What are the guideline sources, guidelines and references for Adrenal incidentaloma ?
Guideline sources The following summarized guidelines for the evaluation and management of adrenal incidentaloma (AI) are prepared by our editorial team based on guidelines from the Canadian Urological Association (CUA 2023), the American Association of Endocrine Surgeons (AAES 2022), the American College of Radiology (ACR 2021), the European Society of Endocrinology (ESE/ENSAT 2016), and the Society of American Gastrointestinal and Endoscopic Surgeons (SAGES 2013). 1 2 3 4 5 6 7 8 9 Definition AI refers to an adrenal mass unexpectedly detected through an imaging procedure performed for reasons unrelated to adrenal pathology. 6 Epidemiology AIs are caused due to hormone excess (adenoma, carcinoma, pheochromocytoma, congenital adrenal hyperplasia, massive macronodular adrenal disease, nodular variant of Cushing's disease), no hormone excess (adenoma, myelolipoma, neuroblastoma, ganglioneuroma, hemangioma, carcinoma, metastasis, cyst, hemorrhage, granuloma, amyloidosis, and infiltrative disease. 7 https://web.pathway.md/diseases/recl3lB15yicFTcCE 1/8 6/23/23, 2:09 AM Adrenal incidentaloma Pathway Disease course A cluster of different pathologies in the adrenal gland due to physiological process of aging detected incidentally during an imaging procedure is known as AI. The majority of tumors are non-functioning, but incidentaloma with hormonal and malignancy evolution may cause life-threatening complications. 8 Prognosis and risk of recurrence AIs are not associated with an increase in mortality, except for adrenal metastasis. 9 Guidelines 1. Classification and risk stratification Risk stratification: consider using washout characteristics on an adrenal protocol CT to stratify the risk of malignancy for adrenal nodules when the non-contrast HU is > 10 and other clinical risk factors for malignancy are not present. Recognize that adrenal protocol CT does not improve diagnostic accuracy for nodules with non-contrast HU < 10 nor does it improve evaluation for pheochromocytoma. C Show 2 more 2. Diagnostic investigations History and physical examination: Elicit a focused history and perform a physical examination to identify signs/symptoms of adrenal hormone excess, adrenal malignancy, and/or extra-adrenal malignancy in patients with an AI. E Set a low threshold for a multidisciplinary review by endocrinologists, surgeons, and radiologists when the imaging is not consistent with a benign lesion, there is evidence of hormone hypersecretion, the tumor has grown significantly during follow-up imaging, or adrenal surgery is being considered. E Diagnostic imaging: As per CUA 2023 guidelines: Obtain non-contrast CT as first-line imaging to distinguish benign lesions from those requiring further radiological investigation in patients with an indeterminate incidental adrenal mass. B Obtain washout CT or chemical-shift MRI as second-line imaging in patients continuing to have an indeterminate adrenal mass on non-contrast CT. B As per AAES 2022 guidelines, consider obtaining adrenal protocol CT to determine washout characteristics in order to stratify the risk of malignancy of adrenal nodules. C As per ACR 2021 guidelines, do not obtain imaging for the initial evaluation of patients with no prior history of malignancy and an incidentally detected indeterminate adrenal mass < 1 cm. D https://web.pathway.md/diseases/recl3lB15yicFTcCE 2/8 6/23/23, 2:09 AM Adrenal incidentaloma Pathway Show 4 more As per ESE 2016 guidelines, obtain adrenal-specific imaging to determine if the mass is homogeneous and lipid-rich (therefore benign) in all patients with AIs. B Show 2 more Dexamethasone suppression test: As per CUA 2023 guidelines, evaluate for autonomous cortisol secretion in all patients with AIs. B Obtain a 1-mg dexamethasone suppression test to identify autonomous cortisol secretion when clinically appropriate. B As per AAES 2022 guidelines, obtain biochemical testing to identify autonomous cortisol secretion in all patients with an AI 1 cm. B As per ESE 2016 guidelines, obtain a 1-mg overnight dexamethasone suppression test to exclude cortisol excess in all patients with AIs. B Show 2 more Metanephrine assays: As per CUA 2023 guidelines: Obtain plasma or 24-hour urinary metanephrines to evaluate for pheochromocytoma in patients with an AI displaying 10 HUs on non-contrast CT or having signs/symptoms of catecholamine excess. B Avoid obtaining evaluation for pheochromocytoma in patients with unequivocal adrenocortical adenomas confirmed on unenhanced CT (< 10 HUs) and no signs or symptoms of adrenergic excess. D As per AAES 2022 guidelines, obtain biochemical evaluation for pheochromocytoma in patients with non-contrast adrenal CT findings of HU > 10. B As per ESE 2016 guidelines, obtain plasma free metanephrines or urinary fractionated metanephrines to exclude pheochromocytoma. A Aldosterone-to-renin ratio: As per CUA 2023 guidelines, obtain aldosterone-to-renin ratio to evaluate for primary aldosteronism in patients with an AI and hypertension and/or hypokalemia. B As per AAES 2022 guidelines, obtain biochemical evaluation for primary aldosteronism in patients with hypertension or hypokalemia. B As per ESE 2016 guidelines, obtain aldosterone-to-renin ratio to evaluate for primary aldosteronism in patients with concomitant hypertension or unexplained hypokalemia. A Hormonal evaluation: As per CUA 2023 guidelines, obtain serum testing for excess androgen in patients with suspected adrenocortical carcinoma and/or when clinical signs of virilization are present. E As per AAES 2022 guidelines, obtain a directed hormonal evaluation in patients with an adrenal mass regardless of history of extra-adrenal malignancy. B https://web.pathway.md/diseases/recl3lB15yicFTcCE 3/8 6/23/23, 2:09 AM Adrenal incidentaloma Pathway As per ESE 2016 guidelines, consider measuring sex hormones and steroid precursors in patients with clinical or imaging features suggestive of adrenocortical carcinoma. B 3. Diagnostic procedures Adrenal mass biopsy: As per CUA 2023 guidelines, do not perform adrenal mass biopsy routinely for the evaluation of an AI. D As per AAES 2022 guidelines, consider performing an image-guided biopsy of a radiographically indeterminate mass, only after confirming a lack of hormone excess, if the results will change overall disease management. B As per ACR 2021 guidelines, perform adrenal gland biopsy or obtain FDG-PET/CT from the skull base to mid-thigh in patients with a history of malignancy and an indeterminate adrenal mass 4 cm without diagnostic benign imaging characteristics on initial images. B As per ESE 2016 guidelines, consider performing adrenal mass biopsy if all of the following criteria are met: the lesion is hormonally inactive (pheochromocytoma has been excluded) the lesion has not been conclusively characterized as benign by imaging determination of histology will affect the management. C 4. Therapeutic procedures Ablation and stereotactic radiation: avoid performing ablation or stereotactic radiation as an alternative to adrenalectomy in patients with adrenal lesions because of inadequate data supporting these modalities. Include surgeons in the decision-making early in the treatment algorithm. D 5. Perioperative care Perioperative corticosteroids: administer perioperative corticosteroids at major surgical stress doses in all patients undergoing surgery for an adrenal tumor when there is evidence of 'possible autonomous cortisol secretion', as evidenced by a lack of cortisol suppression to < 50 nmol/L during the 1-mg dexamethasone overnight suppression test. A 6. Surgical interventions Indications for adrenalectomy, nonfunctioning tumors: As per CUA 2023 guidelines: Consider performing adrenalectomy in patients with AIs growing > 5 mm/year after repeating a functional workup. C https://web.pathway.md/diseases/recl3lB15yicFTcCE 4/8 6/23/23, 2:09 AM Adrenal incidentaloma Pathway Ensure shared decision-making for the management (repeat imaging in 3-6 months versus surgical resection) of patients with indeterminate nonfunctioning adrenal lesions. E As per AAES 2022 guidelines, avoid performing resection of a myelolipoma or adrenal cyst with pathognomonic imaging features to improve the patient's quality of life unless there are symptoms of mass effect. D As per ENSAT/ESE 2016 guidelines, do not perform adrenalectomy in patients with an asymptomatic, nonfunctioning unilateral adrenal mass and obvious benign features on imaging. D Indications for adrenalectomy, hormone-secreting tumors: As per CUA 2023 guidelines, perform unilateral adrenalectomy of the affected adrenal gland, with a minimally invasive technique when feasible, in patients with unilateral cortisol-secreting adrenal masses and clinically apparent Cushing's syndrome. E Show 2 more As per ENSAT/ESE 2016 guidelines, perform adrenalectomy as the standard of care in patients with unilateral adrenal tumors with clinically significant hormone excess. Individualize the decision for surgery according to age, degree of cortisol excess, general health, comorbidities, and patient preferences. Confirm that cortisol excess is independent of ACTH. A Indications for adrenalectomy, malignant tumors: As per CUA 2023 guidelines: Consider performing minimally invasive adrenalectomy in patients with suspected adrenocortical carcinomas suitable for safe resection without rupturing the tumor capsule. C Consider performing open adrenalectomy in patients with larger adrenocortical carcinomas or presenting with locally advanced tumors, lymph node metastases, or tumor thrombus in the renal vein/IVC. B As per ENSAT/ESE 2016 guidelines: Perform open adrenalectomy in patients with unilateral adrenal masses with radiological findings suspicious of malignancy and signs of local invasion. B Consider performing laparoscopic adrenalectomy in patients with unilateral adrenal masses with radiological findings suspicious of malignancy and a diameter 6 cm but without evidence of local invasion. C Technical considerations for adrenalectomy: As per AAES 2022 guidelines, perform minimally invasive adrenalectomy over open adrenalectomy for improved perioperative morbidity when patient and tumor characteristics are appropriate. B Show 2 more As per SAGES 2013 guidelines, prefer minimally invasive adrenalectomy over open surgery for all non-primary adrenal cancer pathology due to less postoperative pain, shorter hospital stay, earlier recovery, and similar long-term outcomes. B Show 8 more https://web.pathway.md/diseases/recl3lB15yicFTcCE 5/8 6/23/23, 2:09 AM Adrenal incidentaloma Pathway 7. Specific circumstances Pediatric, pregnant and elderly patients: obtain an urgent assessment of the adrenal mass in pediatric patients, adolescent patients, pregnant, and < 40 years old adult patients because of a higher likelihood of malignancy. A Show 2 more Patients with bilateral adrenal incidentalomas (evaluation): assess bilateral adrenal masses separately, according to the same imaging protocol as for unilateral adrenal masses, to establish if either or both masses are benign or malignant. A Show 2 more Patients with bilateral adrenal incidentalomas, management: As per ENSAT/ESE 2016 guidelines, consider applying the same indications for surgery and follow-up identical to the recommendations in patients with unilateral AI. C Show 2 more As per SAGES 2013 guidelines: Consider preferring the posterior retroperitoneal approach to eliminate patient repositioning during bilateral adrenalectomies. C Consider performing laparoscopic cortical-sparing surgery as the procedure of choice in patients requiring bilateral adrenalectomy (such as for hereditary pheochromocytomas). C Patients with autonomous cortisol secretion: As per AAES 2022 guidelines, perform laparoscopic adrenalectomy for anticipated significant improvements in cardiometabolic comorbidities in patients with mild autonomous cortisol secretion secondary to a unilateral adenoma. B Show 3 more As per ESE 2016 guidelines, do not view 'autonomous cortisol secretion' as a condition with a high risk for the development of overt Cushing's syndrome. D Show 3 more Patients with extra-adrenal malignancy: obtain plasma or urinary metanephrines to exclude pheochromocytoma in patients with extra-adrenal malignancy with an indeterminate mass, even if the adrenal mass is likely to be a metastasis. Consider obtaining additional hormonal work-up using an individualized approach. A Show 3 more Patients with adrenal metastases: As per AAES 2022 guidelines, consider offering resection of adrenal metastases, after multidisciplinary review, to improve survival in highly selected patients. C As per SAGES 2013 guidelines, consider using a laparoscopic approach, by a surgeon skilled in advanced laparoscopy and adrenal surgery, in patients with solitary adrenal metastases without evidence of local invasion. C Convert to an open approach if a local invasion is found intraoperatively. B https://web.pathway.md/diseases/recl3lB15yicFTcCE 6/8 6/23/23, 2:09 AM Adrenal incidentaloma Pathway 8. Follow-up and surveillance Serial imaging assessment: As per CUA 2023 guidelines, do not obtain further follow-up imaging in patients with benign nonfunctioning adenomas < 4 cm, myelolipomas, and other small masses containing macroscopic fat detected on the initial workup for an AI. D Show 2 more As per AAES 2022 guidelines: Do not obtain routine scheduled follow-up of a nonfunctional adrenal nodule (size < 4 cm) with benign imaging characteristics and non-contrast HU < 10 because the risk of developing malignancy is very low. D Obtain at least one repeated image at 6-12 months to confirm stability in patients with nodules 1-4 cm with indeterminate imaging characteristics (such as non-contrast CT with HU > 10) because of a slightly increased risk of malignancy. B As per ACR 2021 guidelines, obtain non-contrast MRI or non-contrast/contrast-enhanced CT of the abdomen for the 12-month follow-up imaging of patients with no prior history of malignancy and an incidentally detected indeterminate adrenal mass 1-2 cm without diagnostic benign imaging characteristics. B As per ESE 2016 guidelines: Avoid obtaining further imaging for follow-up in patients with an adrenal mass < 4 cm with clear benign features on imaging studies. D Consider obtaining a repeat non-contrast CT or MRI after 6-12 months to exclude significant growth in patients with an indeterminate adrenal mass opting not to undergo adrenalectomy following initial assessment. C Consider performing surgical resection if the lesion enlarges by > 20%m in addition to at least a 5 mm increase in maximum diameter, during this period. C Obtain additional imaging after 6-12 months if there is a growth of the lesion below this threshold. B Serial laboratory assessment: As per CUA 2023 guidelines: Do not obtain further follow-up function testing in patients with benign nonfunctioning adenomas < 4 cm, myelolipomas, and other small masses containing macroscopic fat detected on the initial workup for an AI. D Do not obtain further follow-up function testing in patients with adrenal lesions growing < 3 mm/year on follow-up imaging. D As per AAES 2022 guidelines, consider reevaluating for autonomous cortisol secretion at 2-5- year intervals as it is the most common hormonal excess to develop during surveillance. B As per ESE 2016 guidelines: Avoid obtaining repeated hormonal workup in patients with a normal hormonal workup at the initial evaluation unless new clinical signs of endocrine activity appear or there is a worsening of comorbidities (such as hypertension or T2DM). D https://web.pathway.md/diseases/recl3lB15yicFTcCE 7/8 6/23/23, 2:09 AM Adrenal incidentaloma Pathway Consider obtaining annual clinical reassessment for cortisol excess comorbidities potentially related to cortisol excess in patients with 'autonomous cortisol secretion' without signs of overt Cushing's syndrome. Discuss the potential benefit of surgery based on the outcome of this evaluation. C References 1. Fassnacht M, Arlt W, Bancos I et al. Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors. Eur J Endocrinol. 2016 Aug;175 2 G1 G34. Open 2. Linwah Yip, Quan-Yang Duh, Heather Wachtel et al. American Association of Endocrine Surgeons Guidelines for Adrenalectomy: Executive Summary. JAMA Surg. 2022 Aug 17. Open 3. Dimitrios Stefanidis, Melanie Goldfarb, Kent W Kercher et al. SAGES guidelines for minimally invasive treatment of adrenal pathology. Surg Endosc. 2013 Nov;27 11 3960 80. Open 4. Neal E Rowe, Ravi M Kumar, Nicola Schieda et al. Canadian Urological Association guideline: Diagnosis, management, and followup of the incidentally discovered adrenal mass. Can Urol Assoc J. 2023 Feb;17 2 12 24. Open 5. Expert Panel on Urological Imaging, Rekha N Mody, Erick M Remer et al. ACR Appropriateness Criteria Adrenal Mass Evaluation: 2021 Update. J Am Coll Radiol. 2021 Nov;18 11S S251 S267. Open 6. 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European Society of Endocrinology Clinical Practice Guidelines on the management of adrenocortical carcinoma in adults, in collaboration with the European Network for the Study of Adrenal Tumors. Eur J Endocrinol. 2018 Oct 1;179 4 G1 G46. Open https://web.pathway.md/diseases/recl3lB15yicFTcCE 8/8