AACE and AAES Medical Guidelines for the Management of Adrenal Incidentalomas

The incidence of adrenal incidentaloma, a term coined in reference to the phenomenon of detecting an otherwise unsuspected adrenal mass on radiologic imaging, has been increasing and now approaches the 8.7% incidence reported in autopsy series (1 [evidence level or EL 3], 2 [EL 3]). The definition of incidentaloma excludes patients undergo-ing imaging procedures as part of staging and work-up for cancer. Not only are more incidentalomas being detected by imaging but they are increasingly more likely to be functional because of the more common evaluations for subclinical syndromes (3 [EL 2]). During the evaluation of an adrenal mass, 3 questions need to be addressed: (1) Is the tumor hormonally active? (2) Does it have radio-logic characteristics suggestive of a malignant lesion? and (3) Does the patient have a history of a previous malig-nant lesion? The patient should be tested for evidence of hypercortisolism, aldosteronism (if hypertensive), and the presence of a pheochromocytoma. A summary of the lit-erature revealed that approximately 80% of patients with incidentalomas had a nonfunctioning adenoma, 5% had subclinical Cushing syndrome (SCS), 5% had a pheochro-mocytoma, 1% had an aldosteronoma,

Before consideration of surgical resection, a high degree of certainty of the diagnosis is critical and can be achieved with a combination of biochemical and radio-graphic studies. Patients who present with an adrenal incidentaloma should be referred to an endocrinologist or endocrine surgeon for assessment. Pheochromocytomas necessitate careful preoperative preparation to avoid intra-operative and postoperative morbidity and mortality. In patients with primary aldosteronism, a thorough evalua-tion should be performed to ensure that they do not have adrenocortical hyperplasia and a nonfunctioning adrenal adenoma. Patients with adrenal Cushing syndrome develop adrenal insufficiency after resection and will require steroid coverage and careful withdrawal. Whether those with SCS require surgical treatment is controversial. Those patients with ACC require preoperative planning in collaboration with an endocrinologist or oncologist because the effec-tiveness of the initial resection can be a major predictor of survival. Finally, nonfunctioning adrenal tumors ≥4 cm (Fig. 1) should be considered for surgical resection. In con-trast, small myelolipomas, benign cysts, or nonfunctioning adenomas can be diagnosed with considerable certainty and usually do not necessitate surgical resection unless symptomatic. Depending on the clinical circumstances, resection may be indicated.

The only previously published clinical practice guide-lines on the management of patients with adrenal inciden-talomas originated from a National Institutes of Health consensus conference and was published in 2002 (6 [EL 4]). This current set of clinical practice guidelines sum-marizes the relevant literature as it pertains to the differ-ential diagnosis, laboratory and radiologic evaluation, and clinical management and includes recommendations (each labeled “R” in the subsequent section) based on the “best evidence” rating level (BEL) of the published sources.