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multiple endocrine neoplasia

   Also found in: Dictionary/thesaurus, Acronyms, Encyclopedia, Wikipedia 0.01 sec.
neoplasia /neo·pla·sia/ (-pla´zhah) the formation of a neoplasm.
cervical intraepithelial neoplasia  (CIN) dysplasia of the cervical epithelium, often premalignant, characterized by various degrees of hyperplasia, abnormal keratinization, and the presence of condylomata.
gestational trophoblastic neoplasia  (GTN) a group of neoplastic disorders that originate in the placenta, including hydatidiform mole, chorioadenoma destruens, and choriocarcinoma.
multiple endocrine neoplasia  (MEN) a group of rare diseases caused by genetic defects that lead to hyperplasia and hyperfunction of two or more components of the endocrine system; type I is characterized by tumors of the pituitary, parathyroid glands, and pancreatic islet cells, with peptic ulcers and sometimes Zollinger-Ellison syndrome; type II is characterized by thyroid medullary carcinoma, pheochromocytoma, and parathyroid hyperplasia; type III is similar to type II but includes neuromas of the oral region, neurofibromas, ganglioneuromas of the gastrointestinal tract, and café-au-lait spots.

Multiple endocrine neoplasia
Abnormal tissue growth on one or more of the endocrine (hormone-secreting) glands.

multiple endocrine neoplasia (MEN),
a hereditary hormonal disorder that occurs in an autosomal-dominant pattern. The endocrine neoplasms may be expressed as hyperplasia, adenoma, or carcinoma and may develop synchronously or metachronously. Some kinds are multiple mucosal neuroma syndrome, Sipple's syndrome, and Werner's syndrome. See also multiple endocrine neoplasia, type I, and multiple endocrine neoplasia, type II.

multiple endocrine neoplasia, type I,
a type of multiple endocrine neoplasia that includes tumors of the pituitary, parathyroid glands, and pancreatic islet cells, often with peptic ulcers and sometimes the Zollinger-Ellison syndrome. See also multiple endocrine neoplasia.

multiple endocrine neoplasia, type II,
a type of multiple endocrine neoplasia characterized by medullary carcinoma of the thyroid, pheochromocytoma, and hyperplasia of the parathyroid glands. See also multiple endocrine neoplasia.

neoplasia [ne″o-pla´zhah]
the formation of a neoplasm.
cervical intraepithelial neoplasia (CIN) dysplasia of the cervical epithelium, often premalignant, characterized by various degrees of hyperplasia, abnormal keratinization, and the presence of condylomata.
multiple endocrine neoplasia (MEN) a group of rare hereditary disorders of autonomous hyperfunction of more than one endocrine gland. In Type I (MEN I), called also Wermer's syndrome, there are tumors of the pituitary, parathyroid gland, and pancreatic islet cells in association with a high incidence of peptic ulcer. Type II (MEN II), called also Sipple's syndrome, is characterized by medullary carcinoma of the thyroid, pheochromocytoma, often bilateral and multiple, and parathyroid hyperplasia. Type III (MEN III), called also mucosal neuroma syndrome, resembles Type II except that parathyroid hyperplasia is rare, the mean survival time is shorter, and there may be neuromas and neurofibromas. All forms are transmitted as autosomal dominant traits.

multiple endocrine neoplasia
See MEN.


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Causes Causes of this pathology remain unknown, although some genetic factors could play an important role, especially in patients who have a family history of multiple endocrine neoplasia type 1 (MEN I) or Wermer syndrome.
Biochemical evaluation should include a 24-hour urine collection for norepinephrine, epinephrine, metanephrines, normetanephrines, dopamine, and VMA, or plasma metanephrines and normetanephrines, which is expensive, but more sensitive, especially in patients who may harbor occult diagnoses of familial syndromes, such as multiple endocrine neoplasia Type 2, Von Hippel Lindau or familial paragangliomas.
Primary parathyromatosis occurs in patients with multiple endocrine neoplasia (MEN), most commonly MEN type 1.
 
 
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