Last updated August 30, 2022

Adrenalectomy

Incidentalomas, Myelolipomas, and Cysts

We suggest that washout characteristics on an adrenal protocol CT be used to stratify the risk of malignancy for adrenal nodules when noncontrast HU are greater than 10 and other clinical risk factors for malignancy are not present. Adrenal protocol CT does not improve diagnostic accuracy for nodules with noncontrast HU less than 10 nor does it improve evaluation for pheochromocytoma. (C, L)
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We recommend that all patients with an adrenal incidentaloma 1 cm or larger undergo biochemical testing for autonomous cortisol secretion. Patients with hypertension or hypokalemia also require biochemical evaluation for primary aldosteronism. Patients with adrenal imaging findings that have noncontrast CT with HU greater than 10 should undergo evaluation for pheochromocytoma. (S, L)
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We recommend that a primary adrenal malignancy be considered in patients with an adrenal incidentaloma larger 4 cm and/or HU greater than 20 on noncontrast CT and in any patient younger than 18 years. We recommend that patients with a history of extra-adrenal malignancy be recognized to be at increased risk for adrenal metastases. (S, L)
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We do not recommend routine scheduled follow-up of a nonfunctional adrenal nodule (size <4 cm) with benign imaging characteristics and noncontrast HU less than 10 because the risk of developing malignancy is very low. Nodules from 1 to 4 cm with indeterminate imaging characteristics (such as noncontrast CT with HU >10) have a slightly increased risk of malignancy and should undergo at least 1 repeated image at 6 to 12 months to confirm stability. Autonomous cortisol secretion is the most common hormonal excess to develop during surveillance and thus may be reevaluated at a 2- to 5-year interval. (S, L)
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We do not suggest resecting a myelolipoma or adrenal cyst with pathognomonic imaging features to improve the patient’s quality of life unless there are symptoms of mass effect. (C, L)
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Primary Aldosteronism

We recommend that patients undergo laparoscopic adrenalectomy for unilateral PA because they are more likely to use fewer medications with lower defined daily doses to achieve normalization of blood pressure and potassium levels and have lower risks of new-onset atrial fibrillation, chronic kidney disease, stroke, and all-cause mortality. (S, L)
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We suggest that in patients 35 years and younger with cross-sectional imaging demonstrating a unilateral adenoma and a normal contralateral gland, AVS may be deferred because adrenalectomy directed by CT imaging alone has a cure rate similar to adrenalectomy guided by AVS. However, AVS should still be considered for all patients older than 35 years. (C, L)
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We recommend laparoscopic adrenalectomy for primary aldosteronism due to unilateral disease because it improves quality of life and reduces health care–related costs. (S, L)
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Hypercortisolism

We recommend that patients with MACS secondary to a unilateral adenoma undergo laparoscopic adrenalectomy because of anticipated significant improvements in cardiometabolic comorbidities. (S, M)
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In patients with bilateral macronodular hyperplasia, we suggest consideration of unilateral laparoscopic adrenalectomy in patients with CS as an attempt to achieve biochemical remission of hypercortisolism without causing permanent adrenal insufficiency. (C, L)
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We suggest that patients with moderate to severe ACTH-dependent hypercortisolism refractory to source control undergo bilateral laparoscopic adrenalectomy to ameliorate cortisol excess and improve disease-free survival and mortality. Postoperative adrenal insufficiency is a life-threatening condition that should be prevented and promptly managed in patients undergoing adrenalectomy. Symptoms include fatigue, hypotension, anorexia, abdominal pain, weakness, syncope, back pain, nausea, vomiting, fever, and confusion.

(C, L)
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The incidence of adrenal insufficiency after unilateral adrenalectomy is nearly 100% in patients with overt CS and about 60% in patients with MACS. We recommend empirical postoperative glucocorticoid replacement therapy for all patients with overt CS after undergoing unilateral adrenalectomy. However, we recommend that in patients with MACS, postoperative day 1 morning cortisol or corticotropin stimulation testing could be used to determine the need for glucocorticoid replacement therapy. (S, L)
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Adrenocortical Carcinoma

We recommend that patients with clinical and radiographic findings consistent with ACC should be treated at high-volume multidisciplinary centers to improve recurrence outcomes; data on overall survival are inconclusive. (S, L)
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Regardless of operative approach, we recommend an en bloc radical resection with an intact capsule to microscopically negative (R0) margins because of improved survival. Although open resection is preferred when ACC is suspected, the choice of operative approach should be based on the certainty of a complete R0 resection without tumor disruption. (S, L)
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We suggest that patients with systemic disease be offered resection of the primary tumor if all sites of disease are reasonably amenable to resection or local treatment and if performance status allows. Surgery may also be considered in patients with hormone excess medically refractory to steroidogenic inhibition. (C, L)
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We recommend that neoadjuvant systemic therapy be administered for advanced ACC when R0 surgical resection is not initially feasible. We recommend up-front surgical intervention when R0 resection is possible. (S, L)
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Metastasis to the Adrenal Gland

We recommend that a directed hormonal evaluation should be performed in patients with an adrenal mass regardless of history of extra-adrenal malignancy. (S, L)
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We suggest that in the setting of a radiographically indeterminate mass, image-guided biopsy be rarely performed and reserved for patients in whom results would change overall disease management and that it be performed only after confirming lack of hormone excess. (S, L)
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We suggest that after multidisciplinary review, resection may be offered to highly selected patients to improve survival compared with systemic therapy alone. (C, L)
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Pheochromocytoma and Paraganglioma

We recommend either selective or nonselective α blockade to safely prepare patients for surgical resection of PPGL, depending on the drug availability/cost, experience, and preference of the care team. While there is no significant difference in morbidity or mortality between selective and nonselective α blockade, selective blockade (doxazosin, prazosin, terazosin) is associated with more intraoperative hemodynamic instability while nonselective blockade (phenoxybenzamine) results in more postoperative hypotension. (S, M)
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Because of the decreased rate of steroid dependence, we recommend consideration of cortical-sparing adrenalectomy in patients with bilateral PCCs if technically feasible. However, the patient’s goals of care and a higher risk of recurrent pheochromocytoma should also be considered. (S, L)
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We suggest that in selected cases of metastatic PPGLs, resection of the primary tumor may be performed to improve overall survival. Patients should be carefully evaluated by a multidisciplinary care team to determine if the benefits of resection of the primary tumor outweigh the risks. (C, L)
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Technical Aspects

We recommend that adrenalectomy be preferentially performed by a high-volume adrenal surgeon to optimize outcomes, including lower rates of morbidity and mortality. (S, M)

The utility of percutaneous ablation, mainly with radiofrequency ablation, and stereotactic body radiation therapy for the destruction of hormonally active and inactive tumors and adrenal metastasis has been investigated in small retrospective studies. The studies suffer from small sample sizes and heterogeneity.

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We conditionally suggest ablation and stereotactic radiation not be used as an alternative to adrenalectomy for patients with adrenal lesions because there are inadequate data to support these modalities. Surgeons should be involved in the decision-making early in the treatment algorithm. (C, L)
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Recommendation Grading

Overview

Title

Adrenalectomy

Authoring Organization

Publication Month/Year

August 17, 2022

Document Type

Guideline

Country of Publication

US

Document Objectives

Adrenalectomy is the definitive treatment for multiple adrenal abnormalities. Advances in technology and genomics and an improved understanding of adrenal pathophysiology have altered operative techniques and indications. Objective is to develop evidence-based recommendations to enhance the appropriate, safe, and effective approaches to adrenalectomy.

Inclusion Criteria

Male, Female, Adult, Older adult

Health Care Settings

Hospital, Outpatient, Operating and recovery room

Intended Users

Nurse, nurse practitioner, physician, physician assistant

Scope

Management

Diseases/Conditions (MeSH)

D000315 - Adrenalectomy

Keywords

adrenalectomy

Source Citation

Yip L, Duh QY, Wachtel H, Jimenez C, Sturgeon C, Lee C, Velázquez-Fernández D, Berber E, Hammer GD, Bancos I, Lee JA, Marko J, Morris-Wiseman LF, Hughes MS, Livhits MJ, Han MA, Smith PW, Wilhelm S, Asa SL, Fahey TJ 3rd, McKenzie TJ, Strong VE, Perrier ND. American Association of Endocrine Surgeons Guidelines for Adrenalectomy: Executive Summary. JAMA Surg. 2022 Aug 17. doi: 10.1001/jamasurg.2022.3544. Epub ahead of print. PMID: 35976622.