Description

Multiple endocrine Neoplasia type 1 (MEN 1) is a rare condition. It occurs mainly in tumors of the glands that produce and release hormones. These are the so-called endocrine glands. The condition can also cause tumors in the small intestine and the stomach. Another name for the MEN 1 syndrome is the of Wermer.

The endocrine gland tumors which are formed due to MEN 1 are usually not cancer. Most often, the growth of tumors in the parathyroid glands, the pancreas, and the pituitary gland. Some glands affected MALES 1 may also disclose too many hormones. It can lead to other health problems.

The extra hormones in MEN 1 can cause many symptoms. These symptoms may include tiredness, bone pain, broken bones, kidney stones, and ulcers in the stomach or intestines.

MEN 1 can't be cured. But regular testing can detect health problems, and health care professionals can provide the necessary treatment.

MEN 1 is an inherited disease. That means that people who have a genetic change that causes MEN to 1 can pass it on to their children.

Symptoms

Symptoms of multiple endocrine neoplasia type 1 (MEN 1) may include the following:

  • Fatigue.
  • Pain in the bones or fractures of bones.
  • Stones in the kidney.
  • Ulcers in the stomach or intestines.
  • Stomach pain.
  • The muscle weakness.
  • Depression.
  • The acid reflux.
  • Frequent diarrhea.

The symptoms are caused by the release of too many hormones in the body.

Causes

Multiple endocrine Neoplasia type 1 (MEN 1) is caused by a change in the MEN1 gene. That gene controls the way in which the body produces a protein called menin. Menin helps to keep the cells in the body grow and divide quickly.

Many changes in the MEN1 gene can cause MEN 1 condition to develop. People who have one of the genetic changes can pass it on to their children. Many people with a change in the MEN1 gene inherited from the parents. But some people are the first in their family to have a new MEN1 gene change that does not come from a father.

Risk factors

The risk factors for multiple endocrine neoplasia type 1 (MEN 1) include the following:

  • Children with a parent who has a genetic change in the MEN1 gene are at risk of MEN 1 condition. That is because these children have a 50% chance of having the same genetic change that causes MEN to 1.
  • The parents and brothers of the people that have a change in the MEN1 gene are also at risk. That's because they can have the same genetic change, even if they have not had any of the symptoms in MEN 1.

Diagnosis

To find out if you have multiple endocrine neoplasia type 1 (MEN 1), your health care professional will start by doing a physical exam. You also respond to the questions about your medical history and family history. You can have a blood test and imaging tests, including the following:

  • Magnetic resonance imaging (MRI). An mri uses a magnetic field and radio waves to produce images of the organs and tissues in the body.
  • Computed tomography (CT scan). A ct scan combines a series of X-ray images taken from different angles. Then, a computer that makes detailed pictures of areas inside the body.
  • Positron emission tomography (PET). A PET scan uses a substance that releases low levels of radiation to help make the images of the changes that take place in the interior of the body.
  • Nuclear medicine scans. These scans use liquid substances that emit low levels of radiation to help find tumors.
  • Endoscopic ultrasound of the pancreas, and other explorations. An endoscopic ultrasound uses sound waves to create images of the digestive tract and other organs and surrounding tissues.

Genetic testing can help you find out if someone has a genetic change that causes MEN to 1. If so, that the children of the person is at risk of having the same genetic change and the obtaining of MEN 1. Parents and siblings are also at risk of having the genetic change that causes MEN to 1.

If you are not related to the genetic changes that are found in members of the family, then the family members do not need more testing. But a genetic test can find all of the genetic changes that can cause MEN 1. If genetic testing does not confirm the MEN 1, but it is likely that a person has, the more evidence is needed. That person, as well as members of the family, you still need a professional follow-up check-ups with blood tests and imaging tests.

Treatment

With MEN 1, the tumors can grow in the parathyroid glands, the pancreas, and the pituitary gland. Which can lead to various conditions, all of which can be treated. These conditions and the treatments may include:

  • Pituitary tumors. These types of tumors may be treated with surgery or medications. Rarely, radiation therapy is used.
  • The hyperparathyroidism. The surgery to remove the greater part of the parathyroid glands is the typical treatment for too much parathyroid hormone.
  • Neuroendocrine tumors. These are tumors that are formed in specialized cells called neuroendocrine cells. With MEN 1, which is in the pancreas or small intestine. The treatment depends on the type and size of the tumor.
  • Hypoglycemic syndrome. This condition occurs when tumors in the pancreas called insulinomas do too much insulin-the hormone. Too much insulin causes low blood sugar in the blood that can be life-threatening. The treatment often requires surgery. Part of the pancreas may need to be removed.
  • The Zollinger-Ellison syndrome (ZES). ZES may result in tumors called gastrinomas that produce too much stomach acid. This leads to ulcers and diarrhea. Health professionals can prescribe medication or try to remove the tumor with surgery.
  • Other pancreatic neuroendocrine tumors. These tumors sometimes produce other hormones that can cause health problems. The treatment of these types of tumors may involve medication or surgery. Another treatment is called ablation can be done to destroy irregular tissue that may be present.
  • Metastatic neuroendocrine tumors.Tumors that spread are called metastatic tumors. Sometimes with MEN 1, tumor spread to the lymph nodes or the liver. They can be treated with surgery. The surgical options include surgery of the liver or of the different types of ablation. Radiofrequency ablation uses high-frequency energy that passes through a needle. The energy causes the surrounding tissue to heat up, killing the nearby cells. Cryoablation involves the freezing of the tumors. And chemoembolization involves the injection of strong chemotherapy drugs directly into the liver. When surgery is not an option, health care professionals may use other forms of chemotherapy or hormone-based treatments.
  • Adrenal tumors. The majority of these tumors can be seen with tests over time and not treated. But if the tumors produce hormones or are large and thought to be cancerous, health professionals recommend removing them. Often, the tumors can be removed with surgery, which involves small incisions. This is known as minimally invasive surgery.
  • Carcinoid tumors. These slow-growing tumors in people with MEN-1 can form in the lungs, thymus gland and the gastrointestinal tract. Surgeons remove these tumors when they haven't spread to other areas. Health professionals can use chemotherapy, radiation therapy or hormone-based therapy in advanced carcinoid tumors.

Metastatic neuroendocrine tumors. Tumors that spread are called metastatic tumors. Sometimes with MEN 1, tumor spread to the lymph nodes or the liver. They can be treated with surgery. The surgical options include surgery of the liver or of the different types of ablation.

Radiofrequency ablation uses high-frequency energy that passes through a needle. The energy causes the surrounding tissue to heat up, killing the nearby cells. Cryoablation involves the freezing of the tumors. And chemoembolization involves the injection of strong chemotherapy drugs directly into the liver. When surgery is not an option, health care professionals may use other forms of chemotherapy or hormone-based treatments.

Preparing for your appointment

You can start by seeing your primary healthcare professional. Then, you may be referred to a doctor called an endocrinologist who treats conditions related to hormones. You may also be referred to a genetic counselor.

Here's some information to help you prepare for your appointment.

What you can do

When you make the appointment, ask if there is something that you need to do ahead of time. For example, you may be asked not to eat or drink anything except water for an amount of time before a test. This is called fasting. You can also make a list of:

  • Their symptoms. Include any that do not seem to be related to the reason for your appointment.
  • Key of the personal information. Include the main tensions, recent life changes and family medical history.
  • All medications, vitamins or supplements that you are taking, including the dosage.
  • Questions to ask your health care professional.

Have a family member or friend if you can. This person can help you remember the information they give you.

For MEN 1, some basic questions to ask your health care professional include:

  • What is likely causing my symptoms?
  • Other that the most likely cause, what are other possible causes of the symptoms?
  • What tests do I need?
  • Is my condition likely to be short-term or long-term?
  • What is the best course of action?
  • What are the alternatives to the main treatment you are suggesting?
  • I have other health conditions. How can I best manage them together?
  • There are restrictions that must be followed?
  • You should see a specialist?
  • Are there brochures or other printed material I can have? What sites do you recommend?

Feel free to ask any other questions that you think.

What to expect from your doctor

Your healthcare provider will likely ask questions such as:

  • Does anyone in your family has MEN 1?
  • Do you or a family member has been tested by changes in the MEN1 gene?
  • If you have symptoms, when does it begin?
  • Your symptoms are ongoing or make it happen once in a while?
  • How severe are the symptoms?
  • What, if anything, seems to improve your symptoms?
  • What, if anything, appears to worsen your symptoms?

What you can do in the meantime

If you have symptoms, try not to do something that seems to get worse.

Symptoms and treatment of Multiple endocrine neoplasia type 1 (MEN 1)