Aldosteronoma/Primary Hyperaldosteronism (Conn's Syndrome)
An aldosteronoma is an adrenal tumor which makes excess amounts of aldosterone. Aldosterone normally helps regulate fluid status in the body, sodium and potassium levels, and affects blood pressure. When excess aldosterone is produced by the adrenal glands or an adrenal nodule, patients often present to their physician with new elevation of blood pressure (hypertension) and low potassium (about 40% of patients). University of Michigan's preeminence in the field dates back seven decades to the appointment of Dr. Jerome Conn as director of the University of Michigan Metabolism Research Unit in 1943. Dr. Conn was the first to identify findings associated with excess aldosterone production. This constellation of findings is now known as Conn's syndrome.
Diagnosis of primary hyperaldosteronism is confirmed by having an elevated aldosterone level and decreased renin level with an aldosterone:renin ratio of at least 20:1. Diagnosis is inferred from laboratory work. Imaging is then obtained, if not already performed, and in most cases adrenal vein sampling (an interventional radiology procedure) is performed. Adrenal vein sampling identifies from which adrenal gland excess aldosterone is coming from. In some patients, both adrenal glands are producing too much aldosterone. These patients are treated with medications such as spironolactone, aldactone, or eplerenone as it is not desirable to remove both adrenal glands in most patients.
For patients with excess aldosterone production from one gland, surgery is usually proposed. Most surgeries can be performed by a laparoscopic approach (minimally invasive) through several small incisions. The vast majority of aldosterone producing nodules are benign, but in very rare cases, these nodules can be malignant. Most aldosteronomas are no more than 1-2 centimeters in size. Malignant aldosterone producing adrenocortical carcinomas should be considered when nodule size reaches greater than 3 to 3.5 centimeters, which is lower than the concern cutoff of malignancy for adrenal nodules in general (4 centimeters at University of Michigan, 6 centimeters in some centers).
Cushing’s syndrome may be caused by an adrenal tumor which makes excessive cortisol (a steroid) or by another type of tumor (often lung or a different type of neuroendocrine tumor) that makes adrenocorticotropic hormone (ACTH). Patients often present with new hypertension, diabetes, central obesity (big belly, skinny arms and legs), easy bruising, purplish lines on the abdomen, round "moon" face, flushed face, increased fat near the collar bones, worsening blood sugar control (diabetes), thin skin. Most patients notice a significant difference in their appearance when viewing photographs of themselves that are several years old.
Similarly, Cushing's Disease (as opposed to Syndrome) is caused by a pituitary tumor which causes both adrenal glands to produce excess cortisol. Most patient with Cushing's syndrome have benign (non-cancerous) adrenal nodules. Adrenocortical cancers can (not always) produce excess amounts of hormones, with cortisol being the most common hormone secreted. Imaging characteristics of the nodule/tumor are also usually concerning for malignancy in these cases of cortisol producing adrenal cancers.
Pheochromocytoma and Paraganglioma
An adrenal tumor making excess amounts of adrenaline (epinephrine, norepinephrine, and in very rare cases dopamine). These substances are referred to as catecholamines. Patients with adrenal nodules producing excess amounts of catecholamines often present with new/worsening hypertension, headaches, sweating, palpitations (racing heart), flushing, and panic attacks. Blood pressure elevations can be so severe that they can lead to heart attacks, stroke and sudden death. Patients with pheochromocytomas may have a genetic syndrome. All patients diagnosed with a pheochromocytoma who are seen in the University of Michigan Multidisciplinary Endocrine Oncology clinic are also seen by one of our genetic counselors and may be referred for further genetic testing.
Malignant (cancerous) pheochromocytomas occur in approximately 10% of patients. Those with benign pheochromocytomas are usually offered laparoscopic surgery performed through several small incisions, while those with malignant appearing or very large tumors are offered surgery using an open approach performed through a larger incision. Malignant tumors are diagnosed when there is evidence of tumor invasion into surrounding tissue or organs, involvement of lymph nodes, or evidence of distant metastatic disease.
Paragangliomas are tumors which are very similar to pheochromocytomas. They produce excess norepinephrine and occur outside the adrenal gland. They may occur anywhere along the side of the spine from the neck to the pelvis. Some catecholamine producing tumors are found inside the bladder and may cause symptoms when patients urinate as the bladder contracts.
Prior to surgery, it is extremely important that the blood pressure be well controlled using certain medications called alpha blockers, beta blockers, and on occasion calcium channel blockers. Alpha blockers (such as dibenzyline) should always be started before beta blockers (such as metoprolol, propranolol, or other medications in the same class) in order to prevent cardiac collapse. Medications to control the blood pressure and heart rate are increased until patients achieve blood pressure goals and also if they have symptoms of nasal stuffiness and slight dizziness upon initially standing. If these goals are not met prior to surgery, the blood pressure may become dangerously high during surgery as the tumor is manipulated and lead to heart attacks, strokes, and increased bleeding.
Virilizing and Feminizing Adrenal Tumors
Tumors of this type are rare and some are associated with adrenocortical carcinomas. A virilizing adrenal tumor makes excess androgens (testosterone). Patients often present with increased hair growth (hirsuitism), increased muscle mass, acne and amenorrhea (loss of periods in a female). A feminizing adrenal tumor makes excess estrogen. Patients often present with increased growth of breast tissue (gynecomastia/breast growth in men) and impotence may be experienced by men.
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To see an endocrine surgeon about your adrenal disorder, call our clinic at 734-936-5830.