Multiple Endocrine Neoplasia Treatment
At UT MD Anderson, we treat more patients with multiple endocrine neoplasia (MEN) than most other centers. This gives us a deeper understanding of the disease and allows us to tailor treatment to each patient’s unique needs. Our team includes experts in endocrinology, surgery, genetics and radiation oncology who work together to develop personalized care plans.
Treatment for MEN depends on the type (MEN1 or MEN2), which glands are affected and whether the tumors are benign or cancerous. Because MEN is caused by inherited gene mutations, care often includes long-term monitoring and genetic counseling for patients and their families.
Surgery
Surgery is often the main treatment for MEN, especially when tumors cause hormone imbalances or become cancerous. Our surgeons have extensive experience with the complex procedures MEN may require, including:
- Parathyroid surgery for MEN1: Hyperparathyroidism is a condition in which the parathyroid glands produce too much parathyroid hormone, causing high calcium levels in the blood. It is often the first symptom of MEN1. It is usually treated by removing 3 ½ of the four parathyroid glands. In some cases, all four glands are removed, and a small piece is implanted in the forearm to maintain some hormone function. Learn more about parathyroid disease surgery.
- Pancreatic and duodenal tumor removal: Tumors in the pancreas or duodenum that make hormones (such as gastrinomas or insulinomas) may require surgery. These procedures are highly specialized and are performed by surgeons trained in neuroendocrine tumor care. Learn more about pancreas cancer surgery.
- Thyroidectomy for MEN2: People with MEN2 have a high risk of developing medullary thyroid cancer. Surgery to remove the thyroid is often done in childhood or early adulthood to prevent cancer from developing or spreading. Learn more about thyroid cancer surgery.
- Adrenalectomy: In MEN2, adrenal tumors called pheochromocytomas may need to be surgically removed. If both adrenal glands are involved, care is taken to preserve function whenever possible or replace hormones afterward. Learn more about surgery for adrenal gland tumors.
Medicines
Medications may be used to:
- Control hormone production and relieve symptoms
- Manage tumors that cannot be removed with surgery
- Treat certain conditions such as high calcium (from hyperparathyroidism) or hormone-secreting tumors in the pancreas
- Help shrink or stabilize tumors, especially when surgery is not an immediate option
Most hormone-related symptoms in MEN1 can be managed effectively with medication.
Radiation therapy
Radiation therapy uses powerful, focused beams of energy to kill cancer cells. There are several different radiation therapy techniques. Doctors can use these to accurately target a tumor while minimizing damage to healthy tissue. Radiation is not usually the first treatment choice for MEN, but it may be considered in certain cases, especially for:
- Pituitary tumors that cannot be completely removed by surgery or do not respond to medication
- Thyroid cancer if medullary thyroid cancer spreads beyond the thyroid gland
- Bone or other metastatic sites, in rare, advanced cases
Patients receiving radiation therapy typically undergo one treatment per day for a course of several weeks (often four to six weeks, depending on tumor type, location, and size). Sessions usually last about 10‑30 minutes, depending on preparation, imaging and the time needed to carefully deliver the radiation to the tumor.
Side effects of radiation therapy for MEN‑related tumors are usually mild to moderate when modern techniques are used. The severity and type of side effects depend heavily on the location of the tumor. Common side effects may include fatigue, mild skin redness in the treated area, temporary changes in nearby organ function, or hormone imbalances if endocrine tissue is involved.
Learn more about radiation therapy
Ongoing monitoring
Not all MEN-related tumors require immediate treatment. In some cases, especially when tumors are small, slow-growing or not producing hormones, your doctor may recommend the tumor be monitored instead of treated. Treatment begins when and if the tumor advances to a pre-determined point:
Monitoring for these tumors may include:
- Regular imaging and lab tests
- Hormone level monitoring
This approach helps avoid unnecessary treatment while keeping a close eye on your health.
A lifelong approach to care
Since MEN is a lifelong condition, you will need ongoing follow-up care. Your treatment plan may change over time based on:
- New symptoms
- Tumor growth or changes
- Hormone level fluctuations
- Your personal and family medical history
Learn more about multiple endocrine neoplasia:
6 things to know about pituitary adenomas
Pituitary adenomas, also referred to as pituitary neuroendocrine tumors, are almost always benign tumors that arise from hormone-secreting cells in the pituitary gland.
This gland is situated at the base of the brain, just behind the bridge of the nose, inside a bony cave called the sella turcica. The pituitary gland is considered the “master” gland because it controls all the other hormone-producing glands in the body.
But are pituitary adenomas ever cancerous? What are their symptoms? And, how are they typically treated? Keep reading to learn the answers to these and other questions I sometimes hear about pituitary adenomas.
Are pituitary adenomas ever cancerous?
Pituitary adenomas are benign by definition. That means they are not cancerous. And, despite their location, they are not considered a type of brain tumor.
Very rarely, pituitary adenomas can spread to other parts of the body. At that point, though, they are called something else: pituitary carcinomas.
Do pituitary adenomas ever cause symptoms?
Yes. There are two broad categories of pituitary adenomas: functioning and non-functioning.
Functioning tumors
These make too much of certain hormones that travel to other glands and affect how they behave. People’s symptoms vary based on the type of hormone being produced.
- Prolactinoma: Prolactin, also known as “the breast milk hormone,” can suppress the function of the gonads (ovaries in women and testes in men). Too much of it may cause decreased libido (chiefly men) and infertility (both sexes), missed periods (women), milky breast discharge (women, and very rarely men), and impotence and loss of body hair (men).
- Adrenocorticotropic hormone (ACTH): Too much ACTH leads to excessive production of cortisol, a condition called Cushing disease. Too much cortisol can cause weight gain (especially in the center part of the body), excessive fat deposits above the collar bones and upper back, a rounded face, thin skin, easy bruising, stretch marks, ankle swelling, osteoporosis, diabetes, and high blood pressure.
- Growth hormone: Too much of this hormone can lead to a condition called acromegaly, in which the soft tissues in the hands and feet become abnormally large. Other issues include facial and jaw changes, excessive sweating, heart disease, high blood pressure, joint pains/arthritis, sleep apnea, and diabetes.
- Thyroid-stimulating hormone (TSH): Too much of this hormone can cause weight loss, anxiety, trouble sleeping, feeling too hot, and a rapid and sometimes irregular heartbeat.
Non-functioning tumors
These pituitary adenomas don’t secrete any hormones, so they’re considered “silent.” Once a non-functioning pituitary adenoma gets large enough, however, it can start compressing the optic chiasm. This may cause vision changes, especially along the sides of the visual fields, also known as peripheral vision.
Non-functioning tumors may also cause headaches and abnormally low hormone levels.
How are pituitary adenomas usually diagnosed?
Most patients with functioning pituitary adenomas are diagnosed when they start to experience symptoms and physical changes that lead to an assessment by a doctor. Then, imaging finds the tumor.
Patients with non-functioning pituitary adenomas, on the other hand, typically notice peripheral vision loss, headaches, or symptoms of low hormone levels first, which then leads to a brain MRI that reveals the tumor.
Finally, some people only find out they have a pituitary adenoma by accident when they’re getting a brain scan for some other reason. This is called an incidental finding.
How are pituitary adenomas typically treated? Do they have to be removed?
Pituitary adenomas are not life-threatening. Depending on their type, they can be treated with surgery, medication, and sometimes radiation therapy.
Prolactinomas, for example, can usually be treated with medicine alone. Surgery is another good option for people with small prolactinomas who prefer surgery over medication and for those who do not tolerate medication very well.
For all other functioning tumors, surgery is usually considered first, followed by additional treatment to lower hormone levels if surgery is not curative.
For non-functioning tumors, surgery is usually undertaken to improve vision and alleviate headaches. However, small, incidentally discovered non-functioning tumors may be monitored without therapy in patients who display no symptoms.
Finally, radiation therapy is sometimes needed to control tumor growth and/or treat excessive hormone production when other treatments don’t work.
What is the life expectancy of a person with pituitary adenoma?
Pituitary adenomas can lead to a shorter lifespan if the hormonal problems they cause are not well-controlled. However, with proper treatment, life expectancy for patients with pituitary adenomas is the same as anyone else’s.
What’s the most important thing to know about pituitary adenomas?
Pituitary adenomas and carcinomas are unique tumors that often require multidisciplinary expertise to treat them. Therefore, it is vital for every patient diagnosed with a pituitary tumor to consider being treated at a pituitary tumor center of excellence, such as MD Anderson.
A lot of people don’t realize this, simply because our name contains the word “cancer,” but MD Anderson also provides excellent care to patients with benign tumors.
Steven Waguespack, M.D., is an endocrinologist specializing in pituitary tumors, thyroid cancer and multiple endocrine neoplasia.
Request an appointment at MD Anderson online or call 1-877-632-6789.
Multiple endocrine neoplasias are treated in the Endocrine Center and the Children's Cancer Hospital.