Multiple Endocrine Neoplasia Type 1

What is multiple endocrine neoplasia type 1?

multiple hormone neoplasia type 1 ( MEN1 ) is a ancestral condition associated with tumors of the hormone ( hormone producing ) glands. MEN1 was originally known as Wermer syndrome. The most common tumors seen in MEN1 involve the parathyroid gland gland, isle cells of the pancreas, and pituitary gland. other hormone tumors seen in MEN1 include adrenal cortical tumors, neuroendocrine tumors ( previously called carcinoid tumors ), and rare pheochromocytoma, deoxyadenosine monophosphate well as tumors in early parts of the digestive tract .
Non-endocrine tumors are besides seen in MEN1. These tumors can include :

  • facial angiofibroma, which is a tumor of rake vessels and fibrous tissue
  • collagenoma, which is a flesh-colored tumor on the hide

  • lipoma, which is a fatso tumor
  • leiomyoma, which is a smooth muscle tumor
  • meningioma, which is a tumor from anxious system tissue ; rare
  • ependymoma, which is a tumor from nervous system weave ; rare

The majority of tumors in people with MEN1 are benign ( noncancerous ). however, approximately 1 out of 3 pancreatic neuroendocrine tumors and mediastinal neuroendocrine tumors are cancerous, meaning the tumor can spread to other parts of the body. These tumors can besides cause problems by producing eminent amounts of hormones. There is a wide-eyed assortment of symptoms that can occur due to this increased hormone production by this type of tumor. These include increased production of :

  • Prolactin, which causes abnormal milk production by the breast, miss of menstruation in women, and lowered testosterone production in men
  • emergence hormone, which causes excessive growth of the jaw and other soft tissues
  • adrenocorticotropic hormone, which causes excessive hydrocortisone production by the adrenal glands
  • gastrin, which causes stomach ulcers
  • glucagon, which causes diabetes mellitus and clamber rash
  • vasoactive intestinal peptide, produced by a pancreatic neuroendocrine tumor, causing intense watery diarrhea
  • parathyroid gland hormone, produced by parathyroid gland tumors, causing eminent lineage calcium ( hypercalcemia ) and kidney stones

What causes MEN1?

MEN1 is a familial condition. This means that the cancer risk and other features of MEN1 can be passed from generation to genesis in a family. The gene associated with MEN1 is besides called MEN1. A mutation ( revision ) in the MEN1 gene gives a person an increased hazard of developing hormone tumors and early symptoms of MEN1. More than 90 % of individuals who inherit the MEN1 mutation will develop 1 or more symptoms of MEN1. A small share of people without MEN1 genetic alterations have been found to have germline mutations ( alterations in the body ’ sulfur egg or sperm cells that become incorporated into the deoxyribonucleic acid of every cell through inheritance ) in a class of proteins called cyclin-dependent kinase inhibitors ( CDKIs ) that regulate cell growth and division. Research is ongoing to learn more about MEN1 .

How is MEN1 inherited?

normally, every cell has 2 copies of each gene : 1 inherited from the mother and 1 from the father. MEN1 follows an autosomal dominant inheritance convention, in which a mutation happens in alone 1 copy of the gene. This means that a rear with a gene mutation may pass along a copy of their normal gene or a transcript of the gene with the mutation. therefore, any child from a parent with a genetic mutant has a 50 % gamble of inheriting that mutant. however, if the parents test damaging for the mutation, the risk to the siblings significantly decreases but their hazard may still be higher than an average risk .
Options exist for people concern in having a child when a prospective parent carries a gene mutant that increases the risk for this ancestral cancer syndrome. For more information, talk with an help replica specialist at a birthrate clinic .

How common is MEN1?

It is estimated that about 1 in 30,000 people has MEN1. About 10 % of people with MEN1 do not have a syndicate history of the condition ; they have a de novo ( newfangled ) mutant in the MEN1 gene .

How is MEN1 diagnosed?

MEN1 is suspected when a person has at least 2 of the most common tumors listed below :

  • Parathyroid tumor
  • pancreatic neuroendocrine tumor
  • pituitary gland tumor

If a person has a family history of MEN1, he or she is suspected of besides having MEN1 if diagnosed with a parathyroid gland, pancreatic, or pituitary tumor. genetic testing for mutations in the MEN1 gene is available for people suspected to have MEN1. A mutant in the MEN1 gene is found in about 80 % to 90 % of families diagnosed with MEN1. approximately 65 % of people with 2 or more tumors associated with MEN1, but no family history, will have a mutation in the MEN1 gene .

What are the estimated cancer risks associated with MEN1?

approximately 1 out of 3 pancreatic neuroendocrine tumors are cancerous. If the cancer has spread beyond where it started, the most coarse site of spread is the liver-colored. A humble share of mediastinal neuroendocrine tumors are cancerous and gap to local ( nearby ) lymph nodes or to the liver, lung, or other locations .

What are the screening options for MEN1?

Current suggested screening for people who are known or suspected to have MEN1 includes :

Genetic testing

  • genic testing is available. It should be considered for children or young adults who are members of a syndicate diagnosed with MEN1 and an identify mutant of the MEN1 gene to determine which children and young adults should have the screening studies described below. In a family with an name mutation of the MEN1 gene, children with a genetic test showing no mutation ( expected to be 50 % of children born to an individual affected with MEN1 ) may not need the screening tests described below .

Diagnostic studies

  • regular blood tests every 1 to 3 years for prolactin, insulin-like growth factor 1 ( IGF-1 ), fasting glucose, insulin, and proinsulin, beginning at age 5 to 10 years.

  • A annual ionize or albumin-corrected calcium level test, beginning at age 8
  • regular rake tests for fasting gastrin and fast and meal stimulated pancreatic polypeptide ( PP ), fasting VIP, and glucagon, beginning at senesce 20 .
  • charismatic rapport image ( MRI ) scan of the mind, every 3 to 5 years, beginning between ages 5 to 10, or at any time the results of the tests for serum prolactin or insulin-like growth factor is abnormal .
  • MRI or computed imaging ( CT ) read of the chest of drawers and abdomen, every 2 to 4 years, beginning at age 20 or when the serum gastrin, PP, or VIP is noted to be abnormal .

Screening guidelines may change over time as newly technologies are developed and more is learned about MEN1. It is important to talk with your doctor about appropriate screen tests .

What are the treatment options for the endocrine tumors?

Most of these tumors are treated with operating room or by taking a medicine that suppresses increase or serve of the tumor. Parathyroid tumors, which are about always benign, should be surgically removed when the albumin-corrected serum calcium level is greater than 12 mg/dl, there is significant bone personnel casualty, or kidney wrong or stones. There are challenging treatment issues related to removing pancreatic neuroendocrine tumors. In addition to its function in normal digestion, the pancreas regulates the level of blood glucose through insulin production. removal of the pancreas will cause diabetes mellitus, a condition that can lead to significant health problems and it will be necessary to take pancreatic enzyme supplements to promote digestion. Doctors must balance the benefits of pancreatic removal in a person with MEN1, such as the prevention of development of cancer spread, against the risks of diabetes mellitus. Patients with a pancreatic neuroendocrine tumor that has spread to the liver may be treated with a somatostatin analogue or a drug that regulates signaling in the pancreatic isle cell, everolimus. other neuroendocrine tumors are typically removed by operation and other treatments may be recommended .
pituitary tumors producing the hormone prolactin are most normally managed with dopamine agonists, which are drugs that imitate the action of dopamine, a naturally occurring substance produced in the mind. Tumors that produce increase hormone or adrenocorticoptropin hormone or non-functioning tumors are most normally treated with surgery. There are 2 hormonal therapies, a somatostatin analogue and a growth hormone antagonist, that have been successfully used to treat besides a lot growth hormone in patients who are not cured by operation. Learn more about pituitary tumor treatment .
Learn more about what to expect when having common tests, procedures, and scans .

Questions to ask the health care team

If you are concerned about your risk of an endocrine gland tumor, speak with your health worry team. It can be helpful to bring person along to your appointments to take notes. Consider asking your health manage team the follow questions :

  • What is my risk of developing a neuroendocrine tumor ?
  • What can I do to reduce my gamble of early types of tumors ?
  • What are my options for screening ?

If you are concerned about your kin history and think you or other syndicate members may have MEN1, consider asking the take after questions :

  • Does my family history addition my gamble of developing cancer ?
  • Does it suggest the need for a cancer risk assessment ?
  • Will you refer me to a genic advocate or early genetics specialist ?
  • Should I consider genetic test ?

Related Resources

The Genetics of Cancer
genic test
What to Expect When You Meet With a Genetic counselor
Collecting Your family Cancer History
Sharing genetic Test Results with Your Family
family Genetic Testing Q & A

More Information 

American Multiple Endocrine Neoplasia Support
hypertext transfer protocol : //amensupport.org/
Association for Multiple Endocrine Neoplasia Disorders (AMEND)
www.amend.org.uk

National Cancer Institute
www.cancer.gov
To find a genetic counselor in your area, ask your doctor or visit this website:       
National Society of Genetic Counselors  
www.nsgc.org

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Category : Health

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