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Adrenal Tumors
Diagnosis/Definition
- An adrenal mass by CT, MRI, or ultrasound in an asymptomatic or symptomatic patient (Cushing’s syndrome, pheochromocytoma, aldosteronoma, or other disorders). The mass may be unilateral or bilateral.
- Adrenal masses have an estimated prevalence of 1.4-8.7% in autopsy studies, 0.6% by CT scan.
- It is important to consider pre-test probability when ordering and interpreting laboratory studies on this population. For example, less than one in 1,000 patients with hypertension has a pheochromocytoma.
Initial Diagnosis and Management
- In general, an adrenal mass will be an incidental finding on an imaging study obtained for another purpose. Radiographic studies should only be ordered after a biochemical diagnosis has been established.
- In addition to searching for signs and symptoms of a functional adrenal tumor, the history and physical exam should concentrate on the exclusion of cancers which are commonly metastatic to the adrenal. These include lung, breast, GI, and prostate cancers. Syndromes of Multiple Endocrine Neoplasia (MEN) should be in the diagnostic differential.
- Pheochromocytoma is suggested by headache, inappropriate and excessive sweating and palpitations in a patient with paroxysmal or persistent hypertension. Useful screening labs include urinary metanephrines, normetanephrines, VMA, and fractionated catecholamines. These studies require special dietary and medication considerations. Values greater than 2 x the upper limit of normal are suggestive of a pheochromocytoma. The measurement of free metanephrines in plasma is an alternative.
- Primary hyperaldosteronism is suggested by spontaneous hypokalemia (K+ < 3.5 in the absence of diuretics or K+ < 3.0 in patients taking diuretics and potassium supplementation), inappropriate kaluresis (24 h urine potassium > 30 mEq in face of hypokalemia) and hypertension. Screen for primary hyperaldosteronism by obtaining a random plasma aldosterone concentration (PAC) and measuring plasma renin activity (PRA). Refer any patient with a PAC/PRA > 20 AND PAC > 15 ng/dL to an endocrinologist.
- Cushing’s syndrome is suggested by a cushingoid body habitus, hypertension, glucose intolerance or diabetes, and other characteristic signs and symptoms. Useful screening tests include the 1 mg overnight dexamethasone suppression test, assessment of the circadian production of cortisol, or the collection of 24 hour urine free cortisol.
- Some patients with apparently normal endocrine function will have active adrenal adenomas that have suppressed the contralateral adrenal. These patients are at risk for an adrenal crisis if the adenomatous adrenal is surgically removed.
Ongoing Management and Objectives
- The initial evaluation is intended to (1) exclude metastases from cancers in other locations; (2) determine the probability of adrenocortical carcinoma based on size and appearance of the mass; and (3) identify Cushing’s syndrome, aldosteronoma or pheochromocytoma if present. Another objective is to identify tumors with a potential for growth.
- The presumed incidental adrenal mass should be re-imaged at 3 months, 6 months, and every 6-12 months thereafter until it is evident that the mass is stable.
Indications for Specialty Care Referral
Patients with biochemically inactive masses < 3 cm and without intramural necrosis, hemorrhage or irregular margins can be followed in primary care with serial imaging as noted above.
Patients with tumors > 3 cm in size or with intramural necrosis, hemorrhage or irregular margins on imaging should be referred to an endocrinologist before being sent to a surgeon.
Patients with positive screening tests for pheochromocytoma, Cushing’s syndrome, or aldosteronoma or those in whom the diagnosis of these conditions seems probable should be referred to an endocrinologist before being sent to a surgeon.
Patients with MEN syndromes and their kindred should be referred to an endocrinologist.
Criteria for Return to Primary Care
Exclusion of a functional adrenal mass.
Establishment of a plan of care for the patient with a functional adrenal mass.
Conclusion of surgical episode for the patient undergoing adrenalectomy.
