Adrenal cortical adenoma is often hormonally silent and discovered incidentally. In symptomatic patients, the resultant adrenal disease will reflect which hormone(s) is/are produced abundantly. There are specialized biochemical tests and imaging studies to make the diagnosis.
Presentation
Adrenal cortical adenomas are benign tumors that stem from the cortex of the adrenal gland. These adenomas are frequently discovered as adrenal incidentalomas, adrenal tumors confirmed on imaging during a workup for unrelated causes. Nearly 85% of adrenal incidentalomas are benign and nonfunctional [1] [2]. Functioning adrenal cortical adenomas may cause hypertension, orthostatic hypotension, and tachycardia. A physical exam may reveal central obesity, hirsutism, striae, and gynecomastia. An ophthalmologic exam may reveal retinopathy secondary to chronic hypertension. Endocrine diseases resulting from functioning adrenal cortical adenomas reflect the excess production of adrenal gland hormones such as aldosterone (primary aldosteronism), cortisol (Cushing's syndrome), and sex hormones.
Primary aldosteronism is not characterized by a specific clinical presentation. These patients may have unprovoked hypokalemia and hypertension although potassium levels can be normal [3]. As a result of hypernatremia, hypokalemic alkalosis, and other related mechanisms, patients may develop fatigue, weakness, headaches, tetany, and paresthesias. Also, polyuria and polydipsia could result from hypokalemia-induced nephrogenic diabetes insipidus. Complications secondary to chronic hypertension include renal, cardiac, neurologic, and ophthalmologic manifestations.
Cushing's syndrome is characterized by weakness, bruising, weight gain, and depression. Affected individuals may also develop osteoporosis and metabolic syndrome [4].
Finally, androgen or estrogen-secreting tumors may cause virilization in females and feminization in males.
Workup
All individuals suspected to have adrenal cortical adenoma, whether based on symptoms or an incidental finding on imaging, need a detailed assessment. The workup should include a personal and family history, a thorough physical exam, and appropriate studies.
In the evaluation of adrenal incidentalomas, it is very important to consider the possibility of pheochromocytoma [5]. To identify a pheochromocytoma, the first-line test is the measurement of plasma fractionated metanephrines and normetanephrines and/or 24-hour urine collection of metanephrines and catecholamines [6].
Primary aldosteronism is confirmed with the ratio of plasma aldosterone concentration to plasma renin activity [7]. Moreover, the diagnosis is likely when the aldosterone–to–renin ratio is above 30 and the plasma aldosterone level is elevated (>0.5 nmol/L) [8]. Also, an electrolyte panel should be obtained in these patients.
An important test in the diagnosis of Cushing's syndrome is an overnight dexamethasone suppression test [9]. Other considerations in these patients include screening for metabolic syndrome with a lipid panel and glucose tolerance test.
Patients with virilization or feminization should be tested for androgens and estrogens respectively.
On unenhanced computed tomography (CT) scan, adrenal cortical adenoma is characterized by a low attenuation which reflects the presence of an abundant intracytoplasmatic fat [10]. Also, CT and magnetic resonance imaging (MRI) are both effective in discriminating between benign and malignant masses. Additionally, positron-emission tomography (PET) with 18F-fluorodeoxyglucose (18F-FDG) demonstrates excellent sensitivity for confirming adrenal malignancy [11].
Fine-needle aspiration has limited value and is reserved for cases in which all other findings are inconclusive [12].
Treatment
Treatment for adrenal cortical adenoma depends on the tumor's functionality and size. Non-functioning adenomas that are small and asymptomatic may only require regular monitoring. Functioning adenomas, or those causing symptoms, often necessitate surgical removal, typically through a minimally invasive procedure called laparoscopic adrenalectomy. In cases where surgery is not feasible, medications may be used to manage hormone production and alleviate symptoms.
Prognosis
The prognosis for individuals with adrenal cortical adenoma is generally excellent, especially for non-functioning tumors. Surgical removal of functioning adenomas often results in the resolution of symptoms and normalization of hormone levels. Regular follow-up is essential to monitor for any recurrence or development of new symptoms. The risk of an adrenal adenoma becoming malignant is very low.
Etiology
The exact cause of adrenal cortical adenomas is not well understood. They are thought to arise from genetic mutations that lead to abnormal cell growth in the adrenal cortex. Some cases may be associated with genetic syndromes such as Multiple Endocrine Neoplasia (MEN) or familial adenomatous polyposis, but most occur sporadically without a clear hereditary pattern.
Epidemiology
Adrenal cortical adenomas are relatively common, especially in older adults. They are found in approximately 3-7% of individuals undergoing abdominal imaging for other reasons. The prevalence increases with age, and they are slightly more common in women than in men. Most adenomas are non-functioning and asymptomatic.
Pathophysiology
Prevention
There are no specific measures to prevent adrenal cortical adenomas, as their exact cause is not well understood. However, maintaining a healthy lifestyle, including regular exercise and a balanced diet, may help reduce the risk of developing conditions associated with hormone imbalances, such as hypertension and obesity, which can complicate the management of functioning adenomas.
Summary
Adrenal Cortical Adenoma is a benign tumor of the adrenal gland, often discovered incidentally. While most are non-functioning and asymptomatic, some can produce excess hormones, leading to clinical syndromes. Diagnosis involves imaging and hormonal evaluation, and treatment depends on the tumor's functionality and size. The prognosis is generally excellent, especially with appropriate management.
Patient Information
If you have been diagnosed with an adrenal cortical adenoma, it's important to understand that these tumors are usually benign and often do not cause symptoms. Your doctor will determine if the adenoma is producing hormones and whether treatment is necessary. Regular monitoring and follow-up are key to managing this condition effectively. If surgery is required, it is typically minimally invasive and has a high success rate.
References
- Cawood TJ, Hunt PJ, O'Shea D, Cole D, Soule S. Recommended evaluation of adrenal incidentalomas is costly, has high false-positive rates and confers a risk of fatal cancer that is similar to the risk of the adrenal lesion becoming malignant; time for a rethink? Eur J Endocrinol. 2009;161(4):513-527.
- Nieman LK. Approach to the patient with an adrenal incidentaloma. J Clin Endocrinol Metab. 2010;95(9):4106-4113.
- Cruz DN, Perazella MA. Hypertension and hypokalemia: unusual syndromes. Conn Med. 1997;61(2):67-75.
- Chiodini I, Morelli V, Masserini B, et al. Bone mineral density, prevalence of vertebral fractures, and bone quality in patients with adrenal incidentalomas with and without subclinical hypercortisolism: an Italian multicenter study. J Clin Endocrinol Metab. 2009;94(9):3207-3214.
- Terzolo M, Bovio S, Pia A, Reimondo G, Angeli A. Management of adrenal incidentaloma. Best Pract Res Clin Endocrinol Metab. 2009;23(2):233-243.
- Zeiger MA, Thompson GB, Duh QY. The American Association of Clinical Endocrinologists and American Association of Endocrine Surgeons medical guidelines for the management of adrenal incidentalomas.Endocr Pract. 2009;15(Suppl1):1-20.
- Young WF Jr. Clinical practice. The incidentally discovered adrenal mass. N Engl J Med. 2007;356(6):601-610.
- Toniato A, Merante-Boschin I, Opocher G, et al. Surgical versus conservative management for subclinical Cushing syndrome in adrenal incidentalomas: a prospective randomized study. Ann Surg. 2009;249(3):388-391.
- Emral R, Uysal AR, Asik M, et al. Prevalence of subclinical Cushing's syndrome in 70 patients with adrenal incidentaloma: clinical, biochemical and surgical outcomes. Endocr J. 2003;50(4):399-408.
- Szolar DH, Korobkin M, Reittner P, et al. Adrenocortical carcinomas and adrenal pheochromocytomas: mass and enhancement loss evaluation at delayed contrast-enhanced CT. Radiology. 2005;234(2):479-485.
- Yun M, Kim W, Alnafisi N, et al. 18F-FDG PET in characterizing adrenal lesions detected on CT or MRI. J Nucl Med. 2001;42(12):1795-1799.
- Mazzaglia PJ, Monchik JM. Limited value of adrenal biopsy in the evaluation of adrenal neoplasm: a decade of experience. Arch Surg. 2009;144(5):465-470.