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Encyclopedia > Multiple endocrine neoplasia

Multiple endocrine neoplasia (MEN) (or "multiple endocrine adenomas", or "multiple endocrine adenomatosis" -- "MEA") consists of three syndromes featuring tumors of endocrine glands, each with its own characteristic pattern. The presence of any one tumor type does not automatically have a patient labelled as MEN, but a search of the other at-risk areas is usually undertaken, especially when there are suggestive clinical signs. Neoplasia (literally: new growth) is abnormal, disorganized growth in a tissue or organ, usually forming a distinct mass. ... Adenoma refers to a collection of growths (-oma) of glandular origin. ... Adenoma refers to a collection of growths (-oma) of glandular origin. ... In medicine, the term syndrome is the association of several clinically recognizable features, signs, symptoms, phenomena or characteristics which often occur together, so that the presence of one feature alerts the physician to the presence of the others. ... An endocrine gland is one of a set of internal organs involved in the secretion of hormones into the blood. ...


MEN syndromes are inherited as autosomal dominant disorders. Medullary carcinoma of the thyroid may occur as an autosomal dominant in the absence of other features. An autosomal dominant gene is one that occurs on an autosomal (non-sex determining) chromosome. ...

Contents

MEN type 1

Wermer's syndrome
Classifications and external resources
ICD-10 D44.8
ICD-9 258.0
ICD-O: 8360/1
OMIM 131100
DiseasesDB 7971
MedlinePlus 000398
eMedicine med/2404 

Type 1 is also known as Wermer's syndrome after Dr Paul Wermer, who described it in 1954: Multiple endocrine neoplasia type 1 is part of a group of disorders that affect the endocrine system. ... The International Statistical Classification of Diseases and Related Health Problems (commonly known by the abbreviation ICD) is a detailed description of known diseases and injuries. ... The following codes are used with International Statistical Classification of Diseases and Related Health Problems. ... // C00-D48 - Neoplasms (C00-C14) Malignant neoplasms, lip, oral cavity and pharynx (C00) Malignant neoplasm of lip (C01) Malignant neoplasm of base of tongue (C02) Malignant neoplasm of other and unspecified parts of tongue (C03) Malignant neoplasm of gum (C04) Malignant neoplasm of floor of mouth (C05) Malignant neoplasm of... The International Statistical Classification of Diseases and Related Health Problems (commonly known by the abbreviation ICD) is a detailed description of known diseases and injuries. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... The International Classification of Diseases for Oncology (ICD-O) is a domain specific extension of the International Statistical Classification of Diseases and Related Health Problems for tumor diseases. ... The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ... The Diseases Database is a free website that provides information about the relationships between medical conditions, symptoms, and medications. ... MedlinePlus (medlineplus. ... eMedicine is an online clinical medical knowledge base that was founded in 1996. ...

  1. Parathyroid hyperplasia/tumour causing hyperparathyroidism.
  2. Pancreatic islet cell tumours causing hypoglycaemia (insulinoma) and Zollinger-Ellison syndrome (gastrinoma).
  3. Pituitary adenoma which may cause pituitary hormone excess.

The causative mutation is in the menin gene which encodes a nuclear protein that is believed to act as a tumor suppressor. Most cases of multiple endocrine neoplasia type 1 are inherited in an autosomal dominant pattern. Categories: Anatomy stubs | Endocrine system ... Hyperparathyroidism is overactivity of the parathyroid glands resulting in excess production of parathyroid hormone (PTH). ... A porcine islet of Langerhans. ... Hypoglycemia is a medical term referring to a pathologic state produced and usually defined by a lower than normal amount of sugar (glucose) in the blood. ... An insulinoma is a tumour of the pancreas derived from the beta cells which while retaining the ability to synthesize and secrete insulin is autonomous of the normal feedback mechanisms. ... Zollinger-Ellison syndrome is a disorder where increased levels of the hormone gastrin are produced, causing the stomach to produce excess hydrochloric acid. ... In humans, gastrin is a hormone that stimulates secretion of gastric acid by the stomach. ... Located at the base of the skull, the pituitary gland is protected by a bony structure called the sella turcica. ... An autosomal dominant gene is one that occurs on an autosomal (non-sex determining) chromosome. ...


MEN type 2

MEN syndrome types 2 and 3 have their basis in molecular genetics. Individuals can be tested for this genetic disorder reliably even when asymptomatic. The mutation is in the RET oncogene. Most cases of multiple endocrine neoplasia types 2 and 3 are inherited in an autosomal dominant pattern. Resolution enhancement technology (RET) is a form of image processing technology used to manipulate dot characteristics popular among laser printer and inkjet printer manufacturers. ... An oncogene is a modified gene that increases the malignancy of a tumor cell. ... An autosomal dominant gene is one that occurs on an autosomal (non-sex determining) chromosome. ...

Sipple syndrome
Classifications and external resources
ICD-10 D44.8
ICD-9 193
OMIM 171400
DiseasesDB 7984
MedlinePlus 000399
eMedicine med/1520 

Type 2 is also known as Sipple syndrome (after the American Dr John H. Sipple, who described it in 1961) and used to be called type 2A: Multiple endocrine neoplasia type 2 is part of a group of disorders that affect the endocrine system. ... The International Statistical Classification of Diseases and Related Health Problems (commonly known by the abbreviation ICD) is a detailed description of known diseases and injuries. ... The following codes are used with International Statistical Classification of Diseases and Related Health Problems. ... // C00-D48 - Neoplasms (C00-C14) Malignant neoplasms, lip, oral cavity and pharynx (C00) Malignant neoplasm of lip (C01) Malignant neoplasm of base of tongue (C02) Malignant neoplasm of other and unspecified parts of tongue (C03) Malignant neoplasm of gum (C04) Malignant neoplasm of floor of mouth (C05) Malignant neoplasm of... The International Statistical Classification of Diseases and Related Health Problems (commonly known by the abbreviation ICD) is a detailed description of known diseases and injuries. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ... The Diseases Database is a free website that provides information about the relationships between medical conditions, symptoms, and medications. ... MedlinePlus (medlineplus. ... eMedicine is an online clinical medical knowledge base that was founded in 1996. ...

  1. Medullary carcinoma of the thyroid which is associated with increased calcitonin secretion. A test for elevated calcitonin should be done after pentagastrin injection and/or calcium infusion, to ensure that all affected patients are detected.
  2. Pheochromocytoma
  3. Parathyroid hyperplasia/tumour causing hyperparathyroidism.

Calcitonin is a a 32 amino acid polypeptide hormone that is produced in humans primarily by the C cells of the thyroid, and in many other animals in the ultimobranchial body. ... Pentagastrin is a synthetic polypeptide that has effects like gastrin when given parenterally. ... A pheochromocytoma (also phaeochromocytoma, English spelling) is a tumor of the medulla of the adrenal glands originating in the chromaffin cells, which secretes excessive amounts of catecholamines, usually epinephrine and norepinephrine. ... Categories: Anatomy stubs | Endocrine system ... Hyperparathyroidism is overactivity of the parathyroid glands resulting in excess production of parathyroid hormone (PTH). ...

MEN type 3

Main article: Multiple endocrine neoplasia type 3 (or 2B)
MEN type 2B
Classifications and external resources
ICD-10 D44.8
OMIM 162300
DiseasesDB 7991
eMedicine med/1520 

This syndrome has no eponym; it was described by Schimke et al in 1968. Originally thought to be a third MEN, then considered a variant of II (especially after linkage to RET was confirmed), it is now considered its own syndrome. The International Statistical Classification of Diseases and Related Health Problems (commonly known by the abbreviation ICD) is a detailed description of known diseases and injuries. ... The following codes are used with International Statistical Classification of Diseases and Related Health Problems. ... // C00-D48 - Neoplasms (C00-C14) Malignant neoplasms, lip, oral cavity and pharynx (C00) Malignant neoplasm of lip (C01) Malignant neoplasm of base of tongue (C02) Malignant neoplasm of other and unspecified parts of tongue (C03) Malignant neoplasm of gum (C04) Malignant neoplasm of floor of mouth (C05) Malignant neoplasm of... The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ... The Diseases Database is a free website that provides information about the relationships between medical conditions, symptoms, and medications. ... eMedicine is an online clinical medical knowledge base that was founded in 1996. ...

  1. Pheochromocytoma
  2. Medullary carcinoma of thyroid which is associated with increased calcitonin secretion. A test for elevated calcitonin should be done after pentagastrin injection and/or calcium infusion, to ensure that all affected patients are detected.
  3. Mucosal neuromas which are usually situated in the gastrointestinal tract.
  4. Marfanoid habitus

A pheochromocytoma (also phaeochromocytoma, English spelling) is a tumor of the medulla of the adrenal glands originating in the chromaffin cells, which secretes excessive amounts of catecholamines, usually epinephrine and norepinephrine. ... Calcitonin is a a 32 amino acid polypeptide hormone that is produced in humans primarily by the C cells of the thyroid, and in many other animals in the ultimobranchial body. ... The gastrointestinal tract or digestive tract, also referred to as the GI tract or the alimentary canal, (nourishment canal) or the gut, is the system of organs within multicellular animals which takes in food, digests it to extract energy and nutrients, and expels the remaining waste. ... This article does not cite its references or sources. ...

References

  • Carney JA. Familial multiple endocrine neoplasia: the first 100 years. Am J Surg Pathol. 2005 Feb;29(2):254-74. PMID 15644784
  • Wermer P. Genetic aspect of adenomatosis of endocrine glands. Am J Med 1954;16:363-371. PMID 13138607.
  • Schimke RN, Hartmann WH, Prout TE, Rimoin DL. Syndrome of bilateral pheochromocytoma, medullary thyroid carcinoma and multiple neuromas. A possible regulatory defect in the differentiation of chromaffin tissue. N Engl J Med 1968;279:1-7. PMID 4968712
  • Sipple JH. The association of pheochromocytoma with carcinoma of the thyroid gland. Am J Med 1961;31:163-166.

The New England Journal of Medicine (NEJM) is a peer-reviewed medical journal published by the Massachusetts Medical Society. ...

External links


  Results from FactBites:
 
Multiple Endocrine Neoplasia Type 2 (4908 words)
The endocrine disorders observed in MEN 2 are medullary thyroid carcinoma and/or its precursor, C-cell hyperplasia; pheochromocytoma; and parathyroid adenoma or hyperplasia.
MEN 1 is caused by mutations in the
MEN 1 is characterized by a triad of pituitary adenomas, pancreatic islet cell tumors, and parathyroid disease consisting of hyperplasia or adenoma.
Multiple endocrine neoplasia type 2 - WrongDiagnosis.com (1046 words)
A type of multiple endocrine neoplasia characterized by a virtually 100% incidence of medullary thyroid carcinoma, a 50% incidence of pheochromocytoma, and a lesser incidence of parathyroid adenomas associated with hyperparathyroidism.
Multiple endocrine neoplasia type 2 is part of a group of disorders that affect the endocrine system.
Most cases of multiple endocrine neoplasia type 2 are inherited in an autosomal dominant pattern, which means affected people may have affected siblings and relatives in successive generations (such as parents and children).
  More results at FactBites »

 
 

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