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.
     

Treatment results in adrenal cortex malignant tumors

Malignant tumor of adrenal cortex without early diagnosis and radical surgical treatment is fatal. The results of treatment of 216 patients with adrenocortical carcinoma treated in 1968-2002, which is 32.6% of all the patients with tumors of adrenal cortex treated in the clinic were analyzed. In majority of cases the disease was hormonally active. The best result was observed after primary radical surgery at early stage (without regional metastases). Re-operation in majority of cases ensures remission, however, it cannot be considered as radical method of treatment. Chloditane application permits to prolongs survival, however it has usually been used as additional treatment.     

Tumors of the adrenal cortex

Three cases of adrenal cortical disease have been presented. These three demonstrate every variety of symptom complex which has sometimes been called the adreno-syndrome with the last case falling into the category of Cushing's syndrome.     

Physiology of the adrenal cortex

Knowledge of the functional morphology and physiology of the adrenal cortex and of the regulation of its secretory products is essential to understanding the meaning of the results of various tests in the diagnosis of adrenocortical disease and to providing the postoperative care necessary to correct this condition. Removal of a cortisol-producing adenoma will necessitate replacement of glucocorticoids only, whereas after resection of an aldosterone-producing adenoma, no steroid replacement will be required.