Management of Adrenal Incidentalomas
Publication Date: August 1, 2009
Last Updated: March 14, 2022
Recommendations
Patients with an adrenal incidentaloma should undergo evaluation clinically, biochemically, and radiographically for signs and symptoms of hypercortisolism, aldosteronism (if hypertensive), the presence of a pheochromocytoma, or a malignant tumor. (III, C)
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Patients with adrenal incidentalomas who do not fulfill the criteria for surgical resection need to have radiographic reevaluation at 3 to 6 months and then annually for 1 to 2 years. For all adrenal tumors, hormonal evaluation should be performed at the time of diagnosis and then annually for 5 years. (III, C)
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All patients found to have an incidental adrenal mass should be screened for cortisol excess. Although the best strategy for patients with incidentalomas has not been established, the simplest screening test for autono- mous cortisol secretion from an incidentaloma is a 1-mg overnight dexamethasone suppression test. If clinical suspicion is high, such as in patients with hypertension, obesity, diabetes mellitus, or osteoporosis, 3 tests (salivary cortisol, dexamethasone suppression, and urine free cortisol [UFC]) can be used. (III, C)
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After adrenalectomy for a cortisol-producing adenoma, patients should be treated with exogenous glucocorticoids until the hypothalamic-pituitary-adrenal (HPA) axis has recovered. This process may take 6 to 18 months after unilateral adrenalectomy. (III, C)
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A diagnosis of subclinical Cushing syndrome (SCS) is made if the serum cortisol level is more than 5.0 mg/dL after a 1-mg dexamethasone suppression test, in a patient with an adrenal adenoma and absence of typical physical stigmas of hypercortisolism. A low or suppressed level of adrenocorticotropic hormone (ACTH) or a low dehydroepiandrosterone sulfate concentration supports the diagnosis. (IV, D)
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A second abnormal test result of HPA axis function, such as a 2-day low-dose dexamethasone suppression test, may also be needed to establish the diagnosis of SCS. (II, B)
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In patients with SCS, until further evidence is available regarding the long-term benefits of adrenalectomy, surgical resection should be reserved for those with worsening of hypertension, abnormal glucose tolerance, dyslipidemia, or osteoporosis. (IV, D)
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Perioperative glucocorticoid therapy and postoperative assessment of HPA axis recovery are indicated in patients with SCS. (III, C)
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Patients thought to have a pheochromocytoma should undergo measurement of plasma fractionated metanephrines and normetanephrines or 24-hour total urinary metanephrines and fractionated catecholamines (or both plasma and urine studies). (I, A)
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About one-quarter of patients with a pheochromocytoma will have associated familial syndromes caused by mutations in the RET gene (multiple endocrine neoplasia type 2), VHL gene (von Hippel-Lindau disease), or succinate dehydrogenase genes; genetic study and counseling should be performed, especially for young patients or patients with an extra-adrenal pheochromocytoma. (III, C)
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Surgical resection should be performed for all pheochromocytomas. (III, C)
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In all patients with a pheochromocytoma, an α-adrenergic blocking agent should be administered preoperatively, in an effort to prevent intraoperative hemodynamic instability. (III, C)
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In patients who have undergone resection of a pheochromocytoma, long-term follow-up is necessary because 10% to 15% may have recurrence. (II, B)
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Screening for aldosteronism should be performed in patients with an aldosterone-to-renin ratio (ARR) of >20. (III, C)
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Primary aldosteronism is confirmed in the setting of an adrenal incidentaloma by demonstrating lack of aldosterone suppression (24-hour urine study) with salt loading. (III, C)
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Subtype evaluation should be achieved with highresolution computed tomographic (CT) scanning in all patients and adrenal venous sampling (AVS) in the majority of patients older than 40 years. (III, C)
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In patients with primary aldosteronism and a unilateral source of aldosterone excess, laparoscopic total adrenalectomy is the treatment of choice because it yields excellent outcomes with low associated morbidity relative to open approaches. (III, C)
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Patients with bilateral idiopathic hyperaldosteronism (IHA) or those not amenable or agreeable to surgical intervention should be managed with selective and nonselective mineralocorticoid receptor blockers. (I, A)
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Any adrenal mass with concerning radiographic characteristics and most lesions ≥4 cm should be resected because of increased risk of adrenal cancer. (III, C)
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The presence of pheochromocytoma should be ruled out biochemically before an attempted resection of any adrenal mass. (III, C)
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All patients suspected of having an adrenocortical carcinoma (ACC) should undergo biochemical evaluation to identify any potential hormone excess that serves as a tumor marker and to determine whether the patient requires steroid replacement perioperatively in cases of hypercortisolism. (IV, D)
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Open adrenalectomy should be performed if ACC is suspected. (III, C)
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A metastatic lesion should be suspected in a patient with a history of cancer and an adrenal mass that does not fulfill the criteria for an incidentaloma. (III, C)
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In very rare instances, pathologic confirmation with CT-guided needle biopsy may be required for staging and planning of oncologic treatments. (IV, D)
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The presence of pheochromocytoma should be ruled out with biochemical testing before performance of a biopsy. (III, C)
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Patients with bilateral adrenal metastatic lesions should undergo evaluation for adrenal insufficiency. (IV, D)
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Adrenal metastasectomy is rarely indicated but should be considered in the case of an isolated adrenal metastatic lesion. (III, C)
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Recommendation Grading
Overview
Title
Management of Adrenal Incidentalomas
Authoring Organizations
American Association of Clinical Endocrinologists
American Association of Endocrine Surgeons
Publication Month/Year
August 1, 2009
Last Updated Month/Year
August 21, 2023
Document Type
Guideline
External Publication Status
Published
Country of Publication
US
Inclusion Criteria
Male, Female, Adult, Older adult
Health Care Settings
Ambulatory
Intended Users
Nurse, nurse practitioner, physician, physician assistant
Scope
Diagnosis, Management
Diseases/Conditions (MeSH)
D000307 - Adrenal Gland Diseases, D000310 - Adrenal Gland Neoplasms
Keywords
Adrenal Incidentalomas
Source Citation
Martha Zeiger, Geoffrey Thompson, Quan-Yang Duh, Amir Hamrahian, Peter Angelos, Dina Elaraj, Elliott Fishman, and Julia Kharlip (2009) American Association of Clinical Endocrinologists and American Association of Endocrine Surgeons Medical Guidelines for the Management of Adrenal Incidentalomas: Executive Summary of Recommendations. Endocrine Practice: July 2009, Vol. 15, No. 5, pp. 450-453.