(See also Overview of Adrenal Function Overview of Adrenal Function The adrenal glands, located on the cephalad portion of each kidney (see figure Adrenal glands), consist of a Cortex Medulla The adrenal cortex and adrenal medulla each have separate endocrine... read more .)
The most common nonfunctioning adrenal mass in adults is an adenoma (50%), followed by carcinomas and metastatic tumors. Cysts and lipomas make up most of the remainder. However, the precise proportions depend on the clinical presentation. Masses discovered on incidental screening are usually adenomas. Less commonly, in neonates, spontaneous adrenal hemorrhage may cause large adrenal masses, simulating neuroblastoma Neuroblastoma Neuroblastoma is a cancer arising in the adrenal gland or less often from the extra-adrenal sympathetic chain, including in the retroperitoneum, chest, and neck. Diagnosis is confirmed by biopsy... read more or Wilms tumor Wilms Tumor Wilms tumor is an embryonal cancer of the kidney composed of blastemal, stromal, and epithelial elements. Genetic abnormalities have been implicated in the pathogenesis, but familial inheritance... read more . In adults, bilateral massive adrenal hemorrhage may result from thromboembolic disease or coagulopathy, whether disease- or drug-related.
Benign cysts are observed in older patients and may be due to cystic degeneration or vascular accidents. Lymphomas, bacterial infections, fungal infections (eg, histoplasmosis Histoplasmosis Histoplasmosis is a pulmonary and hematogenous disease caused by Histoplasma capsulatum; it is often chronic and usually follows an asymptomatic primary infection. Symptoms are those of pneumonia... read more ), or parasitic infestations (eg, due to Echinococcus) may also manifest with adrenal masses, sometimes bilateral. Hematogenous spread of Mycobacterium tuberculosis may cause adrenal masses. A nonfunctional adrenal carcinoma causes a diffuse and infiltrating retroperitoneal process. Hemorrhage can occur, causing adrenal hematomas.
Symptoms and Signs of Nonfunctional Adrenal Masses
Most patients are asymptomatic. With any adrenal mass, adrenal insufficiency Overview of Adrenal Function The adrenal glands, located on the cephalad portion of each kidney (see figure Adrenal glands), consist of a Cortex Medulla The adrenal cortex and adrenal medulla each have separate endocrine... read more is rare unless both glands are involved, but hyperfunction occurs more frequently.
The major signs of bilateral massive adrenal hemorrhage are abdominal pain, falling hematocrit, signs of acute adrenal failure, and suprarenal masses on CT or MRI. Tuberculosis of the adrenals may cause calcification and Addison disease Addison Disease Addison disease is an insidious, usually progressive hypofunctioning of the adrenal cortex. It causes various symptoms, including hypotension and hyperpigmentation, and can lead to adrenal crisis... read more . Nonfunctional adrenal carcinoma usually manifests as invasive or metastatic disease.
Diagnosis of Nonfunctional Adrenal Masses
Adrenal hormone measurements
Nonfunctional adrenal masses are usually found incidentally during tests such as CT or MRI conducted for other reasons. Nonfunctionality is established clinically and confirmed by adrenal hormonal measurements. (See also the ACR Appropriateness Criteria on Incidentally Discovered Adrenal Mass and AACE and AAES Medical Guidelines for the Management of Adrenal Incidentalomas.)
Screening adrenal hormonal measurements include dexamethasone suppression testing and serum cortisol (to rule out Cushing syndrome Cushing Syndrome Cushing syndrome is a constellation of clinical abnormalities caused by chronic high blood levels of cortisol or related corticosteroids. Cushing disease is Cushing syndrome that results from... read more ), 24-hour urinary or plasma fractionated metanephrines (to rule out pheochromocytoma Pheochromocytoma A pheochromocytoma is a catecholamine-secreting tumor of chromaffin cells typically located in the adrenals. It causes persistent or paroxysmal hypertension. Diagnosis is by measuring catecholamine... read more ) and plasma aldosterone and renin (to rule out primary aldosteronism Primary Aldosteronism Primary aldosteronism is aldosteronism caused by autonomous production of aldosterone by the adrenal cortex (due to hyperplasia, adenoma, or carcinoma). Symptoms and signs include episodic weakness... read more ).
If metastatic or infectious disease is possible, fine-needle biopsy can be diagnostic but is contraindicated if there is suspicion of adrenal carcinoma (to avoid spread of tumor) or pheochromocytoma (to avoid precipitating acute hypertension).
Treatment of Nonfunctional Adrenal Masses
Sometimes excision, depending on size and/or imaging results
Although new imaging modalities (eg, in-phase and out-of-phase MRI) may be diagnostic, if the tumor is solid, of adrenal origin, and > 4 cm, it should usually be excised unless the imaging characteristics are clearly benign.
Tumors 2 to 4 cm in diameter are a particularly difficult clinical problem. If scanning does not suggest cancer and hormonal function does not seem altered (eg, normal electrolytes and metanephrines, no evidence of Cushing syndrome), it is reasonable to reevaluate periodically with imaging studies, usually for 1 to 2 years. If no progression is seen by then, further follow-up is unnecessary. However, many of these tumors secrete cortisol in quantities too small to cause symptoms, and whether they would eventually cause symptoms and morbidity if untreated is unclear. Most clinicians merely observe patients with these tumors, but clinicians should consider removal of these tumors if there is significant cortisol secretion.
Adrenal adenomas < 2 cm require no special treatment but should be observed for growth or development of secretory function (such as by looking for clinical signs and periodically measuring electrolytes).
Nonfunctional adrenal carcinoma that has metastasized is not amenable to surgery, though mitotane plus corticosteroids may help control symptoms of hypercortisolism.
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