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.

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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