<unk>  is a genetic disease that causes multiple tumors on the mouth, eyes, and endocrine glands. It is the most severe<unk>  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.<unk>  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<unk> , such as Williams-Pollock syndrome, Gorlin-Vickers syndrome, and Wagenmann-Froboese syndrome. However, none ever gained sufficient traction to merit continued use, and they are no longer used in the medical literature. The prevalence of<unk>  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.
Multiple endocrine neoplasia type 2B
Multiple endocrine neoplasia type 1
Autoimmune polyendocrine syndrome type 1
Autoimmune polyendocrine syndrome type 2
Autoimmune polyendocrine syndrome type 3
McCune–Albright syndrome
Late onset congenital adrenal hyperplasia
Primary pigmented nodular adrenocortical disease