Adrenal Cortical Tumors

Adrenal Cortical Tumors

1. Primary Hyperaldosteronism



1. Clinical Manifestations
• accounts for 1% of all cases of hypertension
• Conn’s syndrome: hypertension, hypokalemia, polyuria
• hypertension often requires multiple medications to control
• hypokalemia may result leading to muscle weakness, cramps, or paresthesias
• may result from a solitary adenoma or bilateral adrenal hyperplasia


2. Diagnosis
1. Biochemical Screening
• should be performed in patients with hypertension and unexplained hypokalemia, or in patients requiring multiple antihypertensive agents
• initial study is to determine the plasma aldosterone (PAC) to plasma renin (PRA) ratio
• PAC:PRA ratio > 20 is abnormal and mandates confirmatory testing
• inappropriately high aldosterone levels (nonsuppressible) after oral or IV salt loading confirms the diagnosis



2. Localization
1. CT Scan
• most lesions are less than 15 mm in size, making them difficult to image



• if CT does not localize a lesion, or if bilateral nodules or adrenal hypertrophy are seen, then selective adrenal vein sampling is required







2. Selective Adrenal Vein Sampling (AVS)
• requires simultaneous measurement of cortisol and aldosterone levels in the IVC and right and left adrenal veins
• successful cannulation of an adrenal vein is confirmed by measuring a fivefold increase in cortisol relative to the IVC (positive control)
• aldosterone/cortisol ratios for each adrenal vein are compared
• a positive test requires an aldosterone/cortisol ratio > 4 on one side
• AVS has a relatively low success rate, with the major difficulty being in cannulating the right adrenal vein


3. Treatment
1. Laparoscopic Adrenalectomy
• indicated for a localized aldosteronoma
• spironolactone is used to optimize blood pressure before surgery
• potassium level should also be corrected before surgery
• 80% of patients will have normalization of blood pressure or a significant reduction in blood pressure medication requirement


2. Medical Therapy
• surgery is contraindicated for bilateral adrenal hyperplasia
• spironolactone is the preferred medical therapy


2. Cushing’s Syndrome
1. Clinical Manifestations
• syndrome of chronic glucocorticoid excess: hypertension, hyperglycemia, truncal obesity, moon facies, easy bruising, muscle atrophy
• most common etiology is iatrogenic administration
• non-iatrogenic etiologies include pituitary adenoma (Cushing’s disease – 75%), adrenal adenoma (15%), and ectopic ACTH production (NSCLC – 10%)


2. Diagnosis
1. Biochemical Screening
• 24-hour urine free cortisol test is the most sensitive and specific for Cushing’s syndrome
• after Cushing’s syndrome is confirmed, then measuring ACTH is necessary to determine the specific cause – pituitary vs adrenal or ectopic
• adrenal disease will have very low or undetectable ACTH; high levels suggest a pituitary adenoma or ectopic production



2. Localization
• CT scan can visualize most solitary adrenal adenomas



• CT scan can also detect adrenocortical carcinoma or bilateral nodular hyperplasia







3. Treatment
1. Surgery
• laparoscopic adrenalectomy is preferred
• metyrapone or ketoconazole can be used to control cortisol excess preop
• exercise and a high-protein diet should be encouraged to counteract preexisting muscle atrophy
• perioperative stress doses of hydrocortisone should be given
• contralateral adrenal may remain suppressed for several weeks, so perioperative steroids will have to be gradually tapered
• 90% success rate, but resolution of symptoms may take months or years


2. Medical Therapy
• reserved for cases of bilateral nodular hyperplasia
• bilateral adrenalectomy is a last resort for patients who fail medical management


3. Adrenocortical Carcinoma
1. Epidemiology
• rare tumor
• annual incidence of 1/million population
• most patients are between ages 40 – 50
• also occurs in children < 5 years


2. Clinical Manifestations
• 60% of patients have symptoms of hormone excess – Cushing’s syndrome, Cushing’s syndrome and virilization, virilization alone (rare)
• mean tumor size is 9 – 13 cm
• local invasion is the rule: left adrenal cancers may invade the kidney, pancreas, spleen, diaphragm; right tumors may invade the liver or IVC
• 40% of patients present with metastatic disease: lung, liver, peritoneum, bone
• CT scan typically shows a large heterogenous mass with irregular borders, central necrosis, and local invasion



3. Treatment
• radical surgery with en bloc resection of involved organs or lymph nodes is the only chance for cure
• IVC invasion can be managed with resection/thrombectomy, and may require cardiopulmonary bypass
• adjuvant mitotane improves recurrence-free and overall survival in resected patients
• adjuvant XRT may benefit patients with positive resection margins or stage III disease


4. Adrenal Metastases
1. Clinical Features
• common site of metastasis because of the adrenal gland’s extensive blood supply
• most common primary cancers include lung, GI tract, breast, kidney, melanoma
• usually bilateral
• most often associated with other sites of metastatic disease
• adrenalectomy in the rare patient with an isolated adrenal metastasis can be associated with long term survival