197:
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169:
398:, and excess growth hormone causes acromegaly clinically indistinguishable from sporadically occurring acromegaly. About 3% of tumors secrete ACTH, producing Cushing's disease. Most of the remainder are nonfunctional. Local tumor expansion may cause visual disturbance, headache, and hypopituitarism. Pituitary tumors in MEN 1 patients appear to be larger and behave more aggressively than sporadic pituitary tumors.
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47:
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Although many different types of hormone-producing tumors are associated with multiple endocrine neoplasia, tumors of the parathyroid gland, pituitary gland, and pancreas are most frequent in multiple endocrine neoplasia type 1. MEN1-associated overactivity of these three endocrine organs are briefly
337:
Most islet cell tumors secrete pancreatic polypeptide, the clinical significance of which is unknown. Gastrin is secreted by many non–β-cell tumors (increased gastrin secretion in MEN 1 also often originates from the duodenum). Increased gastrin secretion increases gastric acid, which may inactivate
312:
Hyperparathyroidism is present in ≥ 90% of patients. Asymptomatic hypercalcemia is the most common manifestation: about 25% of patients have evidence of nephrolithiasis or nephrocalcinosis. In contrast to sporadic cases of hyperparathyroidism, diffuse hyperplasia or multiple adenomas are more common
439:
can become neoplastic with only one activating mutation, but tumor suppressors inherited from both mother and father must be damaged before they lose their effectiveness. The exception to the "two-hit hypothesis" occurs when suppressor genes exhibit dose-response, such as ATR. The exact function of
412:
Adenomas of adrenal glands occurs occasionally in MEN 1 patients. Hormone secretion is rarely altered as a result, and the significance of these abnormalities is uncertain. Carcinoid tumors, particularly those derived from the embryologic foregut (lungs, thymus), occur in isolated cases. Multiple
486:
Multiple endocrine neoplasia or MEN is part of a group of disorders that affect the body's network of hormone-producing glands (the endocrine system). Hormones are chemical messengers that travel through the bloodstream and regulate the function of cells and tissues throughout the body. Multiple
321:
Pancreatic islet cell tumors are today the major cause of death in persons with MEN-1. Tumors occur in 60-80% of persons with MEN-1 and they are usually multicentric. Multiple adenomas or diffuse islet cell hyperplasia commonly occurs. About 30% of tumors are malignant and have local or distant
645:
The treatment of choice of parathyroid tumors is open bilateral exploration with subtotal (3/4) or total parathyroidectomy. Autoimplantation may be considered in case of a total parathyroidectomy. Optimal timing for this operation has not yet been established but it should be performed by an
382:) sometimes occur in non–β-cell tumors. All of these are rare in MEN 1.Nonfunctioning pancreatic tumors also occur in patients with MEN 1 and may be the most common type of pancreatoduodenal tumor in MEN 1. The size of the nonfunctioning tumor correlates with risk of metastasis and death.
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The two major forms of multiple endocrine neoplasia are called type 1 and type 2. These two types are often confused because of their similar names. However, type 1 and type 2 are distinguished by the genes involved, the types of hormones made, and the characteristic signs and symptoms.
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secretion also leads to peptic ulcers in > 50% of MEN 1 patients. Usually the ulcers are multiple or atypical in location, and often bleed, perforate, or become obstructed. Peptic ulcer disease may be intractable and complicated. Among patients presenting with
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1255:
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experienced endocrine surgeon. The treatment discovered by Joseph
Shepherd in 1997-2001 does not provide a cure, rather extends life expectancy. The treatment requires frequent monitoring to the 1 in 30,000 that suffer from MEN-1.
636:. Zollinger-Ellison syndrome may include severe gastric ulcers, abdominal pain, loss of appetite, chronic diarrhea, malnutrition, and subsequent weight loss. Other non-beta islet cell tumors associated with MEN1 are discussed below.
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A severe secretory diarrhea can develop and cause fluid and electrolyte depletion with non–β-cell tumors. This complex, referred to as the watery diarrhea, hypokalemia and achlorhydria syndrome (
1393:
491:; tumors can also develop in other organs and tissues. These growths can be noncancerous (benign) or cancerous (malignant). If the tumors become cancerous, some cases can be life-threatening.
407:
534:). Tumors can also develop in organs and tissues other than endocrine glands. If the tumors become cancerous, some cases can be life-threatening. The disorder affects 1 in 30,000 people.
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MEN1 and the protein, menin, produced by this gene is not known, but following the inheritance rules of the "two-hit hypothesis" indicates that it acts as a tumor suppressor.
57:
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gene in each cell. Then, during their lifetime, the other copy of the gene is mutated in a small number of cells. These genetic changes result in no functional copies of the
354:) has been ascribed to vasoactive intestinal polypeptide, although other intestinal hormones or secretagogues (including prostaglandins) may contribute. Hypersecretion of
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233:. Individuals suffering from this disorder are prone to developing multiple endocrine and nonendocrine tumors. It was first described by Paul Wermer in 1954.
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530:. Multiple endocrine neoplasia occurs when tumors are found in at least two of the three main endocrine glands (parathyroid, pituitary, and pancreatico-
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Karges W, Schaaf L, Dralle H, Boehm BO (2000). "Concepts for screening and diagnostic follow-up in multiple endocrine neoplasia type 1 (MEN1)".
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604:). However, many other tumors of the pancreatic Islets of Langerhans can occur in MEN-1. One of these, involving the
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gene in selected cells, allowing the cells to divide with little control and form tumors. This is known as
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708:"Identification of the multiple endocrine neoplasia type 1 (MEN1) gene. The European Consortium on MEN1"
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Endocrine pancreatic tumor are treated with surgery and cytotoxic drugs in case of malignant disease.
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1048:"ATR functions as a gene dosage-dependent tumor suppressor on a mismatch repair-deficient background"
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Pituitary tumors are treated with surgery (acromegaly and Mb. Cushing) or medicine (prolactinomas).
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Thakker RV, Newey PJ, Walls GV, Bilezikian J, Dralle H, Ebeling PR, et al. (September 2012).
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Lemmens I, Van de Ven WJ, Kas K, Zhang CX, Giraud S, Wautot V, et al. (July 1997).
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positive family history including a first degree relative proving to have the MEN1 gene.
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585:. Pituitary tumor in MEN1 can be large and cause signs by compressing adjacent tissues.
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Fang Y, Tsao CC, Goodman BK, Furumai R, Tirado CA, Abraham RT, Wang XF (August 2004).
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People with multiple endocrine neoplasia type 1 are born with one mutated copy of the
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subcutaneous and visceral lipomas, angiofibromas, and collagenomas may also occur.
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1167:"Adrenal Liposarcoma: A Novel Presentation of Multiple Endocrine Neoplasia Type 1"
940:"Adrenal Liposarcoma: A Novel Presentation of Multiple Endocrine Neoplasia Type 1"
749:"Adrenal Liposarcoma: A Novel Presentation of Multiple Endocrine Neoplasia Type 1"
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Multiple endocrine neoplasia type 1 is inherited in an autosomal dominant manner.
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Proceedings of the
National Academy of Sciences of the United States of America
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Brandi ML, Agarwal SK, Perrier ND, Lines KE, Valk GD, Thakker RV (March 2021).
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and is a common feature seen with inherited defects in tumor suppressor genes.
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Wermer P (March 1954). "Genetic aspects of adenomatosis of endocrine glands".
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805:"Clinical practice guidelines for multiple endocrine neoplasia type 1 (MEN1)"
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27:"MEN-1" and "MEN 1" redirect here. For the associated gene and protein, see
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if additional diagnostic criteria are sufficiently met, mainly including:
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Pituitary tumors occur in 15 to 42% of MEN 1 patients. From 25 to 90% are
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individuals with a combination of endocrine neoplasias suggestive of the
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if you can. Unsourced or poorly sourced material may be challenged and
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pancreatic lipase, leading to diarrhea and steatorrhea. Increased
334:. β-cell tumors are more common in patients < 40 years of age.
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These disorders greatly increase the risk of developing multiple
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metastases. About 10-15% of islet cell tumors originate from a
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GeneReview/NIH/UW entry on
Multiple Endocrine Neoplasia Type 1
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40:
612:, resulting in a classic triad of high blood glucose levels (
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Pancreatic tumors associated with MEN-1 usually form in the
989:"Mutation and cancer: statistical study of retinoblastoma"
34:"Wermer syndrome" redirects here. Not to be confused with
1214:
408:
List of conditions associated with café au lait macules
72:
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Experimental and
Clinical Endocrinology & Diabetes
889:"Multiple Endocrine Neoplasia Type 1: Latest Insights"
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The
Journal of Clinical Endocrinology and Metabolism
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endocrine neoplasia involves tumors in at least two
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1213:This article incorporates public domain text from
569:Neoplasia in the pituitary gland can manifest as
1098:Robbins & Cotran Pathologic Basis of Disease
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71:Please review the contents of the article and
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581:, causing a decrease in sex hormones such as
8:
209:(MEN-1) is one of a group of disorders, the
632:) and a constellation of sequelae known as
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1095:Kumar V, Abbas AK, Aster JC (2014-09-05).
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1101:. Elsevier Health Sciences. p. 291.
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624:causes the over-secretion of the hormone
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370:, ectopic secretion of ACTH resulting in
295:Learn how and when to remove this message
577:is secreted, suppressing the release of
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542:Overactivity of the parathyroid gland (
1876:Endocrine-related cutaneous conditions
1215:The U.S. National Library of Medicine
87:"Multiple endocrine neoplasia type 1"
7:
677:Multiple endocrine neoplasia type 2b
672:Multiple endocrine neoplasia type 2a
273:adding citations to reliable sources
207:Multiple endocrine neoplasia type 1
154:Multiple endocrine neoplasia type 1
1165:Duro T, Gonzales KL (2023-01-01).
938:Duro T, Gonzales KL (2023-01-01).
747:Duro T, Gonzales KL (2023-01-01).
477:two or more organ systems affected
25:
1422:Autoimmune polyendocrine syndrome
474:multifocal or recurrent neoplasia
162:MEN-1 syndrome, Wermer's syndrome
1619:ACTH-secreting pituitary adenoma
854:The American Journal of Medicine
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1551:Pancreatic neuroendocrine tumor
1624:GH-secreting pituitary adenoma
376:somatotropin–releasing hormone
73:add the appropriate references
1:
1409:Disorders involving multiple
618:necrolytic migratory erythema
211:multiple endocrine neoplasias
1881:Autosomal dominant disorders
1444:Multiple endocrine neoplasia
866:10.1016/0002-9343(54)90353-8
667:Multiple endocrine neoplasia
596:, causing over-secretion of
500:multiple endocrine neoplasia
498:Comparison of main types of
433:Knudson's two-hit hypothesis
1530:Tumours of endocrine glands
608:, causes over-secretion of
58:reliable medical references
1912:
1183:10.1016/j.aace.2022.11.003
1171:AACE Clinical Case Reports
956:10.1016/j.aace.2022.11.003
944:AACE Clinical Case Reports
765:10.1016/j.aace.2022.11.003
753:AACE Clinical Case Reports
634:Zollinger-Ellison syndrome
456:are recommended to have a
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345:Zollinger-Ellison syndrome
33:
26:
1488:Woodhouse–Sakati syndrome
1417:
987:Knudson AG (April 1971).
558:, thinning of the bones (
330:), and can cause fasting
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166:
64:or relies too heavily on
1747:Adrenocortical carcinoma
1064:10.1038/sj.emboj.7600315
712:Human Molecular Genetics
562:), high blood pressure (
374:, and hypersecretion of
347:, 20 to 60% have MEN 1.
313:than solitary adenomas.
1704:Squamous-cell carcinoma
217:through development of
1742:Adrenocortical adenoma
906:10.1210/endrev/bnaa031
518:in glands such as the
503:
1014:10.1073/pnas.68.4.820
497:
1789:Parathyroid neoplasm
822:10.1210/jc.2012-1230
725:10.1093/hmg/6.7.1177
594:islets of Langerhans
554:, which can lead to
402:Other manifestations
269:improve this section
1886:Endocrine neoplasia
1682:Parafollicular cell
1136:10.1055/s-2000-8146
1005:1971PNAS...68..820K
620:, and weight loss.
544:hyperparathyroidism
458:mutational analysis
326:, secrete insulin (
1439:Carcinoid syndrome
1331:External resources
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372:Cushing's syndrome
237:Signs and symptoms
213:, that affect the
1891:Pancreatic cancer
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1629:Craniopharyngioma
1610:Pituitary adenoma
1546:Pancreatic cancer
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1058:(15): 3164–3174.
893:Endocrine Reviews
616:), a rash called
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579:gonadotropins
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571:prolactinomas
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556:kidney stones
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285:December 2023
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254:This section
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89: –
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83:Find sources:
78:
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68:
67:
63:
59:
54:This article
52:
43:
42:
37:
30:
19:
1844:
1809:Pineal gland
1675:Hurthle cell
1660:
1655:
1614:Prolactinoma
1448:
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1177:(1): 10–12.
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759:(1): 10–12.
756:
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682:Prolactinoma
651:
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644:
602:hypoglycemia
583:testosterone
564:hypertension
560:osteoporosis
536:
509:
505:
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453:
447:
428:
422:
420:
411:
396:galactorrhea
389:
364:chromogranin
360:somatostatin
349:
336:
332:hypoglycemia
320:
311:
291:
282:
267:Please help
255:
206:
205:
128:January 2017
125:
115:
108:
101:
94:
82:
62:verification
55:
1827:Pineocytoma
1781:Parathyroid
1634:Pituicytoma
1560:Glucagonoma
1340:MedlinePlus
606:alpha cells
520:parathyroid
308:Parathyroid
227:parathyroid
221:lesions in
159:Other names
56:needs more
1870:Categories
1694:Anaplastic
1671:Follicular
1587:Gastrinoma
1569:Insulinoma
1316:DiseasesDB
688:References
662:Acromegaly
622:Gastrinoma
590:beta cells
406:See also:
380:acromegaly
368:calcitonin
328:insulinoma
229:gland and
219:neoplastic
98:newspapers
1896:Syndromes
1817:Pinealoma
1799:Carcinoma
1687:Medullary
1666:Papillary
1661:carcinoma
1602:Pituitary
1481:Metageria
1476:Acrogeria
1351:eMedicine
1152:260137587
641:Treatment
575:prolactin
524:pituitary
512:cancerous
462:MEN1 gene
444:Diagnosis
437:Oncogenes
386:Pituitary
378:(causing
256:does not
223:pituitary
182:Specialty
1699:Lymphoma
1538:Pancreas
1466:Progeria
1362:Orphanet
1356:med/2404
1289:: 8360/1
1201:36654999
1144:10989951
1082:15282542
974:36654999
925:33249439
874:13138607
839:40362497
831:22723327
783:36654999
656:See also
610:glucagon
532:duodenum
528:pancreas
356:glucagon
317:Pancreas
231:pancreas
187:Oncology
1794:Adenoma
1754:Medulla
1708:Benign
1644:Thyroid
1310:D018761
1192:9837086
1033:5279523
1001:Bibcode
965:9837086
916:7958143
774:9837086
734:9215690
626:gastrin
598:insulin
592:of the
550:in the
548:calcium
460:of the
417:Genetic
340:gastrin
277:removed
262:sources
112:scholar
77:removed
1737:Cortex
1592:VIPoma
1345:000398
1299:131100
1280:258.01
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526:, and
516:tumors
352:VIPoma
324:β-cell
193:
114:
107:
100:
93:
85:
1286:ICD-O
1265:D44.8
1148:S2CID
835:S2CID
552:blood
482:Types
448:In a
366:, or
119:JSTOR
105:books
1432:APS2
1427:APS1
1321:7971
1305:MeSH
1294:OMIM
1275:9-CM
1197:PMID
1140:PMID
1103:ISBN
1078:PMID
1029:PMID
970:PMID
921:PMID
870:PMID
827:PMID
779:PMID
730:PMID
429:MEN1
424:MEN1
260:any
258:cite
91:news
60:for
29:MEN1
1837:MEN
1367:652
1271:ICD
1256:ICD
1187:PMC
1179:doi
1132:doi
1128:108
1068:PMC
1060:doi
1019:PMC
1009:doi
960:PMC
952:doi
911:PMC
901:doi
862:doi
817:doi
769:PMC
761:doi
720:doi
271:by
1872::
1855:2B
1850:2A
1612::
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1459:2B
1454:2A
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856:.
833:.
825:.
813:97
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791:^
777:.
767:.
755:.
751:.
728:.
714:.
710:.
696:^
522:,
362:,
358:,
225:,
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1583:G
1574:δ
1565:β
1556:α
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298:)
292:(
287:)
283:(
279:.
265:.
130:)
126:(
116:·
109:·
102:·
95:·
69:.
38:.
31:.
20:)
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