • Abdominal pain

  • Fatigue, weight loss, fever, hematuria

Laboratory Findings

• • 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

Imaging Findings

• • 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

• • 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

Rule Out

• • Pheochromocytoma

• • 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

Surgery

Indications

• • Disease localized to the adrenal, or local spread

Contraindications