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Multiple endocrine neoplasia

Multiple endocrine neoplasia (MEN) (or "multiple endocrine adenomas", or "multiple endocrine adenomatosis" -- "MEA") consists of three syndromesfeaturing 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.

MEN syndromes are inherited as autosomal dominantdisorders. Medullary carcinoma of the thyroid may occur as an autosomal dominant in the absence of other features.

Inhaltsverzeichnis

  • 1 MEN type 1
  • 2 MEN type 2
    • 2.1 MEN type 2A
    • 2.2 MEN type 2B
  • 3 References
  • 4 External links

MEN type 1

{{{Name|Multiple endocrine neoplasia}}}
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{{{Caption|}}}
ICD-10 {{ICD10
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ICD-9 {{{ICD9}}}
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|
 ICD9           = 258.0|
 ICDO           = 8360/1 |
 Image          = |
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 OMIM           = 131100 |
 MedlinePlus    = 000398 |
 eMedicineSubj  = med |
 eMedicineTopic = 2404 |
 DiseasesDB     = 7971 |

}} Type 1 is also known as Wermer's syndrome after Dr Paul Wermer, who described it in 1954:

  1. Parathyroidhyperplasia/tumour causing hyperparathyroidism.
  2. Islet celltumours causing hypoglycaemia(insulinoma) and Zollinger-Ellison syndrome(gastrinoma).
  3. Pituitaryadenoma which may cause pituitary hormone excess.

The causative mutation is in the meningene 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 dominantpattern.

MEN type 2

The next two MEN syndrome types have their basis in molecular genetics. Individuals can be tested for this genetic disorder reliably even when asymptomatic patients. The mutation is in the ret oncogene. Most cases of multiple endocrine neoplasia type 2 are inherited in an autosomal dominantpattern.

MEN type 2A

{{{Name|Multiple endocrine neoplasia}}}
[[Image:{{{Image}}}|190px|center|]]
{{{Caption|}}}
ICD-10 {{ICD10
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OMIM }}}
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DiseasesDB }}}
|
 ICD9           = 193|
 ICDO           = |
 Image          = |
 Caption        = |
 OMIM           = 171400 |
 MedlinePlus    = 000399 |
 eMedicineSubj  = med |
 eMedicineTopic = 1520 |
 DiseasesDB     = 7984 |

}} Type 2A is also known as Sipple syndrome (after the American Dr John H. Sipple, who described it in 1961):

  1. Medullary carcinoma of thyroidwhich is associated with increased calcitoninsecretion. A test for elevated calcitonin should be done after pentagastrininjection and/or calcium infusion, to ensure that all affected patients are detected.
  2. Pheochromocytoma
  3. Parathyroidhyperplasia/tumour causing hyperparathyroidism.

MEN type 2B

{{{Name|Multiple endocrine neoplasia}}}
[[Image:{{{Image}}}|190px|center|]]
{{{Caption|}}}
ICD-10 {{ICD10
ICD-O: {{{ICDO}}}
ICD-9 {{{ICD9}}}
OMIM }}}
MedlinePlus }}}
eMedicine }}}
DiseasesDB }}}
|
 ICD9           = |
 ICDO           = |
 Image          = |
 Caption        = |
 OMIM           = 162300 |
 MedlinePlus    = |
 eMedicineSubj  = med |
 eMedicineTopic = 1520 |
 DiseasesDB     = 7991 |

}} This syndrome has no eponym; it was described by Schimke et al in 1968. Originally thought to be a third MEN it is now considered a variant of II, especially after linkage to RET was confirmed.

  1. Pheochromocytoma
  2. Medullary carcinoma of thyroidwhich is associated with increased calcitoninsecretion. 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. Marfanoidhabitus

References

  • 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 Med1968;279:1-7. PMID 4968712
  • Sipple JH. The association of pheochromocytoma with carcinoma of the thyroid gland. Am J Med 1961;31:163-166.

External links

  • OMIM164761(RET gene)de:Multiple endokrine Neoplasie

sv:Multipel endokrin neoplasi




This article is licensed under the GNU Free Documentation License.
It uses material from the http://en.wikipedia.org/wiki/Multiple+endocrine+neoplasia Wikipedia article Multiple endocrine neoplasia.

 
  All text is available under the terms of the GNU Free Documentation License