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The adrenal glands are part of the endocrine system, which releases hormones into the blood system. Hormones are important in many body processes, including metabolism, sexual development and puberty, and stress.
There are two adrenal glands, one on the top of each kidney. They are shaped like triangles, and each is about ½ inch high and 3 inches long.
Each gland has two parts. The medulla is the inner part of the adrenal gland. It makes hormones called catecholamines, which include adrenaline and noradrenaline. These “stress hormones” increase alertness, strength and speed in an emergency. They also affect heart rate, blood pressure and sweating.
The outer part of the adrenal gland is called the cortex. It makes hormones that impact blood pressure, metabolism and how the body uses fats, carbohydrates and proteins.
Malignant Adrenal Gland Tumors
Adrenocortical cancer: These tumors originate in the cortex of the adrenal gland. Adrenocortical cancer is rare, affecting only about 300 to 500 people each year in the United States. There are two main types of adrenocortical cancer:
- Functioning tumors are the most common type and account for about 70% of adrenal cancers. These tumors make hormones, such as cortisol, androgens or aldosterone.
- Non-functioning tumors do not produce hormones.
Malignant adrenal pheochromocytoma: This cancer begins in the medulla. It is extremely rare, with only about 800 cases are diagnosed each year in the United States.
Malignant paragangliomas: These tumors may begin inside or outside the adrenal gland.
Benign Adrenal Gland Tumors
Adenomas: A type of non-cancerous tumor.
Cushing’s Syndrome: In patients with Cushing's syndrome, the adrenal gland produces an excess of cortisol, a hormone that plays a part in regulating blood pressure, heart function and the body’s reaction to stress. Cushing’s syndrome is rare. About two to four new cases per 1 million people are diagnosed in this country each year.
Overproduction of cortisol may be caused by:
- A benign tumor on the adrenal gland called an adenoma
- An abnormality of the pituitary gland, usually a tumor (also called Cushing’s disease)
- Long-term use of corticosteroid medications (such as prednisosne)
- Benign or malignant tumors in other areas of the body that produce adrenocorticotropic hormone (ACTH) (ectopic ACTH syndrome)
Hyperaldosteronism: This condition is caused by a small tumor in the adrenal gland that makes too much aldosterone or an enlargement (hyperplasia) of the adrenal glands. A high level of aldosterone plays a part in the body’s salt and potassium balance, and may cause high blood pressure. In fact, it is believed that 10% of people with high blood pressure have hyperaldosteronism.
Adrenal Gland Tumor Risk Factors
Certain inherited disorders can increase your risk of having an adrenal disease. These include:
- Li-Fraumeni syndrome
- Multiple Endocrine Neoplasia type 2 (MEN2)
- Von Hippel-Lindau disease (VHL)
- Neurofibromatosis type 1, also known as von Recklinghausen’s disease
- Paraganglioma syndrome
- Beckwith-Wiedemann syndrome
Not everyone with these syndromes develops an adrenal tumor. However, if you or anyone in your family has one of these syndromes, your doctor may recommend genetic testing. MD Anderson offers the most advanced genetic testing to let you know your risk of developing adrenal gland tumors.
Adrenal gland tumors may or may not cause symptoms, which depend on the type of hormone made by the tumor. If you have an adrenal gland tumor, your symptoms may include:
- High blood pressure (hypertension)
- Women: Excess facial and body hair, deep voice or problems with menstruation
- Men: Breast tenderness or enlargement, lowered sex drive and/or erectile dysfunction
- Excess fat in the upper back between the shoulders or in the neck
- Round, full face, also called “moon” face
- Thin skin that bruises easily and heals slowly
- Purple-red stretch marks on the belly, thighs or breasts
- Muscle weakness or spasms
- Weight gain or loss
- Insomnia or other sleep disorders
- Low potassium levels
- Rapid or irregular heartbeats
- Feelings of anxiety, panic, fear
- Pallor (paleness)
- Dizziness/lightheadedness with standing
- Temporary/intermittent paralysis (rare)
These symptoms do not always mean you have an adrenal gland tumor. However, it is important to discuss any symptoms with your doctor, since they may signal other health problems.
Adrenal gland tumors are diverse and can be challenging to diagnose. It is essential that an endocrinologist familiar with adrenal gland tumor recommends which tests you should have and analyzes your test results.
The experts at MD Anderson have a high level of expertise in diagnosing adrenal gland tumors. They use the latest techniques and technology to give you the most accurate and concise diagnosis possible, including radiographic scanning, adreno-venous sampling and biochemical testing.
Comprehensive genetic testing and counseling are available if your family members have certain inherited disorders.
If you have symptoms that might signal an adrenal gland tumor, your doctor will examine you and ask you questions about your health and your medical history.
One or more of the following tests may be used to find out if you have an adrenal gland tumor or if treatment is working.
Blood tests to evaluate levels of certain hormones, including cortisol, aldosterone, plasma metanephrines and dehydroepiandrosterone (DHEA), or chemicals such as sodium and potassium.
Urine tests, which may include 24-hour urine collection tests.
Imaging tests, which may include:
- CT or CAT (computed axial tomography) scans
- MRI (magnetic resonance imaging) scans
- Nuclear medicine PET (positron emission tomography)
- MIBG (meta-iodobenzylguanidine) scans
There are no known lifestyle changes to lower the risk of developing adrenal gland tumors.
Our Treatment Approach
Because MD Anderson's experts care for more patients with adrenal gland tumor than most other programs, they have a higher level of experience that can have a direct impact on your chance for successful treatment.
Our renowned endocrinologists are among the most skilled and recognized in the world. They work with teams of other specialists to offer you the most advanced treatment with the least impact on the body.
MD Anderson's accomplished surgeons perform a large number of surgeries for adrenal gland tumors each year, using the least invasive and most effective techniques. They are highly skilled in minimally invasive laparoscopic procedures, which may result in shorter hospital stays, less blood loss and shorter recovery times than with standard surgery techniques.
We are at the forefront of discovering new advancements in treatment of adrenal gland tumor. This means we are able to offer clinical trials for some adrenal gland tumors, which may not be available at other centers.
Adrenal Gland Tumor Treatments
If you are diagnosed with adrenal gland tumor, your doctor will discuss the best options to treat it. This depends on several factors, including the type of the disease and your general health. Your treatment for adrenal gland tumor will be customized to your particular needs.
One or more of the following therapies may be recommended to treat the disease or help relieve symptoms.
Surgery to remove one or both adrenal glands.
Systemic therapies, including chemotherapy, molecular targeted therapies and nuclear medicine agents.
Medicines to balance levels of hormones or replace deficient hormones.
Why choose MD Anderson for your adrenal gland tumor care?
MD Anderson’s Endocrine Center has one of the nation’s few programs dedicated to providing personalized care for benign and malignant adrenal gland tumors, a group of complex and diverse disorders.
Our renowned experts diagnose and treat hundreds of patients with adrenal gland tumors each year, giving us a remarkable level of experience and expertise that translates into exemplary adrenal gland treatment. Your personal team of specialists, which may include endocrinologists, surgeons, nuclear medicine physicians, medical oncologists and radiation oncologists, meets regularly and communicates closely. They work together – and with you – to be sure you receive the best care.
Advanced Adrenal Tumor Diagnosis and Research
Accurate diagnosis of adrenal gland tumors is often challenging, and it requires a high degree of skill and experience. MD Anderson’s laboratories provide a range of specialized approaches, including radiographic scanning, adreno-venous sampling and biochemical testing, to diagnose and determine the extent of disease. And, since many adrenal gland tumors are inherited, we offer comprehensive genetic testing and counseling.
Your adrenal tumor treatment at MD Anderson includes the most advanced methods with the least impact on the body, including targeted therapies and nuclear medicine approaches. If surgery is necessary, our surgeons are often able to perform less-invasive procedures.
Because some adrenal gland tumors are rare, clinical trials can be difficult to find. As one of the nation’s premier programs, we offer research studies of innovative treatments that may be your best option for certain adrenal gland tumors, such as adrenal cancer, malignant pheochromocytomas and paragangliomas.
In 2005, violinist Treesa Gold became concerned when she began experiencing weight gain, hair loss and acne. She saw a doctor, who told her, “You’re 24 and healthy. Stop being paranoid.”
Unsatisfied with this response, she saw an endocrinologist a few months later. After reviewing her bloodwork, he ordered a CT scan, which revealed a 13-centimeter adrenal gland tumor. Treesa was diagnosed with adrenocortical carcinoma. A surgeon removed the tumor, along with her left kidney.
But Treesa knew she needed to see someone with extensive experience in treating adrenal gland tumors for the next phase of her treatment. “My doctor said, ‘I will only see one case of this in your lifetime, and you want to go somewhere where they see many of these cases,’” she recalls.
Adrenal gland tumor treatment
In less than a month, Treesa traveled from New Orleans to Houston for her first appointment at MD Anderson.
“My doctor was the first person who talked bluntly to me about my diagnosis,” Treesa says, recalling that her doctor told her that adrenocortical carcinoma has a very high recurrence rate. “But I needed to hear that to prepare for treatment.”
Because adrenal gland tumors don’t typically respond to traditional chemotherapy, our doctors started Treesa on a half-gram of a type of oral chemotherapy called Mitotane, which treats adrenal gland tumors by suppressing adrenal gland hormone production. She was gradually given higher doses.
“My medical team was so incredibly kind and thorough,” she says. “I don’t think I’ve ever been cared for like I was at MD Anderson, from when I parked my car to when I left for the airport.”
Gratitude for every moment
Throughout chemotherapy, Treesa continued playing the violin with the Louisiana Philharmonic Orchestra. “I just wanted to continue doing what I love,” she says. “I never missed a rehearsal or a gig. I could make it to a break and then throw up."
She and her husband, Matt, cherished life like never before. “I felt so grateful for every single moment,” she says. “When I would play a Christmas tune, I was so emotional. I would think, ‘This could be the last time.’ I felt like everything in my life beyond that point of having cancer was just a bonus.”
Treesa continued taking Mitotane for more than a year while her doctors monitored her for signs of recurrence. After that, her doctor decided to try taking her off of Mitotane.
While she was relieved to be rid of the nausea, Treesa was still nervous about a recurrence. So she continued coming to MD Anderson for regular checkups. “When my doctor would talk about the future, I would think, ‘I’m going to make it to see 30!’” she says.
Life after adrenal cancer
Now, 11 years after her initial adrenal gland tumor diagnosis, Treesa is still cancer-free.
In July, she and her husband welcomed a daughter, Kit Alexandria. “I really do think that if I hadn’t gone to MD Anderson, I wouldn’t be here. I wouldn’t have had my daughter if they hadn’t been there, and if they had just chosen regular chemotherapy,” she says. “I always felt very confident that my doctors were going to consider what was best for me and would keep me alive.”
Today, Treesa is also keeping hope alive for other adrenal gland tumor patients that she meets through a Facebook group. “My advice to them is to do what they need to do to survive,” she says. “The main thing I say is: make sure you have good people on your team for treatment. Go somewhere where they know your cancer.”
Request an appointment at MD Anderson online or by calling 1-877-632-6789.