Multiple Endocrine Neoplasia Syndromes 1 and 2
Multiple endocrine neoplasia syndromes are a rare group of diseases caused by genetic and inherited conditions that affect the endocrine system. MEN disorders are characterized by the development of noncancerous (benign) or cancerous (malignant) tumors, excessive gland growth and/or overproduction of hormones. The two major forms of multiple endocrine neoplasia are called MEN 1 and MEN 2 and categorized by variations in gene mutations.
MEN I
Overview
Multiple endocrine neoplasia type 1 (MEN 1), also known as Wermer’s Syndrome, is an inherited disease caused by a mutation in the MEN 1 gene. The mutation causes glands to grow and divide irregularly which can lead to the development of tumors in the pituitary gland, parathyroid gland and pancreas and the overproduction of hormones.
Signs/Symptoms
The most common signs of MEN 1 are characterized by:
- Kidney stones
- Stomach ulcers
- Headache
- Abdominal pain
- Muscle pain
- Low blood pressure
- Thinning of bones
- Nausea and vomiting
- High blood pressure (hypertension)
- Weakness
- Fatigue
Who’s at risk?
MEN 1 syndrome is a rare condition that occurs in 1 out of every 30,000 people. People who genetically inherit or have a past family history of the MEN 1 gene have the highest risk developing MEN 1 syndrome.
Risk Factors
Factors that increase the risk of developing MEN 1 include a family history of MEN 1 syndrome, Zollinger-Ellison syndrome or tumors in the pituitary glands.
MEN 1 tumors are usually benign but may develop into malignant tumors. Careful monitoring and screening is important for early diagnosis of cancerous tumors and proper treatment.
Testing
Diagnostic testing may include:
- MRI
- Predictive genetic testing
- Endocrine gland screening
- CT scan
- Insulin tests
- Blood sugar tests
- Parathyroid biopsy
Further testing may be needed depending on the situation of each individual and their condition.
Treatment
Treatment options for MEN 1 are dependent on each patient's stage of development. In the early stages, surgery to remove the tumor(s) and therapy to regulate hormones are often suggested. In more progressive stages, chemotherapy, radiation or more specialized hormone therapy may be recommended.
MEN II
Overview
Multiple endocrine neoplasia type 2 (MEN 2) is a commonly inherited mutation in the MEN 2 gene that causes cells to grow and divide too frequently. Uncontrolled growth causes the enlargement and over activity of the parathyroid, adrenal and thyroid glands.
Signs/Symptoms
The most common signs of MEN 2 syndrome are:
- Sweating
- Diarrhea
- Fatigue
- Chronic constipation
- Skin lesions
- High blood pressure/heart rate
- Memory loss
Who’s at Risk?
MEN 2 syndrome is a rare condition that occurs in 1 out of every 20,000 people. People who genetically inherit or have a past family history of the MEN 2 gene have the highest risk developing MEN 2 syndrome.
MEN 2 tumors are usually benign but may develop into malignant tumors. Careful monitoring and screening is important for early diagnosis of cancerous tumors and proper treatment.
Testing
Diagnostic testing may include:
- MRI
- Predictive genetic testing
- Endocrine gland screening
- CT scan
- Insulin test
- Blood sugar test
- Parathyroid biopsy
Further testing may be needed depending on the situation of each individual and their condition.
Treatment
Treatment options for MEN 2 are dependent on each patient's stage of development. In the early stages, surgery to remove the tumor(s) and therapy to regulate hormones is often suggested. In more progressive stages, chemotherapy, radiation or more specialized hormone therapy may be recommended.
Sources
http://www.endocrineweb.com/MEN/MEN1.html
http://www.mayoclinic.org/men1/treatment.html