Management of functioning tumors of the adrenal cortex

• 1.1. The technical and physiologic management of functioning adrenocortical tumors is reviewed. The clinical pictures produced by various types of hyperadrenocorticism are Cushing's syndrome, hyperaldosteronism, the adrenogenital syndrome, feminization and mixed syndromes.
• 2.2. The findings in the history and physical examination which suggest endocrine dysfunction should lead to specific plasma and steroid analyses that are essential to establish the diagnosis on the firmest possible basis.
• 3.3. The clinical picture produced is determined by the specific chemical formula of the overproduced steroid, in addition to the age and sex of the patient.
• 4.4. Functioning adrenocortical tumors are frequently malignant and the prognosis is thus often poor.
• 5.5. The patient with Cushing's syndrome may complain only of weakness and weight gain for many months prior to the development of other more striking physical features of the condition. The diagnosis is established by the demonstration of an increased rate of production of gluco-corticoids. This may be done with control plasma levels, response to the intravenous infusion of ACTH and measurement of corticoid excretion in the urine. The urinary excretion of 17-ketosteroids is also elevated in many cases. Collateral laboratory data of value are derived from the blood count, plasma electrolyte values and urinalysis. The functioning adrenocortical tumor is excised, and during and after operation hydrocortisone is infused in substantial doses to prevent acute adrenocortical failure. An analysis of a far advanced case is presented. Long term follow-up examinations are important aspects of management.
• 6.6. Hyperaldosteronism due to functioning tumors of the adrenal cortex is now a well established entity. The condition should be at least considered in any patient who has hypertension, unexplained episodes of muscular weakness, hypokalemia with hypochloremia or alkalosis, and episodes of polyuria and polydipsia. There is a tendency toward hypernatremia. The diagnosis is confirmed by the demonstration of an increased level of aldosterone excretion in the urine.
• 7.7. The adrenogenital syndrome is due to an excessive production of androgens by adrenal hyperplasia or neoplasia. Such patients exhibit an increased level of 17-ketosteroid excretion in the urine at operation. The level of corticoid excretion may also be elevated. The tumor frequently appears to be encapsulated but is often malignant with blood vessel invasion, and recurrences are common. A case analysis is presented.
• 8.8. The qualitative and quantitative diagnosis of hyperadrenocorticism has now become far more precise than it was a decade ago. Furthermore, the hazard of acute postoperative adrenocortical insufficiency has been almost abolished by the availability and liberal use of effective replacement therapy.