Multiple endocrine neoplasia type 2B (also known as "MEN2B", "Mucosal neuromata with endocrine tumors", "Multiple endocrine neoplasia type 3", and "Wagenmann–Froboese syndrome") is a genetic disease that causes multiple tumors on the mouth, eyes, and endocrine glands. It is the most severe type of multiple endocrine neoplasia, differentiated by the presence of benign oral and submucosal tumors in addition to endocrine malignancies. It was first described by Wagenmann in 1922, and was first recognized as a syndrome in 1965-1966 by E.D. Williams and D.J. Pollock.
MEN 2B typically manifests before a child is 10 years old. Affected individuals tend to be tall and lanky, with an elongated face and protruding, blubbery lips. Benign tumors (neoplasms) develop in the mouth, eyes, and submucosa of almost all organs in the first decade of life. Medullary thyroid cancer almost always occurs, sometimes in infancy. It is often aggressive. Cancer of the adrenal glands (pheochromocytoma) occurs in 50% of cases.
A variety of eponyms have been proposed for MEN 2B, such as Williams-Pollock syndrome, Gorlin-Vickers syndrome, and Wagenmann-Froboese syndrome. However, none ever gained sufficient traction to merit continued use, and are no longer used in the medical literature.
The prevalence of MEN2B is not well established, but has been derived from other epidemiological considerations as 1 in 600,000 to 1 in 4,000,000. The annual incidence has been estimated at 4 per 100 million per year.
Read more about Multiple Endocrine Neoplasia Type 2b: Causes, Symptoms, Differential Diagnosis, Prognosis and Treatment, Abraham Lincoln Controversy, See Also
Famous quotes containing the words multiple and/or type:
“... the generation of the 20’s was truly secular in that it still knew its theology and its varieties of religious experience. We are post-secular, inventing new faiths, without any sense of organizing truths. The truths we accept are so multiple that honesty becomes little more than a strategy by which you manage your tendencies toward duplicity.”
—Ann Douglas (b. 1942)
“The more characteristic American hero in the earlier day, and the more beloved type at all times, was not the hustler but the whittler.”
—Mark Sullivan (1874–1952)