Multiple endocrine neoplasia 2

Overview

What is Multiple endocrine neoplasia type 2?

Multiple endocrine neoplasia Type 2 (MEN2) is a cancer syndrome found in men and women that increases the chance for tumors in the body, most frequently in the hormone producing organs of the endocrine system. Often the first signs of MEN2 are high blood pressure, episodes of sweating, diarrhea, itchy skin bumps, high levels of calcium in the blood (hypercalcemia), neck mass, neck pain, choking, and/or shortness of breath.

The tumors may occur in many different locations and may be benign (non-cancerous) or malignant (cancerous). The most frequently developed tumors in MEN2 occur in the thyroid gland with 98% to 100% of individuals affected by medullary thyroid cancer over the course of their lifetime.

MEN 2 is further divided into subtypes that all have a high risk for medullary thyroid cancer, but have some differences in age at tumor development and medical issues including:

MEN 2A- Accounts for 70%-80% of cases of MEN2. Includes early adulthood risk for onset of medullary thyroid cancer, increased risk for pheochromocytoma (rare tumor of adrenal gland), and parathyroid adenoma (noncancerous tumor in the parathyroid a gland that makes a hormone that helps control the amount of calcium and phosphorus in the blood). MEN2A can further be divided into 4 subtypes.

FMTC (familial medullary thyroid carcinoma)- Accounts for 10%-20% of MEN2 cases. FMTC is often considered a variant of MEN 2A that usually results in later adulthood onset of medullary thyroid cancer and not pheochromocytoma or other tumors.

MEN 2B- Accounts for around 5% of cases of MEN2. MEN2B often results in early childhood onset of medullary thyroid cancer, increased risk for pheochromocytoma (rare tumor of adrenal gland) and nerve cell nodules (mucosal neuromas) on the lips and tongue. Children may also often have distinctive facial features with enlarged lips, ganglioneuromatosis (overgrown nerves) of the intestines, and a tall, thin body type with an abnormally curved spine.

MEN2 can run in families or can occur as the result of a new disease causing gene change in the individual.

References
  • Eng C. Multiple Endocrine Neoplasia Type 2. 1999 Sep 27 [Updated 2019 Aug 15]. In: Adam MP, Ardinger HH, Pagon RA, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2019. Available from: https://www.ncbi.nlm.nih.gov/books/NBK1257/Referenced March 7, 2019 https://www.ncbi.nlm.nih.gov/books/NBK1257
  • Revised American Thyroid Association Guidelines for the Management of Medullary Thyroid Carcinoma Samuel Wells-Sylvia Asa-Henning Dralle-Rossella Elisei-Douglas Evans-Robert Gagel-Nancy Lee-Andreas Machens-Jeffrey Moley-Furio Pacini-Friedhelm Raue-Karin Frank-Raue-Bruce Robinson-M. Rosenthal-Massimo Santoro-Martin Schlumberger-Manisha S. https://www.ncbi.nlm.nih.gov/pubmed/25810047
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More Overview Content

Are there other names for Multiple endocrine neoplasia type II?

How common is Multiple endocrine neoplasia type 2?

What is Multiple endocrine neoplasia type 2 most often called by doctors?

Is MEN2 found more frequently in a particular country?

Are there other names for Multiple endocrine neoplasia type II?

Multiple endocrine neoplasia type II can also be called:

  • multiple endocrine adenomatosis type 2
  • adenomatosis, familial endocrine type 2
  • familial endocrine adenomatosis type 2
  • familial endocrine adenomatosis type II
  • multiple endocrine neoplasms type 2
  • MEN 2
  • MEN type 2
  • MEN II
  • MEN type II

However, it also has other names that vary by type.

Other names for MEN2A include:

  • Pheochromocytoma and amyloid-producing medullary thyroid carcinoma
  • PTC Syndrome (MEN2A)
  • Sipple Syndrome (MEN2A)
  • Familiar Medullary Thyroid Carcinoma
  • FMTC
  • MTC1

MEN2B is also referred to as:

  • MENIIB
  • Multiple endocrine neoplasia type 3
  • Mucosal neuroma syndrome
  • Wagenmann-Froboese syndrome
  • Neuromata, mucosal with endocrine tumors

It should be noted that there is some controversy about whether FMTC is its own sub-type of MEN2 or a variant of MEN2A. In the most strict definition of FMTC, individuals in the family only have FMTC; no one in the family has a pheochromocytoma or hyperparathyroidism. However, others do not agree. Thus, it is important to keep this in mind when reading about MEN2, MEN2A and FMTC.

References
How common is Multiple endocrine neoplasia type 2?

Multiple endocrine neoplasia type 2 (MEN2) occurs in about 1 in 35, 000 people. MEN2 is made up of 3 types, MEN2A is the most common form that accounts for 70-80% of MEN2 cases. FMTC (familial medullary thyroid carcinoma) accounts for 10%-20% of MEN2 cases. 2B accounts for about 5 % of all cases of MEN2.

References
What is Multiple endocrine neoplasia type 2 most often called by doctors?

Multiple endocrine neoplasia type 2 is most often called MEN2 or MEN type 2. However, it has 3 sub types, therefore, it may also be called MEN2A or MEN2B or FMTC (familial medullary thyroid carcinoma).

References
Is MEN2 found more frequently in a particular country?

Multiple endocrine neoplasia type 2 (MEN2) happens to people all over the world. However, there have been reports of families in certain part of the world where MEN2 is more commonly found. These areas include Cyprus, Italy, and Denmark.

References

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