Endocrine: Adrenal Cortex

General Information / Anatomy / Function / Statistics

• Adrenal glands are organs located near the kidneys
• Each of two glands is composed of a cortex (outer shell) and a medulla (inner core)
  Cortex produces 4 hormones:
  • Corticosteroids
  • Hydrocortisone, which helps control the metabolism of protein, fat and carbohydrates
  • Aldosterone, which influences the sodium and potassium levels in body fluids
  • Androgens and estrogens, which affect secondary sex characteristics in both males and females, i.e. facial hair
• Medulla secretes epinephrine (adrenaline) and norepinephrine which help regulate the part of the C.N.S. that controls the muscles of the heart, digestive system and respiratory system
• Cancer of the adrenal cortex is rare
• Variably malignant
• Usually diagnosed only after regional or distant metastatic spread
• Mortality rate less than 0.2 % of all cancer deaths combined
• For statistics, please click here

Symptoms / Signs

• Sometimes there are no symptoms
• Major non-endocrine symptom is pain
• Most common sign is an abdominal mass
• Local extension may displace, obstruct or destroy the kidney and cause pain, hemorrhage and distention
• Weight loss, anorexia, fever and sweats
• Widespread metastases to deep and superficial nodes can appear as lymphadenopathy on a chest X-ray or physical examination
• Bone metastases are unusual
• Hormonal patterns when functioning tissue is involved, i.e. Cushing's Syndrome, virilization, feminization, masculinization, hypertension
• Puberty may come early to children
• Anxiety attacks
• Headaches

Etiology / Carcinogens / Risks

• No etiologic factors have been found
• No familial predisposition
• No particular geographic or climatic distribution
• Malignant adrenal tumours that are confined to the gland are treatable and curable
• Tumours that produce more than one hormone are often malignant
• People with neurofibromatosis and the von Hippel-Lindau syndrome as well as the multiple endocrine neoplasia (MEN2) syndrome are more likely to develop pheochromocytoma

Prevention

• Since etiology is unknown, no preventive measures can be taken.  

Diagnosis / Screening / Staging / Grading / Types

• I.V.P. (intravenous pyelogram) may indicate a suprarenal mass displacing the kidney
• Suprarenal computed tomography (scanning) is the most simple and efficacious test in evaluating adrenal gland neoplasms. Its use has eliminated necessity of retroperitoreal pneumography
• Angiography can demonstrate an extensive vascular network in the adrenal neoplasm and differentiate adrenal gland from upper pole renal tumours
• Adrenal venography - newer technique producing a more precise outline for functional localization of the lesion in cases of hyperaldosteronism (Conn's Syndrome)
• Laboratory studies - urinary excretions of steroids are valuable
• Biopsy is necessary for a definitive diagnosis
• CT and MRI scans

• Curative surgery could be attempted
• Aggressive resection of primary tumour and metastatic sites especially in liver may extend life considerably
• Lesions are usually large so radical "en bloc" excision, often including the spleen, kidney, and part of the pancreas, may be necessary
• Side exposure using a thoracoabdominal approach is frequently necessary
• Small tumours may require only a wide-transverse subcostal incision
• Primary tumours should be removed as much as possible in order to remove the source of the hormones

Radiation Therapy

• Pre- and post-operative radiation therapy have been used with the hope of eradicating local extension but is of limited usefulness
• Requires precise localization techniques, multiple fields and computerized treatment planning
• Gross disease can be arrested but will seldom be ablated since doses required for tumour control exceed surrounding normal tissue tolerance

Chemotherapy

• Can be helpful in inducing regression in primary and metastatic tumour size
• Mitotane most commonly used drug
• Glucocorticoid and mineral corticoid replacement is necessary in most cases to prevent adrenal insufficiency
• In a few cases, chemotherapeutic agents may have some antitumour activity, e.g., cyclophosphamide, doxorubicin, and cisplatin.