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| __NOTOC__
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| {{CMG}}; {{AE}} {{Ammu}}
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| {{SK}} MEN type 4; MEA type 4; Multiple endocrine neoplasms type 4; Multiple endocrine adenomatosis type 4
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| == Overview ==
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| Multiple endocrine neoplasia type 4 is a type of [[tumor]] that arises from [[endocrine]] cells of [[parathyroid]] gland and [[pituitary]] gland. Multiple endocrine neoplasia type 4 is part of the broad group of [[disease]]s called [[multiple endocrine neoplasia]]. Heterozygous [[mutation]] of ''Cdkn1b/CDKN1B'' [[gene]] on [[chromosome]] 12p13 is suspected to be the cause of multiple endocrine neoplasia type 4. Multiple endocrine neoplasia type 4 must be differentiated from other hereditary tumors such as [[medullary thyroid carcinoma]], C-cell hyperplasia, [[von Hippel Lindau syndrome]], familial hyperparathyroidism, [[acromegaly]], [[multiple endocrine neoplasia type 1]] (MEN 1), [[multiple endocrine neoplasia type 2a]], and [[multiple endocrine neoplasia type 2b]]. The most potent risk factor in the development of multiple endocrine neoplasia type 4 is family history. There is insufficient evidence to recommend routine screening for multiple endocrine neoplasia type 4. The most common symptoms of multiple endocrine neoplasia type 4 include [[lethargy]], [[constipation]], and [[headache]]. Transsphenoidal surgery is used for treating [[pituitary adenoma]] of multiple endocrine neoplasia type 4.
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| ==Historical Perspective==
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| * Historical background of multiple endocrine neoplasia type 4 is given in the table below.
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| <br>
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| {| style="border: 0px; font-size: 90%; margin: 3px;" align=center
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| |+'''''MEN IV Historical Background'''''
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| ! style="background: #4479BA; width: 120px;" | {{fontcolor|#FFF|Years}}
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| ! style="background: #4479BA; width: 550px;" | {{fontcolor|#FFF|Scientist}}
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| ! style="background: #4479BA; width: 550px;" | {{fontcolor|#FFF|Contribution}}
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| ! style="background: #DCDCDC;" |2006
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| ! style="background: #F5F5F5;" |Pellegata
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| | style="padding: 5px 5px; background: #F5F5F5;" |Reported a case study of a patient with [[multiple endocrine neoplasia type 1]] like features such as [[acromegaly]], [[pituitary tumor]], and primary [[hyperparathyroidism]]
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| ! style="background: #DCDCDC;" |2007
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| ! style="background: #F5F5F5;" |Georgitsi
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| | style="padding: 5px 5px; background: #F5F5F5;" |Reported a case study of a female with small cell neuro endocrine tumor, [[pituitary adenoma]], and [[hyperparathyroidism]]
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| ! style="background: #DCDCDC;" |2010
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| ! style="background: #F5F5F5;" |Molatore
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| | style="padding: 5px 5px; background: #F5F5F5;" |Reported a case study of a female with bronchial [[carcinoid]], [[pituitary adenoma]], [[parathyroid adenoma]], and papillary thyroid carcinoma
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| ! style="background: #DCDCDC;" |2012
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| ! style="background: #F5F5F5;" |Malanga
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| | style="padding: 5px 5px; background: #F5F5F5;" |Reported a case study of a female with gastric [[carcinoid tumor]] and [[hyperparathyroidism]]
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| ! style="background: #DCDCDC;" |2014
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| ! style="background: #F5F5F5;" |Toneli
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| | style="padding: 5px 5px; background: #F5F5F5;" |Reported a case study of a female with [[hyperparathyroidism]] and gastrointestinal [[neuroendocrine tumor]]
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| |}
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| == Pathophysiology==
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| Multiple endocrine neoplasia type 4 is a rare genetic disease which is the latest addition to the family of multiple endocrine neoplasia. It is characterized by [[parathyroid adenoma]] and [[pituitary adenoma]].<ref name="pmid23933118">{{cite journal| author=Thakker RV| title=Multiple endocrine neoplasia type 1 (MEN1) and type 4 (MEN4). | journal=Mol Cell Endocrinol | year= 2014 | volume= 386 | issue= 1-2 | pages= 2-15 | pmid=23933118 | doi=10.1016/j.mce.2013.08.002 | pmc=PMC4082531 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23933118 }} </ref>
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| === Genetics ===
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| * Multiple endocrine neoplasia type 4 is an [[autosomal dominant]] disorder caused by genetic [[mutation]] of ''Cdkn1b/CDKN1B'' gene.
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| * The chromosome involved in the development of the disease is 12p13.
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| * A tumor supressor protein called p27Kip1 is encoded by ''Cdkn1b/CDKN1B'' gene germline mutation.
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| * The [[protein]] is derived from a cell cycle inhibitor [[p27 (gene)|''p27'']].
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| * The mechanism of action of the [[gene]] is to inhibit [[Cyclin-dependent kinase|cyclin/cyclin-dependent kinase]] complexes.
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| * Inhibition of [[Cyclin-dependent kinase|cyclin/cyclin-dependent kinase]] complexes causes cessation of cell cycle progression.<ref name="LeePellegata2013">{{cite journal|last1=Lee|first1=Misu|last2=Pellegata|first2=Natalia S.|title=Multiple Endocrine Neoplasia Type 4|volume=41|year=2013|pages=63–78|issn=0301-3073|doi=10.1159/000345670}}</ref>
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| === Associated Conditions===
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| * Gastric [[carcinoid syndrome]]
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| * [[Uterine fibroids]]
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| * [[Adrenal tumor|Adrenal mass]]
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| * [[Zollinger-Ellison syndrome]]
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| * [[Gastrinoma]]
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| * [[Acromegaly]]
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| * [[Cushing's syndrome]]
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| * [[Neuroendocrine tumor]]s of [[cervix]]
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| ==Causes==
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| * Heterozygous mutation of ''Cdkn1b/CDKN1B'' gene on chromosome 12p13 is suspected to be the cause of multiple endocrine neoplasia type 4.<ref name="pmid23652671">{{cite journal| author=Lee M, Pellegata NS| title=Multiple endocrine neoplasia type 4. | journal=Front Horm Res | year= 2013 | volume= 41 | issue= | pages= 63-78 | pmid=23652671 | doi=10.1159/000345670 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23652671 }} </ref>
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| ==Differentiating Multiple endocrine neoplasia type 4 from other Diseases==
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| {| style="border: 0px; font-size: 90%; margin: 3px; width: 600px" align=center
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| |valign=top|
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| ! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Disease}}
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| ! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|Definition}}
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| | style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |[[Multiple endocrine neoplasia type 1]] ([[MEN 1]])
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| | style="padding: 5px 5px; background: #F5F5F5;" |An [[autosomal dominant]] genetic disorder causing [[tumor]]s of [[parathyroid]], [[pancreas]], and [[pituitary gland]].
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| | style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |[[Multiple endocrine neoplasia type 2]]
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| | style="padding: 5px 5px; background: #F5F5F5;" |An [[autosomal dominant]] disorder characterized by [[medullary thyroid carcinoma]] (MTC), [[pheochromocytoma]], and primary [[hyperparathyroidism]].
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| | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |[[von Hippel-Lindau syndrome]]
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| | style="padding: 5px 5px; background: #F5F5F5;" |An [[autosomal dominant]] genetic disorder causing abnormal growth of [[blood vessel]]s in different parts of the [[body]].
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| | style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |[[Medullary thyroid carcinoma]]
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| | style="padding: 5px 5px; background: #F5F5F5;" |[[Medullary thyroid cancer]] (MTC) is a form of [[thyroid carcinoma]] which originates from the [[parafollicular cell]]s ([[C cell]]s), which produce the [[hormone]] [[calcitonin]].
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| | style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |C-cell hyperplasia
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| | style="padding: 5px 5px; background: #F5F5F5;" |C-cell hyperplasia is a form of [[thyroid]] disease which originates from the [[parafollicular cell]]s ([[C cell]]s), which produce the [[hormone]] [[calcitonin]].
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| | style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |Familial hyperparathyroidism
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| | style="padding: 5px 5px; background: #F5F5F5;" |A [[disorder]] due to excess production of [[parathyroid]] hormone characterized by [[kidney stone]]s, [[hypercalcemia]], [[constipation]], [[peptic ulcer]]s, and [[depression]].
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| | style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |[[Acromegaly]]
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| | style="padding: 5px 5px; background: #F5F5F5;" |A rare syndrome due to excess [[growth hormone]] characterized by enlargement of the [[hand]]s, [[feet]], [[nose]], [[lip]]s, [[ear]]s, general thickening of the [[skin]], [[hypertrichosis]], [[hyperpigmentation]], [[hyperhidrosis]], and [[carpal tunnel syndrome]].
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| | style="padding: 5px 5px; background: #F5F5F5;" colspan="2"|<small>Thakker RV. Multiple endocrine neoplasia type 1 (MEN1) and type 4 (MEN4). ''Mol Cell Endocrinol''. 2014;386(1-2):2-15.<ref name="pmid23933118">{{cite journal| author=Thakker RV| title=Multiple endocrine neoplasia type 1 (MEN1) and type 4 (MEN4). | journal=Mol Cell Endocrinol | year= 2014 | volume= 386 | issue= 1-2 | pages= 2-15 | pmid=23933118 | doi=10.1016/j.mce.2013.08.002 | pmc=PMC4082531 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23933118 }} </ref> </small>
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| |}
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| == Epidemiology and Demographics ==
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| * It is a very rare disease. So far only 12 index cases have been reported and the prevalence of the disease is estimated to be less than 1 in 100,000.
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| ===Age===
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| * The age of onset of [[tumor]] is variable.
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| * [[Pituitary tumor]]s are reported from 3rd decade.
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| * [[Parathyroid adenoma|Parathyroid tumor]]s are reported from 4th decade of life.
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| ===Gender===
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| * Females are more affected than males.
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| == Risk Factors ==
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| The most potent risk factor in the development of multiple endocrine neoplasia type 4 is family history of pituitary tumors and parathyroid tumors.
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| == Natural History, Complications and Prognosis==
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| There is no established comments on the prognosis of multiple endocrine neoplasia type 4.
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| == Diagnosis ==
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| There is no established criteria for the diagnosis of multiple endocrine neoplasia type 4.
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| === Symptoms ===
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| * [[Lethargy]]
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| * [[Depression]]
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| * [[Constipation]]
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| * [[Headache]]
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| == Treatment ==
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| === Surgery and Device Based Therapy ===
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| * Transsphenoidal surgery is used for treating [[pituitary adenoma]] of multiple endocrine neoplasia type 4.
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| ==References==
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| {{Reflist|2}}
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| {{WikiDoc Sources}}
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| [[Category:Oncology]]
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| [[Category:Disease]]
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