Syn .: Multiple endocrine neoplasia – MEN.
Def.: One of the two forms of endocrine glands, characterized by the presence of multiple endocrine neoplasia (MEN) – adenomas and carcinomas, some of which show hormone-forming activity. MEN are inherited autosomal dominantly.
They can come in two forms:
— MEN 1 (Wermer syndrome) with a defect on chromosome 11q13. There are neoplasms of the parathyroid glands, pituitary adenomas and pancreatic islet cell neoplasms. The most common clinical symptoms include Cushing’s syndrome, gastrointestinal disorders, diabetes mellitus, acromegaly, and multiple facial angiofibromas. There is also an increase in serum calcium.
— MEN 2 with mutations of the oncogene ret on chromosome 10q11. There are two groups within MEN 2:
– ›MEN 2 A (Sipple’s syndrome): parathyroid adenoma, medullary carcinoma of the thyroid gland and phaeochromocytoma;
– ›MEN 2 B (multiple mucosal neuromas): medullary thyroid gland cancer, pheochromocytoma, mucosal neuromas.
Lit .: [1] Guimaraes J.: Multiple endocrine neoplasia. Acta Med Port 2007, 20(1); 65-72. [2] Lakhani V.T., You Y.N., Wells S.A.: Th e multiple endocrine neoplasia syndromes. Ann Rev Med 2007, 58; 253-65. [3] Larsson C., Nordenskjöld M.: Multiple endocrine neoplasia. Cancer Surv 1990, 9(4); 703-23.
Source: A. Kaszuba, Z. Adamski: “Lexicon of dermatology”; XNUMXst edition, Czelej Publishing House