Adrenal Tumors
Adrenal Tumors
What are adrenal tumors?
Each person is usually born with two adrenal glands. The adrenals are paired, goldenrod-yellow colored glands that are situated behind the organs of the gastrointestinal tract next to the spine and just above the kidneys in a space called the retroperitoneum.
The two adrenal glands are responsible for making hormones -- substances that make other cells in the body respond in various ways. These hormones enable the body to respond to stress, regulate blood pressure and the amount of fluid in the body, and are involved in the regulation of electrolytes such as sodium and potassium.
The hormones produced by the adrenal glands include aldosterone, cortisol, epinephrine and norepinephrine. The adrenal glands also make small amounts of the sex hormones testosterone and estrogen. The ovaries and testicles make the majority of sex hormones. When certain cells in the adrenal glands produce too much of any one of the hormones, abnormalities can result which may affect many organ systems of the body. Disturbances in the amount of hormone produced can be the result of all cells in the gland producing too much hormone (hyperplasia) or be the result of a few cells, a nodule or mass within an otherwise normal gland, producing too much hormone.
Adrenal tumors are classified by their growth characteristics:
- Adrenal adenoma - adrenal tumor that is benign
- Adrenal carcinoma - adrenal tumor that is malignant (cancer)
There are several types of adrenal tumors:
This type of tumor is also known as Aldosteronoma/Primary Hyperaldosteronism
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.
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.
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. About 30% of pheochromocytomas and the majority of paragangliomas are arising in patients with a hereditary predisposition syndrome, meaning they carry genetic changes inherited from their parents. Therefore everybody with a diagnosis of pheochromocytoma or paraganglioma should have an evaluation by a genetic counselor as part of a multidisciplinary clinic visit or in the Cancer Genetics Clinic.
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.
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 (hirsutism), 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.
Congenital adrenal hyperplasia (CAH) is a group of genetic disorders affecting the adrenal glands. Although CAH is not a cancer, but an inherited condition, patients are seen by our adrenal specialists in the Multidisciplinary Endocrine Oncology Clinic. CAH patients have a defect in one of the enzymes that are necessary to produce adrenal hormones and most often will have low cortisol levels. They also have often very high blood levels of other steroids that are produced by the adrenal gland, which act like male sex hormones. In women this leads to masculinization either at birth (severe condition) or may lead to male changes and infertility later in life. Patients with CAH need a physician that is familiar with the condition to guide them through therapy and hormone replacement therapy with adrenal hormones.
What is our approach?
As part of the Rogel Cancer Center, our Endocrine Cancer Program brings experts in related fields together to provide coordinated, exceptional patient care to those diagnosed with adrenocortical carcinoma (adrenal cancer). Patients benefit from the experience and expertise of many physicians, not just one.
Our multidisciplinary tumor board meets on a regular basis to discuss every patient who comes through our doors. A room full of specialists sit together to talk through the best treatment options, including clinical trials, for each individual. Patients get the benefit of several expert opinions.
Services include:
- Multidisciplinary team with one of the most active adrenal cancer research programs in the world
- See more than 200 adrenal patients per year
- Recognition as an international center of excellence for treatment of adrenal cancer
- Availability of state-of-the-art genetic counseling
- Advanced diagnostics
- Diagnosis and therapy of benign and malignant adrenal tumors, pheochromocytoma, paraganglioma and neuroendocrine tumors
- Diagnosis and therapy of hormone excess syndromes due to adrenal adenoma, adrenal cancer, pherochromocytoma, paraganglioma or neuroendocrine tumors
- Dedicated radiology and nuclear medicine imaging
- Specialized therapies
- Stereotactic Body Radiation Therapy for adrenal metastasis
- Dedicated adrenal and endocrine surgeons
- Innovative care models
- Remote Second Opinion Program for adrenal cancer
- Pathology Consultation
- Therapy and surveillance of endocrine issues in cancer survivors
Appointment Information
We offer diagnosis and treatment for those with adrenal tumors. If you have been diagnosed, or suspect you have an adrenal tumor, patients, please call 734-647-8902.
Healthcare professionals, please contact our M-LINE service: 800-962-3555. Our Endocrine Oncology Program also offers a consultation with one of our Pathology experts. Please visit the M-Labs Consult page for more information.
Remote Second Opinion
U-M Health's Remote Second Opinion is available to those with adrenal cancer or are suspected to have adrenal cancer. With our Remote Second Opinion, you can obtain a medical consultation regarding your diagnosis and treatment plan by allowing our physicians to review your medical records and remotely interact with you and your local doctor. So, even if you can’t travel to Michigan for an appointment, you can still get an opinion from our medical specialists.
How are adrenal tumors diagnosed?
Diagnosis of Conns Syndrome (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.
Patients with symptoms suspicious for adrenal cancer will undergo tests to determine the cause of these symptoms. The first step is a thorough medical history and physical exam to determine the extent of symptoms and their possible causes.
Evaluation for other adrenal tumors may include the following:
- Blood and urine tests will be done to evaluate levels of adrenal hormones. Remember that adenomas (benign tumors) may produce high levels of adrenal hormones. Therefore, high hormone levels do not always indicate an adrenal cancer. Some blood and urine tests may be done after you are given a steroid such as dexamethasone. The blood and urine tests after the medication will measure your bodies’ response to the steroid, indicating the presence of a hormone secreting tumor or other problem with the adrenal gland.
- Computed Tomography (CT)
The CT scan can show small tumors as well as important blood vessels that the tumor might be growing into or around. A CT scan can look at surrounding organs for spread (metastasis) of the cancer into lymph nodes or other organs in the abdomen.
A CT scan takes longer than a regular x-ray. You will lie still on a table while it is being done. Usually, you will need an IV placed for a dye injection just before the scan. You may also be asked to drink a contrast solution before the exam. This helps outline the intestine so it is not mistaken for tumors. - Magnetic Resonance Imaging (MRI)
The MRI scan uses magnets to make a picture of the inside of the body. MRIs produce very sharp, precise pictures of the area and can be helpful in distinguishing an adenoma from a cancer. - Ultrasound of the abdomen:
An ultrasound can identify a tumor or mass by using sound waves to make a picture of the inside of the abdomen. This is the fastest and cheapest test used; however it is the least accurate. It can be difficult to tell the difference between an adenoma and cancer with an ultrasound. An ultrasound may be used to guide the biopsy procedure as described above under CT scan. - Positron emission tomography (PET)
A PET scan involves the injection of radioactive sugar into a vein followed by a body scan that looks for areas taking up the sugar. Cancer cells take up sugar much faster than normal tissue, so cancerous areas can be located with this test. Research studies have shown the usefulness of PET scanning in identifying adrenal tumors. However, studies are still in progress to look at ways PET scanning can be used to tell the difference between adrenal cancers and benign adenomas and between primary adrenal cancers from metastatic tumors that started in other organs. One of these studies uses a substance called metomidate as the radioactive substance. Some studies suggest it can be helpful in distinguishing primary tumors from metastatic ones. - Angiography
The adrenal gland is situated near a number of important veins and arteries. Some studies may be necessary to evaluate the involvement of these blood vessels, such as whether the tumor is compressing or blocking them. These studies are referred to as selective angiography and adrenal venography.
How are adrenal tumors treated?
Surgery is the treatment of choice if possible. In many cases, patients present after tumor has spread to other organs in the body or the tumor has invaded structures that cannot be removed and the tumor is considered inoperable. If patients are able to undergo surgery, it is extremely important that your surgery is performed by a surgeon experienced in the treatment of adrenal cancer (most surgeons see one adrenal cancer patient in a lifetime), as the covering of the tumor must not be penetrated during surgery and a wide margin of normal tissue around the tumor should be included to ensure complete resection and minimize the chance for local recurrence. At U-M Health, our endocrine surgeons often operate on patients with advanced adrenal cancer, including patients who have been told by other health care teams that their tumor is unresectable. Appropriate preoperative evaluation and planning is of the utmost importance in adrenal tumor patients to assure optimal outcome.
Based on experience and research done at the Rogel Cancer Center, we generally do not recommend removing adrenal tumors (or any mass/nodule suspected of potentially being malignant) using a laparoscopic approach as it often leads to early recurrence in the abdominal cavity due to microscopic tumor spillage. Often, the entire tumor is not resected when the procedure is performed laparoscopically, and the "margins" (edges of the tissue removed) are positive for tumor cells. An open approach (through a larger incision along the rib cage or along the midline of the abdomen) allows the surgeon to remove a rim of normal (benign) tissue around the tumor more easily to help decrease rates of local recurrence and spread within the abdomen and has been found to improve survival in patients with adrenal cancers which have not already spread outside the adrenal gland.
Pheochromocytoma and Paraganglioma Treatment
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.
Locations
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Endocrine Oncology Clinic | Rogel Cancer Center 1500 E Medical Center Dr
Floor B1 Reception A
Ann Arbor, MI 48109-5911Get Directions
Doctors
Richard Joseph Auchus, MD, PhD
Professor
Endocrinology, Internal Medicine
Tobias Else, MD
Associate Professor
Endocrinology, Internal Medicine
Paul Glenn Gauger, MD
Professor
Surgery, Surgical Critical Care
Thomas James Giordano, MD, PhD
Professor
Anatomic Pathology
Gary Douglas Hammer, MD, PhD
Professor
Endocrinology, Internal Medicine
David Thomas Hughes, MD
Clinical Associate Professor
Surgery
Shruti Jolly, MD, MBA
Clinical Professor
Radiation Oncology
Lauren Krumeich, MD, MS, FACS
Assistant Professor
Surgery
Susan Clare Pitt, MD
Associate Professor
Surgery
Hunter J Underwood, MD
Assistant Professor
Surgery
Providers
Elizabeth A Hesseltine, NP
Advanced Practice Nurse
Nurse Practitioner