52:
181:
The renal cell carcinoma tends to be of the papillary (type 2) form and tends to occur more commonly in women than men with this syndrome. These cancers present earlier than is usual for renal cell carcinomas (typically in the twenties and thirties) and tend to be at relatively advanced stages at
928:
Marque M, Gardie B, Bressac de
Paillerets B, Rustin P, Guillot B, Richard S, et al. (December 2010). "Novel FH mutation in a patient with cutaneous leiomyomatosis associated with cutis verticis gyrata, eruptive collagenoma and Charcot-Marie-Tooth disease".
134:
The uterine fibroids tend to occur at younger age and are larger and more numerous than in the general population. They may be distinguishable from sporadic fibroids by special histological features such as prominent nucleoli with perinucleolar halos.
457:
Leiomyomas do not typically require treatment unless they cause pain. The skin lesions may be difficult to treat as they tend to recur after excision or destructive treatment. Drugs which affect smooth muscle contraction, such as
502:
A 2006 review stated that Reed's
Syndrome often is the leading cause of renal cancer between ages 30–50. Renal cancer kills about 1 in 3 people, but 5-year survival rates improved between 1974–1976 and 1995–2000, from 52% to 64%.
1375:
Tomlinson IP, Alam NA, Rowan AJ, Barclay E, Jaeger EE, Kelsell D, et al. (April 2002). "Germline mutations in FH predispose to dominantly inherited uterine fibroids, skin leiomyomata and papillary renal cell cancer".
291:
which are thought to predispose to tumorigenesis. An alternative pathway for the metabolism of fumarate in the presence of these mutations has been described. Other genes involved affected by this mutation are
666:
Adam MP, Feldman J, Mirzaa GM, Pagon RA, Wallace SE, Bean LJ, et al. (2010) . Pagon RA, Adam MP, Bird TD, Dolan CR, Fong CT, Stephens K (eds.). "Hereditary
Leiomyomatosis and Renal Cell Cancer".
692:
Garg K, Tickoo SK, Soslow RA, Reuter VE (August 2011). "Morphologic features of uterine leiomyomas associated with hereditary leiomyomatosis and renal cell carcinoma syndrome: a case report".
2201:
201:
Other relatively rare conditions have been reported in association with this disease. It is not yet known if these associations are fortuitous or manifestations of the condition itself.
1134:
Frezza C, Zheng L, Folger O, Rajagopalan KN, MacKenzie ED, Jerby L, et al. (August 2011). "Haem oxygenase is synthetically lethal with the tumour suppressor fumarate hydratase".
182:
presentation. Tumours have rarely been reported in children. These tumours occur in ~20% of those with this mutation suggesting that other factors are involved in the pathogenesis.
889:"Hereditary leiomyomatosis associated with bilateral, massive, macronodular adrenocortical disease and atypical cushing syndrome: a clinical and molecular genetic investigation"
2754:
220:
in association with a mutation in the fumarate hydratase gene has also been reported. Two cases of ovarian mucinous cystadenoma have also been reported with this mutation.
267:
While the pathogenetic mechanisms underlying the lesions remain unclear, it has been suggested that the accumulation of fumarate may lead to overexpression of the
1565:
62:
showing the characteristic hyalinized papillary cores found in some hereditary leiomyomatosis and renal cell carcinoma syndrome-associated renal cell carcinomas.
2194:
51:
484:. Substantially elevated risk of progression to or independent development of uterine leiomyosarcoma has been reported which may influence treatment methods.
142:), although they may occur anywhere, including the face. The lesions, which are typically painful and most often present during the third decade of life, are
1421:"The FH mutation database: an online database of fumarate hydratase mutations involved in the MCUL (HLRCC) tumor syndrome and congenital fumarase deficiency"
338:
separating the tumour from the overlying skin. Histological examination shows dense dermal nodules composed of elongated cells with abundant eosinophilic
283:. The cytoplasmic form appears to have a role in the protection of DNA from molecular injury. Fumarate has been shown to be a competitive inhibitor of
1533:
531:
A database of the mutations of the fumarate hydratase gene is available. Autosomal recessive mutations cause a serious neurological disease known as
2865:
2187:
1236:"Treatment of cutaneous leiomyomas with 5% lidocaine patches in a patient with hereditary leiomyomatosis and renal cell cancer (Reed syndrome)"
848:"Cerebral cavernomas in a family with multiple cutaneous and uterine leiomyomas associated with a new mutation in the fumarate hydratase gene"
2555:
2402:
217:
2240:
2040:
1489:
386:
138:
The skin presentation is of asymmetrical, reddish-brown nodules or papules with a firm consistency, predominantly located on the limbs (
1609:
1558:
171:
2870:
2317:
185:
These growths increase in size and number throughout the lifetime. People with HLRCC have an approximately 15% chance of developing
1537:
103:, an association denominated hereditary leiomyomatosis and renal cell cancer. It is also associated with increased risk of uterine
1187:"Succination of Keap1 and activation of Nrf2-dependent antioxidant pathways in FH-deficient papillary renal cell carcinoma type 2"
494:
Topical lidocaine patches have been reported to decrease the severity and frequency of pain associated with cutaneous leiomyomas.
2806:
2090:
2890:
2885:
2574:
2285:
2094:
1927:
1016:
393:. As the skin lesions are typically painful, it is also often necessary to exclude other painful tumors of the skin (including
2350:
2332:
1715:
769:
612:"Mutations in the fumarate hydratase gene cause hereditary leiomyomatosis and renal cell cancer in families in North America"
544:
190:
2448:
2355:
2032:
1551:
2749:
2739:
2460:
1821:
139:
92:
2397:
2079:
1996:
1629:
208:
and massive, macronodular adrenocortical disease have also been reported in association with this syndrome. A case of
2677:
2255:
2136:
2118:
2028:
1979:
2801:
2689:
2470:
2113:
2036:
1873:
1699:
1662:
480:
can be treated with the same methods as sporadic uterine fibroids including anti-hormonal treatment, surgery, or
288:
1044:"An antioxidant response phenotype shared between hereditary and sporadic type 2 papillary renal cell carcinoma"
2340:
2141:
2074:
2009:
1883:
1863:
1830:
1808:
1770:
1503:
17:
2786:
2875:
2768:
2526:
2370:
2312:
2264:
1619:
549:
2880:
2604:
2455:
2412:
2407:
2002:
1826:
1766:
394:
1085:"Fumarase: a mitochondrial metabolic enzyme and a cytosolic/nuclear component of the DNA damage response"
433:; the mnemonic "BLEND-AN-EGG" may be helpful). Other skin lesions that may need to be considered include
2164:
1888:
799:"Hereditary leiomyomatosis and renal cell cancer (HLRCC): renal cancer risk, surveillance and treatment"
209:
1083:
Yogev O, Yogev O, Singer E, Shaulian E, Goldberg M, Fox TD, et al. (March 2010). Elledge S (ed.).
359:
355:
972:
Ylisaukko-oja SK, Cybulski C, Lehtonen R, Kiuru M, Matyjasik J, Szymañska A, et al. (July 2006).
887:
Matyakhina L, Freedman RJ, Bourdeau I, Wei MH, Stergiopoulos SG, Chidakel A, et al. (June 2005).
2791:
2636:
2360:
2290:
1992:
1851:
1788:
1624:
1604:
1143:
797:
Menko FH, Maher ER, Schmidt LS, Middelton LA, Aittomäki K, Tomlinson I, et al. (December 2014).
363:
320:. Special histologic features of fibroids may allow an early diagnosis in absence of other symptoms.
186:
143:
88:
846:
Campione E, Terrinoni A, Orlandi A, Codispoti A, Melino G, Bianchi L, et al. (September 2007).
2781:
2707:
2589:
2389:
2365:
2327:
2322:
2235:
2128:
1780:
1614:
532:
523:
HLRCC affects males and females equally. Thus far, HLRCC has been found in 300 families worldwide.
430:
402:
268:
2744:
2719:
2682:
2579:
2548:
2512:
2345:
1961:
1793:
1756:
1634:
1401:
1357:
1308:
1167:
1042:
Ooi A, Wong JC, Petillo D, Roossien D, Perrier-Trudova V, Whitten D, et al. (October 2011).
954:
735:
Kogan EA, MikhaÄlov OI, Sekamova SM, Kornev BM, Osipova IN, Osipenko VI, et al. (2001). "".
717:
390:
284:
233:
229:
151:
108:
1652:
571:"Strong family history of uterine leiomyomatosis warrants fumarate hydratase mutation screening"
333:
2844:
2816:
2724:
2584:
2375:
2210:
2105:
1514:
1452:
1393:
1349:
1300:
1265:
1216:
1159:
1116:
1065:
995:
946:
910:
869:
828:
744:
709:
671:
641:
592:
446:
434:
237:
163:
100:
2179:
336:
2673:
2569:
2507:
2443:
2066:
1984:
1798:
1710:
1442:
1432:
1385:
1339:
1292:
1255:
1247:
1206:
1198:
1151:
1106:
1096:
1055:
985:
938:
900:
859:
818:
810:
701:
631:
623:
582:
471:
312:
The diagnosis is made either by testing of the fumarate hydratase activity in cultured skin
116:
2660:
2488:
2084:
1747:
477:
317:
128:
96:
2796:
1835:
1147:
610:
Toro JR, Nickerson ML, Wei MH, Warren MB, Glenn GM, Turner ML, et al. (July 2003).
2821:
2811:
2729:
2714:
2649:
2560:
2498:
2478:
1761:
1690:
1447:
1420:
1260:
1235:
1211:
1186:
1111:
1084:
823:
798:
636:
611:
463:
410:
343:
256:
167:
104:
2859:
974:"Germline fumarate hydratase mutations in patients with ovarian mucinous cystadenoma"
942:
276:
155:
147:
84:
1361:
1312:
958:
2839:
2483:
2385:
2304:
2277:
2245:
2019:
1971:
1915:
1816:
1405:
1283:
Laber DA (2006). "Risk factors, classification, and staging of renal cell cancer".
1171:
721:
481:
426:
422:
418:
367:
347:
329:
248:
112:
1543:
316:
or lymphoblastoid cells and demonstrating reduced activity (≤60%) or by molecular
63:
1508:
1101:
705:
2831:
2669:
2543:
2538:
2438:
2214:
1957:
1732:
1644:
414:
213:
175:
1519:
1251:
487:
The predisposition to renal cell cancer calls for screening and, if necessary,
2776:
2625:
1868:
1737:
1727:
1583:
1296:
1202:
1060:
1043:
814:
467:
313:
59:
1344:
1327:
131:, approximately 76% with dermal manifestations and 10–16% with renal tumors.
2702:
2645:
2594:
1920:
1856:
1596:
1592:
990:
973:
587:
570:
488:
459:
398:
374:
339:
280:
205:
1456:
1437:
1397:
1304:
1269:
1220:
1163:
1120:
1069:
999:
950:
914:
873:
864:
847:
832:
748:
713:
675:
645:
596:
449:; these tend to be painless and have other useful distinguishing features.
1353:
515:
in 1973. The link with the fumarate hydratase gene was uncovered in 2002.
2217:
905:
888:
569:
Tolvanen J, Uimari O, Ryynänen M, Aaltonen LA, Vahteristo P (June 2012).
535:, which is associated with a variety of congenital lesions in the brain.
241:
1481:
1155:
2300:
2273:
2221:
1878:
1685:
1657:
406:
351:
272:
159:
2534:
2434:
2426:
2155:
1948:
1742:
1575:
442:
438:
627:
2621:
1676:
1389:
301:
293:
1497:
1494:
297:
252:
2183:
1547:
350:
are uniform, blunt-ended and cigar-shaped with only occasional
328:
The skin lesions may be difficult to diagnose clinically but a
354:. Special stains that may be of use in the diagnosis include
287:. This inhibition leads to the stabilisation of a number of
1234:
Hsu T, Cornelius LA, Rosman IS, Nemer KM (September 2017).
83:
is rare autosomal dominant disorder associated with benign
1540:
at the
Genetic and Rare Diseases Information Center (GARD)
247:
The fumarate hydratase gene is located on the long arm of
1017:"Hereditary leiomyomatosis and renal cell cancer (HLRCC)"
275:. It has also been found that fumarate is present in the
154:
muscles of the skin. These tumours may also arise in the
2735:
Multiple cutaneous and uterine leiomyomatosis syndrome
385:
Differential diagnosis of this condition includes the
373:
The renal cell carcinomas have prominent eosinophilic
1471:
893:
The
Journal of Clinical Endocrinology and Metabolism
770:"Hereditary Leiomyomatosis and Renal Cell Carcinoma"
2830:
2767:
2658:
2634:
2612:
2603:
2525:
2497:
2469:
2425:
2384:
2299:
2272:
2263:
2254:
2228:
2154:
2127:
2104:
2065:
2054:
2018:
1970:
1947:
1936:
1901:
1844:
1807:
1779:
1698:
1684:
1675:
1643:
1591:
1582:
1475:
39:
34:
1419:Bayley JP, Launonen V, Tomlinson IP (March 2008).
1185:Kinch L, Grishin NV, Brugarolas J (October 2011).
661:
659:
657:
655:
73:Hereditary leiomyomatosis and renal cell carcinoma
1328:"Cutaneous leiomyomata with uterine leiomyomata"
119:and screening can typically begin in childhood.
1538:Hereditary leiomyomatosis and renal cell cancer
774:NORD (National Organization for Rare Disorders)
18:Hereditary leiomyomatosis and renal cell cancer
107:. The syndrome is caused by a mutation in the
2195:
1559:
8:
687:
685:
251:(1q42.3-43), spans 22 kilobases and has 10
2609:
2269:
2260:
2202:
2188:
2180:
2062:
1944:
1695:
1681:
1588:
1566:
1552:
1544:
1472:
694:The American Journal of Surgical Pathology
236:gene, which results in dysfunction of the
50:
31:
1446:
1436:
1343:
1259:
1210:
1110:
1100:
1059:
989:
904:
863:
822:
635:
586:
511:The syndrome was first described by Reed
189:in their lifetime. This is most commonly
162:and the mammillary muscle of the nipple (
852:The Journal of Investigative Dermatology
111:gene, which leads to an accumulation of
792:
790:
561:
166:), the smooth muscle of blood vessels (
1326:Reed WB, Walker R, Horowitz R (1973).
1011:
1009:
764:
762:
760:
758:
232:condition caused by a mutation in the
191:type II papillary renal cell carcinoma
44:Familial leiomyomatosis cutis et uteri
7:
2241:Desmoplastic small-round-cell tumor
2041:Papillary serous cystadenocarcinoma
1023:. U.S. National Library of Medicine
1610:Combined small-cell lung carcinoma
978:European Journal of Human Genetics
931:The British Journal of Dermatology
616:American Journal of Human Genetics
172:pulmonary lymphangioleiomyomatosis
25:
2318:Aggressive infantile fibromatosis
2807:Clear-cell sarcoma of the kidney
943:10.1111/j.1365-2133.2010.09912.x
240:, leading to an accumulation of
99:. It predisposes individuals to
87:tumors and an increased risk of
2866:Dermal and subcutaneous growths
2575:Intradermal spindle cell lipoma
2286:Dermatofibrosarcoma protuberans
1928:Syringocystadenoma papilliferum
193:, which is an aggressive form.
2403:Malignant fibrous histiocytoma
2351:Infantile digital fibromatosis
2337:Familial myxovascular fibromas
2333:Diffuse infantile fibromatosis
1716:Familial adenomatous polyposis
127:Almost all women present with
1:
2449:Superficial acral fibromyxoma
2356:Juvenile hyaline fibromatosis
2091:Paget's disease of the breast
2033:Pancreatic serous cystadenoma
255:; the first exon codes for a
115:. The inheritance pattern is
93:multiple cutaneous leiomyomas
2750:Solitary cutaneous leiomyoma
2740:Multiple cutaneous leiomyoma
2461:Ossifying fibromyxoid tumour
2095:Extramammary Paget's disease
1822:Multiple endocrine neoplasia
1102:10.1371/journal.pbio.1000328
706:10.1097/PAS.0b013e318223ca01
377:surrounded by a clear halo.
140:multiple cutaneous leiomyoma
2398:Benign fibrous histiocytoma
2080:Pancreatic ductal carcinoma
1997:Mucinous cystadenocarcinoma
1630:Transitional cell carcinoma
474:, may provide pain relief.
218:Charcot–Marie–Tooth disease
2907:
2678:Embryonal rhabdomyosarcoma
2256:Connective tissue neoplasm
2137:Medullary breast carcinoma
2119:Invasive lobular carcinoma
2029:Ovarian serous cystadenoma
1980:Signet ring cell carcinoma
1332:Acta Dermato-Venereologica
1252:10.1016/j.jdcr.2017.06.007
2802:Malignant rhabdoid tumour
2769:Complex mixed and stromal
2698:
2690:Alveolar rhabdomyosarcoma
2114:Lobular carcinoma in situ
2037:Serous cystadenocarcinoma
1874:Clear-cell adenocarcinoma
1663:Bartholin gland carcinoma
1574:Glandular and epithelial
1203:10.1016/j.ccr.2011.10.005
1061:10.1016/j.ccr.2011.08.024
815:10.1007/s10689-014-9735-2
545:Von Hippel–Lindau disease
289:hypoxia-inducible factors
91:. It is characterised by
58:
49:
2871:Gynaecological neoplasia
2341:Fibroma of tendon sheath
2142:Medullary thyroid cancer
2075:Mammary ductal carcinoma
2010:Mucoepidermoid carcinoma
1864:Adenoid cystic carcinoma
1831:Adrenocortical carcinoma
1771:Hepatocellular carcinoma
1345:10.2340/0001555553409416
150:tumour arising from the
2371:Oral submucous fibrosis
2313:Aggressive fibromatosis
2229:Not otherwise specified
1620:Squamous-cell carcinoma
1021:Genetics Home Reference
991:10.1038/sj.ejhg.5201630
550:Birt-Hogg-Dube syndrome
387:Birt–Hogg–Dubé syndrome
2891:Gynaecologic disorders
2886:Rare genetic syndromes
2413:Solitary fibrous tumor
2408:Atypical fibroxanthoma
2003:Pseudomyxoma peritonei
1827:Adrenocortical adenoma
1767:Hepatocellular adenoma
1438:10.1186/1471-2350-9-20
865:10.1038/sj.jid.5700851
395:blue rubber bleb nevus
381:Differential diagnosis
332:will usually reveal a
2787:Mixed MĂĽllerian tumor
2165:Acinic cell carcinoma
1889:Papillary hidradenoma
588:10.1093/humrep/des105
346:(spindle cells). The
210:cutis verticis gyrata
197:Associated conditions
2792:Mesoblastic nephroma
2361:Plantar fibromatosis
2291:Desmoplastic fibroma
1993:Mucinous cystadenoma
1852:Neuroendocrine tumor
1789:Renal cell carcinoma
1625:Basal-cell carcinoma
1605:Small-cell carcinoma
1425:BMC Medical Genetics
906:10.1210/jc.2004-2377
364:phosphotungstic acid
187:renal cell carcinoma
89:renal cell carcinoma
2782:Pleomorphic adenoma
2590:Spindle cell lipoma
2390:histiocytic sarcoma
2366:Pleomorphic fibroma
2328:Collagenous fibroma
2323:Aponeurotic fibroma
2236:Soft-tissue sarcoma
2129:Medullary carcinoma
1615:Verrucous carcinoma
1297:10.1385/MO:23:4:443
1156:10.1038/nature10363
1148:2011Natur.477..225F
533:fumarase deficiency
431:granular cell tumor
403:eccrine spiradenoma
269:aldo-keto reductase
2745:Neural fibrolipoma
2720:Angiolipoleiomyoma
2683:Sarcoma botryoides
2580:Pleomorphic lipoma
2549:Myxoid liposarcoma
2513:Clear-cell sarcoma
2346:Fibromatosis colli
1962:Cystadenocarcinoma
1794:Endometrioid tumor
1757:Cholangiocarcinoma
1645:Complex epithelial
1635:Inverted papilloma
575:Human Reproduction
391:tuberous sclerosis
360:Van Gieson's stain
356:Masson's trichrome
285:prolyl hydroxylase
230:autosomal dominant
152:arrectores pilorum
123:Signs and symptoms
117:autosomal dominant
109:fumarate hydratase
97:uterine leiomyomas
2853:
2852:
2845:Adenomatoid tumor
2817:Pancreatoblastoma
2763:
2762:
2725:Genital leiomyoma
2585:Lipoblastomatosis
2521:
2520:
2421:
2420:
2376:Pachydermodactyly
2177:
2176:
2173:
2172:
2150:
2149:
2106:Lobular carcinoma
2050:
2049:
1897:
1896:
1671:
1670:
1529:
1528:
1240:JAAD Case Reports
1142:(7363): 225–228.
447:trichoepithelioma
238:citric acid cycle
164:genital leiomyoma
101:renal cell cancer
70:
69:
29:Medical condition
16:(Redirected from
2898:
2674:rhabdomyosarcoma
2663:
2639:
2615:
2610:
2570:Chondroid lipoma
2508:Synovial sarcoma
2444:Cutaneous myxoma
2270:
2261:
2204:
2197:
2190:
2181:
2097:
2067:Ductal carcinoma
2063:
2043:
1999:
1985:Krukenberg tumor
1945:
1799:Renal oncocytoma
1711:Linitis plastica
1700:Gastrointestinal
1696:
1682:
1589:
1568:
1561:
1554:
1545:
1473:
1461:
1460:
1450:
1440:
1416:
1410:
1409:
1372:
1366:
1365:
1347:
1323:
1317:
1316:
1285:Medical Oncology
1280:
1274:
1273:
1263:
1231:
1225:
1224:
1214:
1182:
1176:
1175:
1131:
1125:
1124:
1114:
1104:
1080:
1074:
1073:
1063:
1039:
1033:
1032:
1030:
1028:
1013:
1004:
1003:
993:
969:
963:
962:
937:(6): 1337–1339.
925:
919:
918:
908:
899:(6): 3773–3779.
884:
878:
877:
867:
858:(9): 2271–2273.
843:
837:
836:
826:
794:
785:
784:
782:
780:
766:
753:
752:
737:Arkhiv Patologii
732:
726:
725:
700:(8): 1235–1237.
689:
680:
679:
663:
650:
649:
639:
607:
601:
600:
590:
581:(6): 1865–1869.
566:
478:Uterine fibroids
472:phenoxybenzamine
178:may be present.
170:) and the lung (
129:uterine fibroids
54:
32:
21:
2906:
2905:
2901:
2900:
2899:
2897:
2896:
2895:
2856:
2855:
2854:
2849:
2826:
2759:
2694:
2661:Skeletal muscle
2659:
2654:
2635:
2630:
2613:
2599:
2517:
2493:
2489:Phyllodes tumor
2471:Fibroepithelial
2465:
2417:
2380:
2295:
2250:
2224:
2208:
2178:
2169:
2146:
2123:
2100:
2089:
2085:Comedocarcinoma
2058:
2056:
2046:
2027:
2014:
1991:
1966:
1940:
1938:
1932:
1903:
1893:
1840:
1803:
1775:
1748:Somatostatinoma
1691:adenocarcinomas
1689:
1667:
1653:Warthin's tumor
1639:
1578:
1572:
1530:
1525:
1524:
1484:
1470:
1465:
1464:
1418:
1417:
1413:
1378:Nature Genetics
1374:
1373:
1369:
1325:
1324:
1320:
1282:
1281:
1277:
1233:
1232:
1228:
1184:
1183:
1179:
1133:
1132:
1128:
1095:(3): e1000328.
1082:
1081:
1077:
1041:
1040:
1036:
1026:
1024:
1015:
1014:
1007:
971:
970:
966:
927:
926:
922:
886:
885:
881:
845:
844:
840:
803:Familial Cancer
796:
795:
788:
778:
776:
768:
767:
756:
734:
733:
729:
691:
690:
683:
665:
664:
653:
609:
608:
604:
568:
567:
563:
558:
541:
529:
521:
509:
500:
455:
383:
326:
318:genetic testing
310:
265:
226:
212:, disseminated
199:
144:piloleiomyomata
125:
95:and, in women,
81:Reed's syndrome
35:Reed’s syndrome
30:
23:
22:
15:
12:
11:
5:
2904:
2902:
2894:
2893:
2888:
2883:
2878:
2876:Uterine tumour
2873:
2868:
2858:
2857:
2851:
2850:
2848:
2847:
2842:
2836:
2834:
2828:
2827:
2825:
2824:
2822:Carcinosarcoma
2819:
2814:
2812:Hepatoblastoma
2809:
2804:
2799:
2794:
2789:
2784:
2779:
2773:
2771:
2765:
2764:
2761:
2760:
2758:
2757:
2752:
2747:
2742:
2737:
2732:
2730:Leiomyosarcoma
2727:
2722:
2717:
2715:Angioleiomyoma
2712:
2711:
2710:
2699:
2696:
2695:
2693:
2692:
2687:
2686:
2685:
2666:
2664:
2656:
2655:
2653:
2652:
2650:leiomyosarcoma
2642:
2640:
2632:
2631:
2629:
2628:
2618:
2616:
2607:
2601:
2600:
2598:
2597:
2592:
2587:
2582:
2577:
2572:
2566:
2565:
2564:
2563:
2561:Angiomyolipoma
2553:
2552:
2551:
2546:
2531:
2529:
2523:
2522:
2519:
2518:
2516:
2515:
2510:
2504:
2502:
2495:
2494:
2492:
2491:
2486:
2481:
2479:Brenner tumour
2475:
2473:
2467:
2466:
2464:
2463:
2458:
2453:
2452:
2451:
2446:
2431:
2429:
2423:
2422:
2419:
2418:
2416:
2415:
2410:
2405:
2400:
2394:
2392:
2382:
2381:
2379:
2378:
2373:
2368:
2363:
2358:
2353:
2348:
2343:
2338:
2335:
2330:
2325:
2320:
2315:
2309:
2307:
2297:
2296:
2294:
2293:
2288:
2282:
2280:
2267:
2258:
2252:
2251:
2249:
2248:
2243:
2238:
2232:
2230:
2226:
2225:
2209:
2207:
2206:
2199:
2192:
2184:
2175:
2174:
2171:
2170:
2168:
2167:
2161:
2159:
2152:
2151:
2148:
2147:
2145:
2144:
2139:
2133:
2131:
2125:
2124:
2122:
2121:
2116:
2110:
2108:
2102:
2101:
2099:
2098:
2087:
2082:
2077:
2071:
2069:
2060:
2052:
2051:
2048:
2047:
2045:
2044:
2024:
2022:
2016:
2015:
2013:
2012:
2007:
2006:
2005:
1989:
1988:
1987:
1976:
1974:
1968:
1967:
1965:
1964:
1954:
1952:
1942:
1934:
1933:
1931:
1930:
1925:
1924:
1923:
1918:
1907:
1905:
1904:skin appendage
1899:
1898:
1895:
1894:
1892:
1891:
1886:
1881:
1876:
1871:
1866:
1861:
1860:
1859:
1848:
1846:
1845:Other/multiple
1842:
1841:
1839:
1838:
1833:
1824:
1819:
1813:
1811:
1805:
1804:
1802:
1801:
1796:
1791:
1785:
1783:
1777:
1776:
1774:
1773:
1764:
1762:Klatskin tumor
1759:
1753:
1752:
1751:
1750:
1745:
1740:
1735:
1730:
1719:
1718:
1713:
1704:
1702:
1693:
1679:
1673:
1672:
1669:
1668:
1666:
1665:
1660:
1655:
1649:
1647:
1641:
1640:
1638:
1637:
1632:
1627:
1622:
1617:
1612:
1607:
1601:
1599:
1586:
1580:
1579:
1573:
1571:
1570:
1563:
1556:
1548:
1542:
1541:
1527:
1526:
1523:
1522:
1511:
1500:
1485:
1480:
1479:
1477:
1476:Classification
1469:
1468:External links
1466:
1463:
1462:
1411:
1384:(4): 406–410.
1367:
1338:(5): 409–416.
1318:
1291:(4): 443–454.
1275:
1246:(5): 407–409.
1226:
1197:(4): 418–420.
1177:
1126:
1075:
1054:(4): 511–523.
1034:
1005:
984:(7): 880–883.
964:
920:
879:
838:
809:(4): 637–644.
786:
754:
727:
681:
651:
628:10.1086/376435
602:
560:
559:
557:
554:
553:
552:
547:
540:
537:
528:
525:
520:
517:
508:
505:
499:
496:
464:nitroglycerine
454:
451:
411:dermatofibroma
382:
379:
325:
322:
309:
306:
264:
261:
257:signal peptide
225:
222:
198:
195:
168:angioleiomyoma
124:
121:
105:leiomyosarcoma
68:
67:
56:
55:
47:
46:
41:
37:
36:
28:
24:
14:
13:
10:
9:
6:
4:
3:
2:
2903:
2892:
2889:
2887:
2884:
2882:
2881:Rare diseases
2879:
2877:
2874:
2872:
2869:
2867:
2864:
2863:
2861:
2846:
2843:
2841:
2838:
2837:
2835:
2833:
2829:
2823:
2820:
2818:
2815:
2813:
2810:
2808:
2805:
2803:
2800:
2798:
2795:
2793:
2790:
2788:
2785:
2783:
2780:
2778:
2775:
2774:
2772:
2770:
2766:
2756:
2753:
2751:
2748:
2746:
2743:
2741:
2738:
2736:
2733:
2731:
2728:
2726:
2723:
2721:
2718:
2716:
2713:
2709:
2706:
2705:
2704:
2701:
2700:
2697:
2691:
2688:
2684:
2681:
2680:
2679:
2675:
2671:
2668:
2667:
2665:
2662:
2657:
2651:
2647:
2644:
2643:
2641:
2638:
2637:Smooth muscle
2633:
2627:
2623:
2620:
2619:
2617:
2611:
2608:
2606:
2602:
2596:
2593:
2591:
2588:
2586:
2583:
2581:
2578:
2576:
2573:
2571:
2568:
2567:
2562:
2559:
2558:
2557:
2554:
2550:
2547:
2545:
2542:
2541:
2540:
2536:
2533:
2532:
2530:
2528:
2524:
2514:
2511:
2509:
2506:
2505:
2503:
2500:
2496:
2490:
2487:
2485:
2482:
2480:
2477:
2476:
2474:
2472:
2468:
2462:
2459:
2457:
2454:
2450:
2447:
2445:
2442:
2441:
2440:
2436:
2433:
2432:
2430:
2428:
2424:
2414:
2411:
2409:
2406:
2404:
2401:
2399:
2396:
2395:
2393:
2391:
2387:
2383:
2377:
2374:
2372:
2369:
2367:
2364:
2362:
2359:
2357:
2354:
2352:
2349:
2347:
2344:
2342:
2339:
2336:
2334:
2331:
2329:
2326:
2324:
2321:
2319:
2316:
2314:
2311:
2310:
2308:
2306:
2302:
2298:
2292:
2289:
2287:
2284:
2283:
2281:
2279:
2275:
2271:
2268:
2266:
2262:
2259:
2257:
2253:
2247:
2244:
2242:
2239:
2237:
2234:
2233:
2231:
2227:
2223:
2219:
2216:
2212:
2205:
2200:
2198:
2193:
2191:
2186:
2185:
2182:
2166:
2163:
2162:
2160:
2157:
2153:
2143:
2140:
2138:
2135:
2134:
2132:
2130:
2126:
2120:
2117:
2115:
2112:
2111:
2109:
2107:
2103:
2096:
2092:
2088:
2086:
2083:
2081:
2078:
2076:
2073:
2072:
2070:
2068:
2064:
2061:
2059:and medullary
2053:
2042:
2038:
2034:
2030:
2026:
2025:
2023:
2021:
2017:
2011:
2008:
2004:
2001:
2000:
1998:
1994:
1990:
1986:
1983:
1982:
1981:
1978:
1977:
1975:
1973:
1969:
1963:
1959:
1956:
1955:
1953:
1950:
1946:
1943:
1935:
1929:
1926:
1922:
1919:
1917:
1914:
1913:
1912:
1909:
1908:
1906:
1900:
1890:
1887:
1885:
1882:
1880:
1877:
1875:
1872:
1870:
1867:
1865:
1862:
1858:
1855:
1854:
1853:
1850:
1849:
1847:
1843:
1837:
1834:
1832:
1828:
1825:
1823:
1820:
1818:
1815:
1814:
1812:
1810:
1806:
1800:
1797:
1795:
1792:
1790:
1787:
1786:
1784:
1782:
1778:
1772:
1768:
1765:
1763:
1760:
1758:
1755:
1754:
1749:
1746:
1744:
1741:
1739:
1736:
1734:
1731:
1729:
1726:
1725:
1724:
1721:
1720:
1717:
1714:
1712:
1709:
1706:
1705:
1703:
1701:
1697:
1694:
1692:
1687:
1683:
1680:
1678:
1674:
1664:
1661:
1659:
1656:
1654:
1651:
1650:
1648:
1646:
1642:
1636:
1633:
1631:
1628:
1626:
1623:
1621:
1618:
1616:
1613:
1611:
1608:
1606:
1603:
1602:
1600:
1598:
1594:
1590:
1587:
1585:
1581:
1577:
1569:
1564:
1562:
1557:
1555:
1550:
1549:
1546:
1539:
1535:
1534:Reed syndrome
1532:
1531:
1521:
1517:
1516:
1512:
1510:
1506:
1505:
1501:
1499:
1496:
1492:
1491:
1487:
1486:
1483:
1478:
1474:
1467:
1458:
1454:
1449:
1444:
1439:
1434:
1430:
1426:
1422:
1415:
1412:
1407:
1403:
1399:
1395:
1391:
1390:10.1038/ng849
1387:
1383:
1379:
1371:
1368:
1363:
1359:
1355:
1351:
1346:
1341:
1337:
1333:
1329:
1322:
1319:
1314:
1310:
1306:
1302:
1298:
1294:
1290:
1286:
1279:
1276:
1271:
1267:
1262:
1257:
1253:
1249:
1245:
1241:
1237:
1230:
1227:
1222:
1218:
1213:
1208:
1204:
1200:
1196:
1192:
1188:
1181:
1178:
1173:
1169:
1165:
1161:
1157:
1153:
1149:
1145:
1141:
1137:
1130:
1127:
1122:
1118:
1113:
1108:
1103:
1098:
1094:
1090:
1086:
1079:
1076:
1071:
1067:
1062:
1057:
1053:
1049:
1045:
1038:
1035:
1022:
1018:
1012:
1010:
1006:
1001:
997:
992:
987:
983:
979:
975:
968:
965:
960:
956:
952:
948:
944:
940:
936:
932:
924:
921:
916:
912:
907:
902:
898:
894:
890:
883:
880:
875:
871:
866:
861:
857:
853:
849:
842:
839:
834:
830:
825:
820:
816:
812:
808:
804:
800:
793:
791:
787:
775:
771:
765:
763:
761:
759:
755:
750:
746:
742:
738:
731:
728:
723:
719:
715:
711:
707:
703:
699:
695:
688:
686:
682:
677:
673:
669:
662:
660:
658:
656:
652:
647:
643:
638:
633:
629:
625:
622:(1): 95–106.
621:
617:
613:
606:
603:
598:
594:
589:
584:
580:
576:
572:
565:
562:
555:
551:
548:
546:
543:
542:
538:
536:
534:
526:
524:
518:
516:
514:
506:
504:
497:
495:
492:
490:
485:
483:
479:
475:
473:
469:
465:
461:
452:
450:
448:
444:
440:
436:
432:
428:
424:
420:
416:
412:
408:
404:
400:
396:
392:
388:
380:
378:
376:
371:
369:
365:
361:
357:
353:
349:
345:
341:
337:
335:
331:
323:
321:
319:
315:
307:
305:
303:
299:
295:
290:
286:
282:
278:
274:
270:
262:
260:
258:
254:
250:
245:
243:
239:
235:
231:
223:
221:
219:
215:
211:
207:
202:
196:
194:
192:
188:
183:
179:
177:
173:
169:
165:
161:
157:
156:tunica dartos
153:
149:
148:smooth muscle
145:
141:
136:
132:
130:
122:
120:
118:
114:
110:
106:
102:
98:
94:
90:
86:
85:smooth muscle
82:
78:
74:
65:
64:H&E stain
61:
57:
53:
48:
45:
42:
38:
33:
27:
19:
2840:Mesothelioma
2797:Wilms' tumor
2734:
2484:Fibroadenoma
2386:Histiocytoma
2305:fibromatosis
2278:fibrosarcoma
2246:Skin sarcoma
1916:Hidrocystoma
1910:
1836:HĂĽrthle cell
1817:Prolactinoma
1722:
1707:
1513:
1502:
1488:
1428:
1424:
1414:
1381:
1377:
1370:
1335:
1331:
1321:
1288:
1284:
1278:
1243:
1239:
1229:
1194:
1190:
1180:
1139:
1135:
1129:
1092:
1089:PLOS Biology
1088:
1078:
1051:
1047:
1037:
1025:. Retrieved
1020:
981:
977:
967:
934:
930:
923:
896:
892:
882:
855:
851:
841:
806:
802:
777:. Retrieved
773:
743:(6): 39–44.
740:
736:
730:
697:
693:
667:
619:
615:
605:
578:
574:
564:
530:
522:
519:Epidemiology
512:
510:
501:
493:
491:management.
486:
482:embolisation
476:
456:
427:glomus tumor
423:endometrioma
419:neurilemmoma
384:
372:
368:haematoxylin
342:arranged in
330:punch biopsy
327:
311:
277:mitochondria
266:
263:Pathogenesis
249:chromosome 1
246:
228:HLRCC is an
227:
203:
200:
184:
180:
174:). A pseudo-
137:
133:
126:
80:
76:
72:
71:
43:
26:
2832:Mesothelial
2670:Rhabdomyoma
2544:Myelolipoma
2539:liposarcoma
2456:Angiomyxoma
2439:myxosarcoma
2265:Fibromatous
2215:soft tissue
1958:Cystadenoma
1911:sweat gland
1902:Adnexal and
1733:Glucagonoma
1191:Cancer Cell
1048:Cancer Cell
668:GeneReviews
415:angiolipoma
314:fibroblasts
279:and in the
214:collagenoma
176:Darier sign
40:Other names
2860:Categories
2777:Adenomyoma
2626:myosarcoma
2527:Lipomatous
2427:Myxomatous
2211:Connective
1941:and serous
1884:Cylindroma
1869:Oncocytoma
1781:Urogenital
1738:Gastrinoma
1728:Insulinoma
1584:Epithelium
1515:DiseasesDB
556:References
489:urological
468:nifedipine
435:cylindroma
334:Grenz zone
206:cavernomas
146:—a benign
60:Micrograph
2708:Cutaneous
2703:Leiomyoma
2646:Leiomyoma
2605:Myomatous
2595:Hibernoma
1939:mucinous,
1921:Syringoma
1857:Carcinoid
1809:Endocrine
1597:carcinoma
1593:Papilloma
498:Prognosis
460:doxazosin
453:Treatment
399:leiomyoma
344:fascicles
340:cytoplasm
324:Histology
308:Diagnosis
281:cytoplasm
204:Cerebral
2499:Synovial
2222:sarcomas
2057:lobular,
1972:Mucinous
1723:pancreas
1686:Adenomas
1457:18366737
1398:11865300
1362:34376892
1313:40025455
1305:17303902
1270:28884140
1221:22014567
1164:21849978
1121:20231875
1070:22014576
1000:16639410
959:32673334
951:20560959
915:15741255
874:17476294
833:25012257
749:11810925
714:21753700
676:20301430
646:12772087
597:22473397
539:See also
375:nucleoli
271:enzyme,
242:fumarate
113:fumarate
2614:General
2301:Fibroma
2274:Fibroma
2055:Ductal,
1951:general
1937:Cystic,
1879:Apudoma
1658:Thymoma
1509:C537112
1448:2322961
1406:9756355
1354:4127477
1261:5581856
1212:3226726
1172:4397075
1144:Bibcode
1112:2834712
1027:8 April
824:4574691
779:8 April
722:1342593
637:1180594
507:History
407:neuroma
352:mitoses
273:AKR1B10
160:scrotum
158:of the
2556:PEComa
2535:Lipoma
2435:Myxoma
2218:tumors
2156:Acinar
2020:Serous
1949:Cystic
1743:VIPoma
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1677:Glands
1576:cancer
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439:lipoma
348:nuclei
2755:STUMP
2622:Myoma
2501:-like
1520:34278
1402:S2CID
1358:S2CID
1309:S2CID
1168:S2CID
955:S2CID
718:S2CID
527:Notes
513:et al
302:HMOX1
294:Keap1
253:exons
224:Cause
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2220:and
2158:cell
1536:and
1504:MeSH
1490:OMIM
1453:PMID
1394:PMID
1350:PMID
1301:PMID
1266:PMID
1217:PMID
1160:PMID
1117:PMID
1066:PMID
1029:2019
996:PMID
947:PMID
911:PMID
870:PMID
829:PMID
781:2019
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593:PMID
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362:and
300:and
298:Nrf2
216:and
1443:PMC
1433:doi
1386:doi
1340:doi
1293:doi
1256:PMC
1248:doi
1207:PMC
1199:doi
1152:doi
1140:477
1107:PMC
1097:doi
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939:doi
935:163
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