576:
725:
non-endocrine neoplasms including adrenocortical and thyroid tumors, visceral and cutaneous lipomas, meningiomas, facial angiofibromas and collagenomas, and thymic, gastric, and bronchial carcinoids also occur. The phenotype of MEN1 is broad, and over 20 different combinations of endocrine and non-endocrine manifestations have been described. MEN1 should be suspected in patients with an endocrinopathy of two of the three characteristic affected organs, or with an endocrinopathy of one of these organs plus a first-degree relative affected by MEN1 syndrome.
114:
95:
25:
662:, inherited from one parent (familial cases) or developed in an early embryonic stage (sporadic cases) and present in all cells at birth. The second hit is a MEN1 somatic mutation, usually a large deletion, that occurs in the predisposed endocrine cell as loss of the remaining wild-type allele and gives cells the survival advantage needed for tumor development.
647:(47%) (47-51). Human and mouse MEN1 amino acid sequences share 95.8% identity and 98.4% similarity. Analysis of menin amino acid sequence did not reveal homologies to any other known human or mammalian protein, sequence motif, or signal peptide. The absence of significant homology to any other protein complicates efforts to elucidate the functions of menin.
733:
malignancy. Entero-pancreatic gastrinomas and thymic and bronchial carcinoids are the leading cause of morbidity and mortality. Consequently, the average age of death in untreated individuals with MEN1 is significantly lower (55.4 years for men and 46.8 years for women) than that of the general population.
820:
The term multiple endocrine neoplasias are used when two or more endocrine tumor types, known to occur as a part of one of the defined MEN syndromes, occurs in a single patient and there is evidence for either a causative mutation or hereditary transmission. The presence of two or more tumor types in
715:
50% of patients develop signs and symptoms by 20 years of age and more than 95% have symptoms by 40 years of age. There is significant intra- and inter-familial variability in the age of onset, the severity of disease, and tumor types. Despite numerous studies, no genotype-phenotype correlations have
634:
gene consists of ten exons, spanning about 10 kb, and encodes a 610 amino acid protein named menin. The first exon and the last part of exon 10 are not translated. The main transcript of 2.8 kb has been described in a large variety of human tissues (pancreas, thymus, adrenal glands, thyroid, testis,
596:
is sometimes known as MEN 3 and the designation varies by institution (c.f. www.ClinicalReview.com). Although a variety of additional eponyms have been proposed for MEN2B (e.g. Williams-Pollock syndrome, Gorlin-Vickers syndrome, and
Wagenmann–Froboese syndrome), none ever gained sufficient traction
711:
gene mutations can be identified in 70–95% of MEN1 patients and in about 20% of familial isolated hyperparathyroidism cases. Almost all patients are heterozygous for mutations. One affected family has been identified with individuals both homozygous and heterozygous for MEN1 mutations. In this
724:
Multiple
Endocrine Neoplasia type 1 (MEN1) is a rare hereditary endocrine cancer syndrome characterized primarily by tumors of the parathyroid glands (95% of cases), endocrine gastroenteropancreatic (GEP) tract (30–80% of cases), and anterior pituitary (15–90% of cases). Other endocrine and
732:
Many endocrine tumors in MEN1 are benign and cause symptoms by overproduction of hormones or local mass effects, while other MEN1 tumors are associated with an elevated risk for malignancy. About one-third of patients affected with MEN1 will die early from MEN1-related cancer or associated
956:
Boyce, Alison M.; Collins, Michael T. (1993). "Fibrous
Dysplasia/McCune-Albright Syndrome". In Adam, Margaret P.; Ardinger, Holly H.; Pagon, Roberta A.; Wallace, Stephanie E.; Bean, Lora J.H.; Mefford, Heather C.; Stephens, Karen; Amemiya, Anne; Ledbetter, Nikki (eds.).
791:
showed that the MEN 2 category included two groups of patients with MTC and pheochromocytoma: one with parathyroid disease and a normal appearance (MEN 2A) and the other without parathyroid disease but with mucosal neuromas and mesodermal abnormalities (MEN 2B).
728:
MEN1 patients usually have a family history of MEN1. Inheritance is autosomal dominant; any affected parent has a 50% chance to transmit the disease to his or her progeny. MEN1 gene mutations can be identified in 70–95% of MEN1 patients.
821:
a single patient does not automatically designate that individual as having MEN because there is a small statistical chance that the development of two "sporadic" tumors that occur in one of the MEN syndromes could occur by chance.
1567:
Germ-line , Mulligan LM, Kwok JB, Healey CS, Elsdon MJ, Eng C, Gardner E, Love DR, Mole SE, Moore JK, Papi L; et al. (Jun 1993). "Germ-line mutations of the RET proto-oncogene in multiple endocrine neoplasia type 2A".
1301:
Schimke RN, Hartmann WH, Prout TE, Rimoin DL (1968). "Syndrome of bilateral pheochromocytoma, medullary thyroid carcinoma and multiple neuromas. A possible regulatory defect in the differentiation of chromaffin tissue".
635:
leukocytes, heart, brain, lung, muscle, small intestine, liver, and kidney); an additional transcript of approximately 4 kb has been detected in pancreas and thymus, suggesting tissue-specific alternative splicing.
1754:
1739:
149:, each with its own characteristic pattern. In some cases, the tumors are malignant, in others, benign. Benign or malignant tumors of nonendocrine tissues occur as components of some of these tumor syndromes.
783:
introduced the term "multiple endocrine neoplasias" (MEN) to describe disorders featuring combinations of endocrine tumors and proposed the terms 'Wermer syndrome' for MEN 1 and 'Sipple syndrome' for MEN 2.
1034:"Cutaneous tumors in patients with multiple endocrine neoplasm type 1 (MEN1) and gastrinomas: prospective study of frequency and development of criteria with high sensitivity and specificity for MEN1"
862:
1840:
1484:
Riegert-Johnson, D. L.; Boardman, L. A.; Hefferon, T.; Roberts, M.; Riegert-Johnson, D.; Gleeson, F. C.; Westra, W.; Hefferon, T.; Wong Kee Song, L. M.; Spurck, L.; Boardman, L. A. (2009).
1455:
Riegert-Johnson, D. L.; Boardman, L. A.; Hefferon, T.; Roberts, M.; Riegert-Johnson, D.; Gleeson, F. C.; Westra, W.; Hefferon, T.; Wong Kee Song, L. M.; Spurck, L.; Boardman, L. A. (2009).
741:
A recommend surveillance program for
Multiple Endocrine Neoplasia Type 1 has been suggested by the International Guidelines for Diagnosis and Therapy of MEN syndromes group.
177:
are two other autosomal dominant endocrine tumor syndromes with features that overlap the clinical features of the MEN syndromes. Although not transmitted in the germline,
1229:
This reference quotes a prevalence of 1 in 40,000, but this figure is inconsistent with the same reference's calculated incidence of 4 per 100 million per year for MEN2B.
1833:
1826:
1769:
1081:
123I labeled metaiodobenzylguanidine for diagnosis of neuroendocrine tumors. Jiang L, Schipper ML, Li P, Cheng Z, Reports in
Medical Imaging. 2009: 2 79-89
2596:
1539:
1352:
Pellegata NS, Quintanilla-Martinez L, Siggelkow H; Quintanilla-Martinez; Siggelkow; Samson; Bink; Hofler; Fend; Graw; Atkinson; et al. (Oct 2006).
798:
In 1993 mutations in the RET oncogene were shown to be the cause of MEN 2A by Lois
Mulligan, working in the laboratory of Bruce Ponder in Cambridge.
1619:
1411:
1960:
1538:
Riegert-Johnson, D. L.; Boardman, L. A.; Hefferon, T.; Roberts, M.; Marini, F.; Falchetti, A.; Luzi, E.; Tonelli, F.; Maria Luisa, B. (2009).
1566:
643:
Menin is a 610 amino acid (67Kda) nuclear protein, highly conserved from mouse (98%), rat (97%) and, more distantly, zebrafish (75%) and
2699:
716:
been established, suggesting that unknown genetic and environmental modifiers are involved in the expression of the MEN1 phenotype.
1871:
1866:
2589:
1074:
2961:
1215:
1013:
749:
In 1903 Erdheim described the case of an acromegalic patient with a pituitary adenoma and three enlarged parathyroid glands.
185:
characterized by endocrine neoplastic features involving endocrine glands that overlap with those involved in MEN1 or MEN2.
2930:
1898:
867:
843:
838:
593:
219:
2925:
2920:
2435:
1893:
1888:
1336:
833:
214:
207:
202:
2582:
2004:
1953:
597:
to merit continued use and, indeed, are all but abandoned in the medical literature. Another early report was
Schimke
658:
follows
Knudson’s “two-hit” model for tumor suppressor gene carcinogenesis (30). The first hit is a heterozygous MEN1
1861:
824:
The term "multiple endocrine neoplasia" was introduced in 1968, but descriptions of the condition date back to 1903.
224:
61:
1351:
2485:
1486:"Multiple Endocrine Neoplasia Type 1 : In Familial Cancer Syndromes. DL Riegert-Johnson and others. NCBI 2009"
769:
In 1961 Sipple described a combination of a pheochromocytoma, medullary thyroid carcinoma and parathyroid adenoma.
193:
Percentages in the table below refer to the percentage of people with the MEN type who develop the neoplasia type.
2779:
2626:
2489:
2322:
1485:
1456:
2110:
756:
reported a case series of 8 patients with a syndrome of pituitary, parathyroid, and pancreatic islet adenomas.
712:
family, there was no difference in disease history between the homozygous and heterozygous mutation carriers.
2427:
1946:
1927:
1540:"Multiple Endocrine Neoplasia Type 1: In Familial Cancer Syndromes. DL Riegert-Johnson and others. NCBI 2009"
1457:"Multiple Endocrine Neoplasia Type 1: In Familial Cancer Syndromes. DL Riegert-Johnson and others. NCBI 2009"
170:
2951:
178:
2956:
2474:
2391:
2024:
583:
394:
2966:
2531:
2513:
2423:
2374:
43:
2769:
2746:
2508:
2431:
2268:
2094:
2057:
1633:
Guru, S. C.; Manickam, P.; Crabtree, J. S.; Olufemi, S. E.; Agarwal, S. K.; Debelenko, L. V. (1998).
35:
113:
2822:
2762:
2741:
2605:
2536:
2469:
2404:
2278:
2258:
2225:
2203:
2165:
1777:
146:
125:
RET receptor defect Which is growth signal receptor, its type of self-sufficiency of growth signals
1513:
1430:
2732:
2014:
1354:"Germ-line mutations in p27Kip1 cause a multiple endocrine neoplasia syndrome in rats and humans"
776:
described the combination of mucosal neuromas, pheochromocytoma and medullary thyroid carcinoma.
1818:
700:
2817:
2397:
2221:
2161:
882:"Familial multiple endocrine neoplasia syndromes: components, classification, and nomenclature"
1806:
2874:
2559:
2283:
1613:
1405:
1274:
Sipple JH (1961). "The association of pheochromocytoma with carcinoma of the thyroid gland".
1142:
Marx, Stephen J (2011). "Chapter 41: Multiple endocrine neoplasia". In Melmed, Shlomo (ed.).
575:
1635:"Identification and characterization of the multiple endocrine neoplasia type 1 (MEN1) gene"
2864:
2694:
2387:
2246:
2183:
2019:
1999:
1577:
1365:
1032:
Asgharian, B; Turner, ML; Gibril, F; Entsuah, LK; Serrano, J; Jensen, RT (November 2004).
8:
2869:
2757:
2523:
2175:
2009:
1758:
1581:
1369:
921:
Callender GG, Rich TA, Perrier ND (Aug 2008). "Multiple endocrine neoplasia syndromes".
2677:
2356:
2188:
2151:
2047:
2029:
1878:
1707:
1695:
1664:
1601:
1388:
1353:
1188:
1002:
508:
498:
488:
153:
1763:
607:
also includes a fourth form of multiple endocrine neoplasia ("MEN4"), associated with
2856:
2704:
2685:
2621:
2500:
1699:
1656:
1651:
1634:
1593:
1543:
1489:
1460:
1393:
1319:
1287:
1256:
1252:
1211:
1180:
1124:
1055:
1009:
964:
938:
903:
898:
881:
694:
659:
317:
102:
1711:
1682:
Carney JA (Feb 2005). "Familial multiple endocrine neoplasia: the first 100 years".
1668:
1192:
1109:
1092:
2834:
2774:
2719:
2461:
2379:
2193:
2105:
1691:
1646:
1605:
1585:
1383:
1373:
1311:
1283:
1248:
1170:
1114:
1104:
1072:
1045:
930:
893:
684:
413:
182:
1938:
2897:
2829:
2787:
2653:
2479:
2142:
1910:
1850:
1782:
1227:
1175:
1158:
1093:"Pancreatitis as the first manifestation of multiple endocrine neoplasia type 2A"
1078:
432:
2230:
1315:
958:
2812:
2727:
2156:
2085:
1748:
174:
169:
Although not officially categorized as multiple endocrine neoplasia syndromes,
94:
2574:
934:
2945:
2846:
2839:
2804:
2792:
759:
In 1954 Wermer noted that this syndrome was transmitted as a dominant trait.
107:
1378:
2884:
2750:
2689:
2414:
2366:
2310:
2211:
1703:
1547:
1493:
1464:
1397:
1260:
1184:
1128:
1059:
968:
942:
814:
587:
336:
1660:
1597:
1323:
907:
2902:
2709:
2635:
2352:
2127:
2039:
1801:
1050:
1033:
374:
298:
1731:
1239:
Wermer P (1954). "Genetic aspects of adenomatosis of endocrine glands".
1226:
by
Electron Kebebew, Jessica E. Gosnell and Emily Reiff. Pages 695-701.
704:
mutations in multiple endocrine neoplasia patients and clinical genetics
2662:
2649:
2644:
2631:
2263:
2132:
2122:
1978:
1119:
1031:
579:
530:
288:
283:
277:
1537:
1483:
1454:
2892:
2640:
2315:
2251:
1991:
1987:
1920:
1915:
1589:
1091:
Dora JM, Siqueira DR, Meyer EL, Puñales MK, Maia AL (November 2008).
999:
133:
2613:
1905:
810:
142:
670:
A useful mnemonic to remember the associated neoplasias is below:
2273:
2080:
2052:
1848:
611:. The presentation is believed to overlap that of MEN1 and MEN2.
453:
1340:
521:
512:
502:
492:
482:
270:
265:
260:
255:
2667:
2658:
2550:
2343:
2137:
1970:
1743:
608:
355:
293:
231:
795:
In 1988 the MEN1 locus was assigned to
Chromosome 11 (11q13).
2071:
614:
517:
1632:
630:
604:
478:
472:
250:
1300:
1090:
817:" (MEA), have been replaced by the current terminology.
2079:
1008:. Hagerstwon, MD: Lippincott Williams & Wilkins.
40:
In particular, it has problems with not using MEDMOS.
1721:
1341:
MULTIPLE ENDOCRINE NEOPLASIA, TYPE IV; MEN4 - 610755
1038:
The Journal of Clinical Endocrinology and Metabolism
920:
141:) is a condition which encompasses several distinct
1968:
963:. Seattle (WA): University of Washington, Seattle.
1159:"Multiple endocrine neoplasia type 2: An overview"
1001:
736:
2943:
1208:Neurocutaneous Disorders : The Phakomatoses
1205:
1000:Elizabeth D Agabegi; Agabegi, Steven S. (2008).
2604:
994:
992:
990:
988:
986:
984:
982:
980:
978:
766:described medullary (solid) thyroid carcinoma.
2590:
1954:
1834:
1675:
1618:: CS1 maint: multiple names: authors list (
1410:: CS1 maint: multiple names: authors list (
1345:
975:
955:
1144:Williams Textbook of Endocrinology, 12th ed
1027:
1025:
914:
2597:
2583:
1961:
1947:
1841:
1827:
873:
693:- Pheochromocytoma, Medullary Thyroid Ca,
615:Multiple Endocrine Neoplasia Type 1 (MEN1)
112:
93:
1681:
1650:
1387:
1377:
1273:
1174:
1156:
1118:
1108:
1049:
897:
879:
572:FMTC = familial medullary thyroid cancer
62:Learn how and when to remove this message
1626:
1238:
1022:
574:
569:*- of patients with MEN1 and gastrinoma
1425:
1423:
1421:
586:, as may be seen in MEN 2A and MEN 2B.
2944:
2578:
1942:
1822:
1224:Multiple Endocrine Neoplasia Type 2B
809:The older names, "multiple endocrine
164:
1518:The Lecturio Medical Concept Library
1435:The Lecturio Medical Concept Library
1418:
1141:
1004:Step-Up to Medicine (Step-Up Series)
844:Multiple endocrine neoplasia type 2b
839:Multiple endocrine neoplasia type 2a
677:- Pituitary, Parathyroid, Pancreatic
638:
18:
2436:Papillary serous cystadenocarcinoma
1337:Online Mendelian Inheritance in Man
834:Multiple endocrine neoplasia type 1
687:, Parathyroid, Medullary Thyroid Ca
13:
2005:Combined small-cell lung carcinoma
1696:10.1097/01.pas.0000147402.95391.41
801:In 1998 the MEN1 gene was cloned.
650:
14:
2978:
1862:Autoimmune polyendocrine syndrome
719:
2695:ACTH-secreting pituitary adenoma
1652:10.1046/j.1365-2796.1998.00346.x
899:10.1046/j.1365-2796.1998.00345.x
619:
23:
2627:Pancreatic neuroendocrine tumor
2323:Syringocystadenoma papilliferum
1560:
1531:
1506:
1477:
1448:
1330:
1294:
1267:
1232:
1199:
1150:
1110:10.1590/S0004-27302008000800021
737:Recommended cancer surveillance
159:
152:MEN syndromes are inherited as
2700:GH-secreting pituitary adenoma
2111:Familial adenomatous polyposis
1514:"Multiple Endocrine Neoplasia"
1431:"Multiple Endocrine Neoplasia"
1135:
1084:
1066:
949:
863:"multiple endocrine neoplasia"
856:
804:
32:This article needs editing to
1:
2486:Paget's disease of the breast
2428:Pancreatic serous cystadenoma
1849:Disorders involving multiple
849:
188:
2962:Autosomal dominant disorders
2490:Extramammary Paget's disease
2217:Multiple endocrine neoplasia
1884:Multiple endocrine neoplasia
1358:Proc. Natl. Acad. Sci. U.S.A
1288:10.1016/0002-9343(61)90234-0
1253:10.1016/0002-9343(54)90353-8
1176:10.1097/GIM.0b013e318216cc6d
868:Dorland's Medical Dictionary
242:Wagenmann–Froboese syndrome
80:Multiple endocrine neoplasia
7:
2606:Tumours of endocrine glands
2475:Pancreatic ductal carcinoma
2392:Mucinous cystadenocarcinoma
2025:Transitional cell carcinoma
1316:10.1056/NEJM196807042790101
1097:Arq Bras Endocrinol Metabol
827:
665:
584:medullary thyroid carcinoma
554:Initial description (year)
395:Medullary thyroid carcinoma
16:Group of genetic conditions
10:
2983:
2532:Medullary breast carcinoma
2514:Invasive lobular carcinoma
2424:Ovarian serous cystadenoma
2375:Signet ring cell carcinoma
813:" and "multiple endocrine
744:
147:tumors of endocrine glands
2911:
2883:
2855:
2803:
2718:
2676:
2612:
2549:
2522:
2509:Lobular carcinoma in situ
2499:
2460:
2449:
2432:Serous cystadenocarcinoma
2413:
2365:
2342:
2331:
2296:
2269:Clear-cell adenocarcinoma
2239:
2202:
2174:
2093:
2070:
2058:Bartholin gland carcinoma
2038:
1986:
1977:
1969:Glandular and epithelial
1928:Woodhouse–Sakati syndrome
1857:
1792:
1725:
1206:Martino Ruggieri (2005).
935:10.1016/j.suc.2008.05.001
206:
201:
198:
171:Von Hippel–Lindau disease
121:
101:
92:
84:
79:
2823:Adrenocortical carcinoma
2537:Medullary thyroid cancer
2470:Mammary ductal carcinoma
2405:Mucoepidermoid carcinoma
2259:Adenoid cystic carcinoma
2226:Adrenocortical carcinoma
2166:Hepatocellular carcinoma
1157:Moline J, Eng C (2011).
179:McCune–Albright syndrome
34:comply with Knowledge's
2780:Squamous-cell carcinoma
2015:Squamous-cell carcinoma
1379:10.1073/pnas.0603877103
2818:Adrenocortical adenoma
2398:Pseudomyxoma peritonei
2222:Adrenocortical adenoma
2162:Hepatocellular adenoma
880:Carney JA (Jun 1998).
590:
2560:Acinic cell carcinoma
2284:Papillary hidradenoma
1146:. pp. 1728–1767.
578:
2865:Parathyroid neoplasm
2388:Mucinous cystadenoma
2247:Neuroendocrine tumor
2184:Renal cell carcinoma
2020:Basal-cell carcinoma
2000:Small-cell carcinoma
1210:. Berlin: Springer.
1163:Genetics in Medicine
1051:10.1210/jc.2004-0218
923:Surg. Clin. North Am
2952:Endocrine neoplasia
2758:Parafollicular cell
2524:Medullary carcinoma
2010:Verrucous carcinoma
1684:Am. J. Surg. Pathol
1582:1993Natur.363..458M
1370:2006PNAS..10315558P
132:Multiple endocrine
44:improve the content
2957:Hereditary cancers
2357:Cystadenocarcinoma
2189:Endometrioid tumor
2152:Cholangiocarcinoma
2040:Complex epithelial
2030:Inverted papilloma
1879:Carcinoid syndrome
1793:External resources
1077:2017-03-14 at the
752:In 1953 Underdahl
591:
165:Related conditions
154:autosomal dominant
2967:Medical mnemonics
2939:
2938:
2705:Craniopharyngioma
2686:Pituitary adenoma
2622:Pancreatic cancer
2572:
2571:
2568:
2567:
2545:
2544:
2501:Lobular carcinoma
2445:
2444:
2292:
2291:
2066:
2065:
1936:
1935:
1816:
1815:
1217:978-3-211-21396-4
1015:978-0-7817-7153-5
787:In 1974 Sizemore
772:In 1966 Williams
697:/mucosal neuroma
695:Marfanoid habitus
660:germline mutation
639:The Menin Protein
567:
566:
318:Pituitary adenoma
129:
128:
74:Medical condition
72:
71:
64:
2974:
2835:Pheochromocytoma
2599:
2592:
2585:
2576:
2575:
2492:
2462:Ductal carcinoma
2458:
2457:
2438:
2394:
2380:Krukenberg tumor
2340:
2339:
2194:Renal oncocytoma
2106:Linitis plastica
2095:Gastrointestinal
2091:
2090:
2077:
2076:
1984:
1983:
1963:
1956:
1949:
1940:
1939:
1851:endocrine glands
1843:
1836:
1829:
1820:
1819:
1723:
1722:
1716:
1715:
1679:
1673:
1672:
1654:
1630:
1624:
1623:
1617:
1609:
1590:10.1038/363458a0
1576:(6428): 458–60.
1564:
1558:
1557:
1555:
1554:
1535:
1529:
1528:
1526:
1524:
1510:
1504:
1503:
1501:
1500:
1481:
1475:
1474:
1472:
1471:
1452:
1446:
1445:
1443:
1441:
1427:
1416:
1415:
1409:
1401:
1391:
1381:
1364:(42): 15558–63.
1349:
1343:
1334:
1328:
1327:
1298:
1292:
1291:
1271:
1265:
1264:
1236:
1230:
1221:
1203:
1197:
1196:
1178:
1154:
1148:
1147:
1139:
1133:
1132:
1122:
1112:
1088:
1082:
1070:
1064:
1063:
1053:
1029:
1020:
1019:
1007:
996:
973:
972:
953:
947:
946:
918:
912:
911:
901:
877:
871:
860:
779:In 1968 Steiner
685:Pheochromocytoma
549:1 in 4,000,000)
547:(1 in 600,000 to
414:Pheochromocytoma
239:Sipple syndrome
236:Wermer syndrome
196:
195:
183:genetic disorder
117:
116:
97:
77:
76:
67:
60:
56:
53:
47:
27:
26:
19:
2982:
2981:
2977:
2976:
2975:
2973:
2972:
2971:
2942:
2941:
2940:
2935:
2907:
2898:Pinealoblastoma
2879:
2851:
2799:
2788:Thyroid adenoma
2733:epithelial-cell
2714:
2672:
2654:Somatostatinoma
2608:
2603:
2573:
2564:
2541:
2518:
2495:
2484:
2480:Comedocarcinoma
2453:
2451:
2441:
2422:
2409:
2386:
2361:
2335:
2333:
2327:
2298:
2288:
2235:
2198:
2170:
2143:Somatostatinoma
2086:adenocarcinomas
2084:
2062:
2048:Warthin's tumor
2034:
1973:
1967:
1937:
1932:
1911:Werner syndrome
1853:
1847:
1817:
1812:
1811:
1788:
1787:
1734:
1720:
1719:
1680:
1676:
1631:
1627:
1611:
1610:
1565:
1561:
1552:
1550:
1536:
1532:
1522:
1520:
1512:
1511:
1507:
1498:
1496:
1482:
1478:
1469:
1467:
1453:
1449:
1439:
1437:
1429:
1428:
1419:
1403:
1402:
1350:
1346:
1335:
1331:
1304:N. Engl. J. Med
1299:
1295:
1272:
1268:
1237:
1233:
1218:
1204:
1200:
1155:
1151:
1140:
1136:
1089:
1085:
1079:Wayback Machine
1071:
1067:
1044:(11): 5328–36.
1030:
1023:
1016:
997:
976:
954:
950:
919:
915:
878:
874:
861:
857:
852:
830:
807:
762:In 1959 Hazard
747:
739:
722:
706:
688:
678:
668:
653:
651:Pathophysiology
641:
626:
617:
548:
546:
538:
537:(1 in 20,000 to
536:
516:
302:
297:
292:
287:
191:
167:
162:
111:
75:
68:
57:
51:
48:
41:
36:Manual of Style
28:
24:
17:
12:
11:
5:
2980:
2970:
2969:
2964:
2959:
2954:
2937:
2936:
2934:
2933:
2928:
2923:
2917:
2915:
2909:
2908:
2906:
2905:
2900:
2895:
2889:
2887:
2881:
2880:
2878:
2877:
2872:
2867:
2861:
2859:
2853:
2852:
2850:
2849:
2844:
2843:
2842:
2837:
2827:
2826:
2825:
2820:
2809:
2807:
2801:
2800:
2798:
2797:
2796:
2795:
2790:
2782:
2777:
2772:
2767:
2766:
2765:
2755:
2754:
2753:
2744:
2728:Thyroid cancer
2724:
2722:
2716:
2715:
2713:
2712:
2707:
2702:
2697:
2692:
2682:
2680:
2674:
2673:
2671:
2670:
2665:
2656:
2647:
2638:
2629:
2624:
2618:
2616:
2610:
2609:
2602:
2601:
2594:
2587:
2579:
2570:
2569:
2566:
2565:
2563:
2562:
2556:
2554:
2547:
2546:
2543:
2542:
2540:
2539:
2534:
2528:
2526:
2520:
2519:
2517:
2516:
2511:
2505:
2503:
2497:
2496:
2494:
2493:
2482:
2477:
2472:
2466:
2464:
2455:
2447:
2446:
2443:
2442:
2440:
2439:
2419:
2417:
2411:
2410:
2408:
2407:
2402:
2401:
2400:
2384:
2383:
2382:
2371:
2369:
2363:
2362:
2360:
2359:
2349:
2347:
2337:
2329:
2328:
2326:
2325:
2320:
2319:
2318:
2313:
2302:
2300:
2299:skin appendage
2294:
2293:
2290:
2289:
2287:
2286:
2281:
2276:
2271:
2266:
2261:
2256:
2255:
2254:
2243:
2241:
2240:Other/multiple
2237:
2236:
2234:
2233:
2228:
2219:
2214:
2208:
2206:
2200:
2199:
2197:
2196:
2191:
2186:
2180:
2178:
2172:
2171:
2169:
2168:
2159:
2157:Klatskin tumor
2154:
2148:
2147:
2146:
2145:
2140:
2135:
2130:
2125:
2114:
2113:
2108:
2099:
2097:
2088:
2074:
2068:
2067:
2064:
2063:
2061:
2060:
2055:
2050:
2044:
2042:
2036:
2035:
2033:
2032:
2027:
2022:
2017:
2012:
2007:
2002:
1996:
1994:
1981:
1975:
1974:
1966:
1965:
1958:
1951:
1943:
1934:
1933:
1931:
1930:
1925:
1924:
1923:
1918:
1913:
1903:
1902:
1901:
1896:
1891:
1881:
1876:
1875:
1874:
1869:
1858:
1855:
1854:
1846:
1845:
1838:
1831:
1823:
1814:
1813:
1810:
1809:
1797:
1796:
1794:
1790:
1789:
1786:
1785:
1774:
1766:
1751:
1735:
1730:
1729:
1727:
1726:Classification
1718:
1717:
1674:
1625:
1559:
1530:
1505:
1476:
1447:
1417:
1344:
1329:
1293:
1266:
1231:
1216:
1198:
1169:(9): 755–764.
1149:
1134:
1083:
1065:
1021:
1014:
974:
948:
913:
886:J. Intern. Med
872:
854:
853:
851:
848:
847:
846:
841:
836:
829:
826:
806:
803:
746:
743:
738:
735:
721:
720:Manifestations
718:
705:
699:
667:
664:
652:
649:
640:
637:
625:
618:
616:
613:
565:
564:
561:
558:
555:
551:
550:
545:1 in 1,000,000
543:
540:
533:
526:
525:
506:
496:
486:
476:
469:
468:
465:
462:
459:
456:
449:
448:
445:
442:
439:
436:
429:
428:
425:
422:
419:
416:
410:
409:
406:
403:
400:
397:
391:
390:
387:
384:
381:
378:
371:
370:
367:
364:
361:
358:
352:
351:
348:
345:
342:
339:
333:
332:
329:
326:
323:
320:
314:
313:
310:
307:
304:
281:
274:
273:
268:
263:
258:
253:
247:
246:
243:
240:
237:
234:
228:
227:
222:
217:
211:
210:
205:
200:
190:
187:
175:Carney complex
166:
163:
161:
158:
127:
126:
123:
119:
118:
105:
99:
98:
90:
89:
86:
82:
81:
73:
70:
69:
31:
29:
22:
15:
9:
6:
4:
3:
2:
2979:
2968:
2965:
2963:
2960:
2958:
2955:
2953:
2950:
2949:
2947:
2932:
2929:
2927:
2924:
2922:
2919:
2918:
2916:
2914:
2910:
2904:
2901:
2899:
2896:
2894:
2891:
2890:
2888:
2886:
2882:
2876:
2873:
2871:
2868:
2866:
2863:
2862:
2860:
2858:
2854:
2848:
2847:Paraganglioma
2845:
2841:
2840:Neuroblastoma
2838:
2836:
2833:
2832:
2831:
2828:
2824:
2821:
2819:
2816:
2815:
2814:
2811:
2810:
2808:
2806:
2805:Adrenal tumor
2802:
2794:
2793:Struma ovarii
2791:
2789:
2786:
2785:
2783:
2781:
2778:
2776:
2773:
2771:
2768:
2764:
2761:
2760:
2759:
2756:
2752:
2748:
2745:
2743:
2740:
2739:
2738:
2735:
2734:
2730:(malignant):
2729:
2726:
2725:
2723:
2721:
2717:
2711:
2708:
2706:
2703:
2701:
2698:
2696:
2693:
2691:
2687:
2684:
2683:
2681:
2679:
2675:
2669:
2666:
2664:
2660:
2657:
2655:
2651:
2648:
2646:
2642:
2639:
2637:
2633:
2630:
2628:
2625:
2623:
2620:
2619:
2617:
2615:
2611:
2607:
2600:
2595:
2593:
2588:
2586:
2581:
2580:
2577:
2561:
2558:
2557:
2555:
2552:
2548:
2538:
2535:
2533:
2530:
2529:
2527:
2525:
2521:
2515:
2512:
2510:
2507:
2506:
2504:
2502:
2498:
2491:
2487:
2483:
2481:
2478:
2476:
2473:
2471:
2468:
2467:
2465:
2463:
2459:
2456:
2454:and medullary
2448:
2437:
2433:
2429:
2425:
2421:
2420:
2418:
2416:
2412:
2406:
2403:
2399:
2396:
2395:
2393:
2389:
2385:
2381:
2378:
2377:
2376:
2373:
2372:
2370:
2368:
2364:
2358:
2354:
2351:
2350:
2348:
2345:
2341:
2338:
2330:
2324:
2321:
2317:
2314:
2312:
2309:
2308:
2307:
2304:
2303:
2301:
2295:
2285:
2282:
2280:
2277:
2275:
2272:
2270:
2267:
2265:
2262:
2260:
2257:
2253:
2250:
2249:
2248:
2245:
2244:
2242:
2238:
2232:
2229:
2227:
2223:
2220:
2218:
2215:
2213:
2210:
2209:
2207:
2205:
2201:
2195:
2192:
2190:
2187:
2185:
2182:
2181:
2179:
2177:
2173:
2167:
2163:
2160:
2158:
2155:
2153:
2150:
2149:
2144:
2141:
2139:
2136:
2134:
2131:
2129:
2126:
2124:
2121:
2120:
2119:
2116:
2115:
2112:
2109:
2107:
2104:
2101:
2100:
2098:
2096:
2092:
2089:
2087:
2082:
2078:
2075:
2073:
2069:
2059:
2056:
2054:
2051:
2049:
2046:
2045:
2043:
2041:
2037:
2031:
2028:
2026:
2023:
2021:
2018:
2016:
2013:
2011:
2008:
2006:
2003:
2001:
1998:
1997:
1995:
1993:
1989:
1985:
1982:
1980:
1976:
1972:
1964:
1959:
1957:
1952:
1950:
1945:
1944:
1941:
1929:
1926:
1922:
1919:
1917:
1914:
1912:
1909:
1908:
1907:
1904:
1900:
1897:
1895:
1892:
1890:
1887:
1886:
1885:
1882:
1880:
1877:
1873:
1870:
1868:
1865:
1864:
1863:
1860:
1859:
1856:
1852:
1844:
1839:
1837:
1832:
1830:
1825:
1824:
1821:
1808:
1804:
1803:
1799:
1798:
1795:
1791:
1784:
1780:
1779:
1775:
1772:
1771:
1767:
1765:
1761:
1760:
1756:
1752:
1750:
1746:
1745:
1741:
1737:
1736:
1733:
1728:
1724:
1713:
1709:
1705:
1701:
1697:
1693:
1690:(2): 254–74.
1689:
1685:
1678:
1670:
1666:
1662:
1658:
1653:
1648:
1644:
1640:
1636:
1629:
1621:
1615:
1607:
1603:
1599:
1595:
1591:
1587:
1583:
1579:
1575:
1571:
1563:
1549:
1545:
1541:
1534:
1519:
1515:
1509:
1495:
1491:
1487:
1480:
1466:
1462:
1458:
1451:
1436:
1432:
1426:
1424:
1422:
1413:
1407:
1399:
1395:
1390:
1385:
1380:
1375:
1371:
1367:
1363:
1359:
1355:
1348:
1342:
1338:
1333:
1325:
1321:
1317:
1313:
1309:
1305:
1297:
1289:
1285:
1281:
1277:
1270:
1262:
1258:
1254:
1250:
1247:(3): 363–71.
1246:
1242:
1235:
1228:
1225:
1219:
1213:
1209:
1202:
1194:
1190:
1186:
1182:
1177:
1172:
1168:
1164:
1160:
1153:
1145:
1138:
1130:
1126:
1121:
1116:
1111:
1106:
1103:(8): 1332–6.
1102:
1098:
1094:
1087:
1080:
1076:
1073:
1069:
1061:
1057:
1052:
1047:
1043:
1039:
1035:
1028:
1026:
1017:
1011:
1006:
1005:
998:Table 4-7 in:
995:
993:
991:
989:
987:
985:
983:
981:
979:
970:
966:
962:
961:
952:
944:
940:
936:
932:
929:(4): 863–95.
928:
924:
917:
909:
905:
900:
895:
892:(6): 425–32.
891:
887:
883:
876:
870:
869:
864:
859:
855:
845:
842:
840:
837:
835:
832:
831:
825:
822:
818:
816:
812:
802:
799:
796:
793:
790:
785:
782:
777:
775:
770:
767:
765:
760:
757:
755:
750:
742:
734:
730:
726:
717:
713:
710:
703:
698:
696:
692:
686:
682:
676:
671:
663:
661:
657:
648:
646:
636:
633:
632:
623:
612:
610:
606:
602:
600:
595:
589:
588:H&E stain
585:
581:
577:
573:
570:
562:
559:
556:
553:
552:
544:
541:
539:1 in 40,000)
534:
532:
528:
527:
523:
519:
514:
510:
507:
504:
500:
497:
494:
490:
487:
484:
480:
477:
474:
471:
470:
466:
463:
460:
457:
455:
451:
450:
446:
443:
440:
437:
435:body habitus
434:
431:
430:
426:
423:
420:
417:
415:
412:
411:
407:
404:
401:
398:
396:
393:
392:
388:
385:
382:
379:
376:
373:
372:
368:
365:
362:
359:
357:
354:
353:
349:
346:
343:
340:
338:
335:
334:
330:
327:
324:
321:
319:
316:
315:
311:
308:
305:
300:
295:
290:
285:
282:
279:
276:
275:
272:
269:
267:
264:
262:
259:
257:
254:
252:
249:
248:
244:
241:
238:
235:
233:
230:
229:
226:
223:
221:
218:
216:
213:
212:
209:
204:
197:
194:
186:
184:
180:
176:
172:
157:
155:
150:
148:
144:
140:
137:(abbreviated
136:
135:
124:
120:
115:
109:
108:Endocrinology
106:
104:
100:
96:
91:
87:
83:
78:
66:
63:
55:
45:
39:
37:
30:
21:
20:
2912:
2885:Pineal gland
2751:Hurthle cell
2736:
2731:
2690:Prolactinoma
2311:Hidrocystoma
2305:
2231:HĂĽrthle cell
2216:
2212:Prolactinoma
2117:
2102:
1883:
1800:
1776:
1768:
1753:
1738:
1687:
1683:
1677:
1645:(6): 433–9.
1642:
1639:J Intern Med
1638:
1628:
1614:cite journal
1573:
1569:
1562:
1551:. Retrieved
1533:
1521:. Retrieved
1517:
1508:
1497:. Retrieved
1479:
1468:. Retrieved
1450:
1438:. Retrieved
1434:
1406:cite journal
1361:
1357:
1347:
1332:
1307:
1303:
1296:
1279:
1275:
1269:
1244:
1240:
1234:
1223:
1207:
1201:
1166:
1162:
1152:
1143:
1137:
1100:
1096:
1086:
1068:
1041:
1037:
1003:
959:
951:
926:
922:
916:
889:
885:
875:
866:
858:
823:
819:
815:adenomatosis
808:
800:
797:
794:
788:
786:
780:
778:
773:
771:
768:
763:
761:
758:
753:
751:
748:
740:
731:
727:
723:
714:
708:
707:
701:
690:
680:
674:
672:
669:
655:
654:
644:
642:
629:
627:
621:
603:
598:
592:
571:
568:
542:1 in 40,000
535:1 in 35,000
377:hyperplasia
337:Angiofibroma
192:
168:
160:Presentation
151:
138:
131:
130:
58:
49:
42:Please help
33:
2903:Pineocytoma
2857:Parathyroid
2710:Pituicytoma
2636:Glucagonoma
2353:Cystadenoma
2306:sweat gland
2297:Adnexal and
2128:Glucagonoma
1802:MedlinePlus
1222:- Chapter:
1120:10183/40059
960:GeneReviews
805:Terminology
375:Parathyroid
299:glucagonoma
156:disorders.
85:Other names
2946:Categories
2770:Anaplastic
2747:Follicular
2663:Gastrinoma
2645:Insulinoma
2336:and serous
2279:Cylindroma
2264:Oncocytoma
2176:Urogenital
2133:Gastrinoma
2123:Insulinoma
1979:Epithelium
1553:2009-09-01
1499:2009-09-11
1470:2009-09-01
1310:(1): 1–7.
1276:Am. J. Med
1241:Am. J. Med
850:References
645:Drosophila
580:Micrograph
531:prevalence
289:insulinoma
284:gastrinoma
278:Pancreatic
189:Comparison
145:featuring
52:March 2019
2893:Pinealoma
2875:Carcinoma
2763:Medullary
2742:Papillary
2737:carcinoma
2678:Pituitary
2334:mucinous,
2316:Syringoma
2252:Carcinoid
2204:Endocrine
1992:carcinoma
1988:Papilloma
1921:Metageria
1916:Acrogeria
1523:11 August
1282:: 163–6.
691:(1P, 2Ms)
681:(2Ps, 1M)
601:in 1968.
433:Marfanoid
143:syndromes
134:neoplasia
103:Specialty
2775:Lymphoma
2614:Pancreas
2452:lobular,
2367:Mucinous
2118:pancreas
2081:Adenomas
1906:Progeria
1773:: 8360/1
1712:31430510
1704:15644784
1669:23149408
1548:21249756
1494:21249755
1465:21249755
1398:17030811
1339:(OMIM):
1261:13138607
1193:22402472
1185:21552134
1129:19169490
1075:Archived
1060:15531478
969:25719192
943:18672144
828:See also
811:adenomas
689:MEN IIb
679:MEN IIa
666:Mnemonic
529:Approx.
452:Mucosal
421:>33%
199:Feature
2870:Adenoma
2830:Medulla
2784:Benign
2720:Thyroid
2450:Ductal,
2346:general
2332:Cystic,
2274:Apudoma
2053:Thymoma
1783:D009377
1661:9681840
1606:4349714
1598:8099202
1578:Bibcode
1389:1622862
1366:Bibcode
1324:4968712
908:9681839
745:History
454:neuroma
291:(20%),
286:(50%),
280:tumors
245:(none)
2813:Cortex
2668:VIPoma
2551:Acinar
2415:Serous
2344:Cystic
2138:VIPoma
2103:tract:
2072:Glands
1971:cancer
1807:000398
1749:E31.20
1710:
1702:
1667:
1659:
1604:
1596:
1570:Nature
1546:
1492:
1463:
1440:4 July
1396:
1386:
1322:
1259:
1214:
1191:
1183:
1127:
1058:
1012:
967:
941:
906:
789:et al.
781:et al.
774:et al.
764:et al.
754:et al.
675:(3 Ps)
673:MEN I
609:CDKN1B
599:et al.
594:MEN 2B
522:191315
513:164761
503:164761
493:164761
483:613733
356:Lipoma
303:PPoma
294:VIPoma
271:155240
266:162300
261:171400
256:131100
232:Eponym
220:MEN 2B
215:MEN 2A
122:Causes
110:
1770:ICD-O
1764:258.0
1708:S2CID
1665:S2CID
1602:S2CID
1189:S2CID
582:of a
563:1965
560:1961
557:1954
518:NTRK1
464:100%
408:100%
402:100%
360:17%*
341:64%*
208:MEN 2
203:MEN 1
181:is a
2553:cell
1872:APS2
1867:APS1
1778:MeSH
1759:9-CM
1700:PMID
1657:PMID
1620:link
1594:PMID
1544:PMID
1525:2021
1490:PMID
1461:PMID
1442:2021
1412:link
1394:PMID
1320:PMID
1257:PMID
1212:ISBN
1181:PMID
1125:PMID
1056:PMID
1010:ISBN
965:PMID
939:PMID
904:PMID
709:MEN1
702:MEN1
656:MEN1
631:MEN1
628:The
624:gene
622:MEN1
620:The
605:OMIM
479:MEN1
475:(s)
473:Gene
444:80%
424:50%
405:85%
383:50%
380:90%
322:66%
251:OMIM
225:FMTC
173:and
2913:MEN
1755:ICD
1740:ICD
1692:doi
1647:doi
1643:243
1586:doi
1574:363
1384:PMC
1374:doi
1362:103
1312:doi
1308:279
1284:doi
1249:doi
1171:doi
1115:hdl
1105:doi
1046:doi
931:doi
894:doi
890:243
865:at
509:RET
499:RET
489:RET
139:MEN
88:MEN
2948::
2931:2B
2926:2A
2688::
2661::
2652::
2643::
2634::
2488:/
2434:/
2430:/
2426:/
2390:/
1899:2B
1894:2A
1805::
1781::
1762::
1747::
1744:10
1706:.
1698:.
1688:29
1686:.
1663:.
1655:.
1641:.
1637:.
1616:}}
1612:{{
1600:.
1592:.
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1280:31
1278:.
1255:.
1245:16
1243:.
1187:.
1179:.
1167:13
1165:.
1161:.
1123:.
1113:.
1101:52
1099:.
1095:.
1054:.
1042:89
1040:.
1036:.
1024:^
977:^
937:.
927:88
925:.
902:.
888:.
884:.
683:-
524:)
520:(
515:),
511:(
505:)
501:(
495:)
491:(
485:)
481:(
467:-
461:-
458:-
447:-
441:-
438:-
427:-
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399:-
389:-
386:-
369:-
366:-
363:-
350:-
347:-
344:-
331:-
328:-
325:-
312:-
309:-
306:-
301:,
296:,
2921:1
2749:/
2659:G
2650:δ
2641:β
2632:α
2598:e
2591:t
2584:v
2355:/
2224:/
2164:/
2083:/
1990:/
1962:e
1955:t
1948:v
1889:1
1842:e
1835:t
1828:v
1757:-
1742:-
1732:D
1714:.
1694::
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1376::
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1117::
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1018:.
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933::
910:.
896::
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59:(
54:)
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38:.
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