40:
72:
398:, which is responsible for 5-10% of childhood pediatric tumors, occurs predominantly in children from 2 to 3 years of age. Unlike mesoblastic nephroma, clear cell sarcoma of the kidney presents with metastasis, particularly to bone, in 5-6% of cases; it histology is diverse and has been mistaken for mesoblastic nephroma. One chromosomal translocations t,(10;17)(q22;p13), has been repeatedly reported to be associated with clear cell sarcoma of the kidney.
1251:
405:
an excellent prognosis and can regress spontaneously except for those in which there is visceral involvement where the prognosis is poor. While infantile myofibromatosis and classic mesoblastic nephroma have been suggested to be the same diseases because of their very similar histology, studies on the distribution of cell-type markers (i.e.
384:, which accounts for 5-10% of childhood kidney neoplasms, occurs predominantly in children from 1 to 2 years of age. Unlike mesoblastic nephroma, rhabdoid tumors may present with tumors in other tissues including in ~13% of cases, the brain. Rhabdoid tumors have a distinctive histology and abnormalities (i.e.
435:
treatment. Removal of the entire afflicted kidney plus the peri-renal fat appears critical to avoiding local recurrences. In general, patients who were older than 3 months of age at diagnosis or had the cellular form of the disease, stage III disease, or involvement of renal lymph nodes had a higher
499:
fusion gene was successfully treated with larotrectinib. The success of these drugs, however, will likely depend on the relative malignancy-promoting roles of ETV6-NTRK3 protein's tyrosine kinase activity, the lose of ETV6-related transcription activity accompanying formation of ETV6-NTRK3 protein,
426:
appeared curative for the majority of all types of mesoblastic nephroma; the patient overall survival rate was 94%. Of the 4% of non-survivors, half were due to surgical or chemotherapeutic treatments. Another 4% of these patients suffered relapses, primarily in the local area of surgery rare cases
404:
is a fibrous tumor of infancy and childhood most commonly presenting during the first 2 years of life as a single subcutaneous nodule of the head and neck region or less commonly as multiple lesions of skin, muscle, bone, and in ~33% of these latter cases, visceral organs. All of these lesions have
355:
is the most common childhood kidney neoplasm, representing some 85% of cases. Unlike mesoblastic nephroma, <2% of Wilms tumor patients present at under 3 months of age and most present in patients of >3 years of age. Bilateral kidney tumors, concurrent birth defects, and/or metastatic disease
323:
fusion gene appears to occur in most cases of the cellular and some cases of the mixed but no cases of the classical types of congenital mesolastic nephroma. However, a more recent study of 19 patients detected the fused gene in all 8 cases of cellular, 5 of 6 cases of mixed, and 0 of 5 cases of
318:
product of this gene lacks ETV6 protein's transcription factor activity while having NTRK3 protein's tyrosine kinase in an unregulated and continuously active form. Either event can drive the malignant growth of cells but in most cases the chimeric protein's tyrosine kinase activity appears
378:) within this tumor are the same as those found in cellular mesoblastic nephroma. Mesoblastic nephroma and congenital infantile sarcoma appear to be the same diseases with mesoblastic lymphoma originating in the kidney and congenital infantile sarcoma originating in non-renal tissues.
682:
El
Demellawy D, Cundiff CA, Nasr A, Ozolek JA, Elawabdeh N, Caltharp SA, Masoudian P, Sullivan KJ, de Nanassy J, Shehata BM (2016). "Congenital mesoblastic nephroma: a study of 19 cases using immunohistochemistry and ETV6-NTRK3 fusion gene rearrangement".
334:
have also found trisomy 8 (9 cases), 17 (4 cases), 20 (4 cases), 7 (3 cases), 10 (3 cases), 18 (2 cases), 2 (2 cases), and 9 (2 cases) associated with the disease. The contribution of these trisomies to the development of mesoblastic nephroma is unclear.
330:, i.e. pathological presence of an extra chromosome, also occurs in these neoplasms. Trisomy of chromosome 11 (e.g. trisomy 11) appears to be the most commonly found trisomy in this disease, being detected in 7 of 13 genetically studied cases. Individual
343:
Diagnosis of mesoblastic nephroma and its particular type (i.e. classic, mixed, or cellular) is made by histological examination of tissues obtained at surgery. Besides its histological appearance, various features of this disease aid in making a
984:"Genomic analysis using high-density single nucleotide polymorphism-based oligonucleotide arrays and multiplex ligation-dependent probe amplification provides a comprehensive analysis of INI1/SMARCB1 in malignant rhabdoid tumors"
427:
of relapse due to lung or bone metastasis.. About 60% of these recurrent cases had a complete remission following further treatment. Recurrent disease was treated with a second surgery, radiation, and/or chemotherapy that often
125:
diagnosed in the first three months of life and accounts for 3-5% of all childhood renal neoplasms. It is generally non-aggressive and amenable to surgical removal, though there is a subtype that is more aggressive and tends to
1255:
202:) disease. Most patients' disease is classified at presentation as stage I or II (i.e. localized), few patients present with stage III (i.e. locally advanced/infiltrating), and virtually no patients present with stage IV (
946:
Walther C, Nilsson J, von
Steyern FV, Wiebe T, Bauer HC, Nord KH, Gisselsson D, Domanski HA, Mandahl N, Mertens F (2013). "Cytogenetic and single nucleotide polymorphism array findings in soft tissue tumors in infants".
1294:
1829:
1204:
1849:
162:
or by clinical inspection (84% of cases) either at birth or by 3.8 years of age (median age ~1 month). The neoplasm shows a slight male preference. Concurrent findings include
324:
classic mesoblastic nephroma. This suggests that expression of this fused gene may be more common in cellar and mixed mesoblastic nephroma than previously appreciated.
537:
624:
Lamb MG, Aldrink JH, O'Brien SH, Yin H, Arnold MA, Ranalli MA (2017). "Renal Tumors in
Children Younger Than 12 Months of Age: A 65-Year Single Institution Review".
718:
Wang, ZP; Li, K; Dong, KR; Xiao, XM; Zheng, S (November 2014). "Congenital mesoblastic nephroma: Clinical analysis of eight cases and a review of the literature".
1287:
1972:
577:
Gooskens SL, Houwing ME, Vujanic GM, Dome JS, Diertens T, Coulomb-l'Herminé A, Godzinski J, Pritchard-Jones K, Graf N, van den Heuvel-Eibrink MM (2017).
226:
examination of these tumors provides critical information on their prognoses. This examination divides congenital mesoblastic nephroma into three types:
903:
Ud Din N, Minhas K, Shamim MS, Mushtaq N, Fadoo Z (2015). "Congenital (infantile) fibrosarcoma of the scalp: a case series and review of literature".
1280:
495:) that are driven by mutated, overly active tyrosine kinases. Relevant to this issue, a 16-month-old girl with infantile fibrosarcoma harboring the
860:
Anderson J, Gibson S, Sebire NJ (2006). "Expression of ETV6-NTRK in classical, cellular and mixed subtypes of congenital mesoblastic nephroma".
366:
typically presenting in the lower extremities, head, or neck of infants during their first year of life. The histology, association with the
2185:
1650:
1497:
1031:
Gooskens SL, Furtwängler R, Vujanic GM, Dome JS, Graf N, van den Heuvel-Eibrink MM (2012). "Clear cell sarcoma of the kidney: a review".
1333:
190:(1%). Congenital anomalies have been reported in 11 patients: 6 with genitourinary anomalies, 2 with gastrointestinal anomalies, 1 with
488:
439:
It has been suggested that mesoblastic nephroma patients with lymph node involvement or recurrent disease might benefit by adding the
256:
3) The mixed type occurs in ~19% of patients. It shows a mixture of the classic and cellular types in different areas of the neoplasm.
158:
Congenital mesoblastic nephroma typically (76% of cases) presents as an abdominal mass which is detected prenatally (16% of cases) by
436:
recurrence rate. Among patients with these risk factors, only those with lymph node involvement are recommended for further therapy.
1410:
463:
treatment regimens. These drugs inhibit NTRK3's tyrosine kinase activity. Crizotinib has proven useful in treating certain cases of
2045:
1965:
1901:
1142:
Laetsch TW, Nagasubramanian R, Casanova M (2017). "Targeting NTRK fusions for the treatment of congenital mesoblastic nephroma".
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1068:"Clear cell sarcoma of the kidney misdiagnosed as mesoblastic nephroma: a case report and review of the literature"
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A study conducted in 1998 found that congenital mesoblastic nephroma tissues taken from some patients contained an
61:
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99:
982:
Jackson EM, Sievert AJ, Gai X, Hakonarson H, Judkins AR, Tooke L, Perin JC, Xie H, Shaikh TH, Biegel JA (2009).
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fusion gene while larotrectinib and entrectinib have been useful in treating various cancers (e.g. a metastatic
1435:
1212:
476:
238:
activity with no evidence of tumor encapsulation; and infiltration into and entrapment of normal kidney tissue.
139:
1881:
2159:
2131:
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1863:
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130:. Congenital mesoblastic nephroma was first named as such in 1967 but was recognized decades before this as
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1502:
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270:
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fusion gene along with certain chromosome trisomies, and the distribution of markers for cell type (i.e.
143:
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gene located on the short arm (designated p) of chromosome 12 at position p13.2 (i.e. 12p13.2) to the
2012:
1731:
1455:
1383:
319:
responsible for doing so. Based on a limited number of genetic studies (a total of 65 patients), the
294:
gene located on the long arm (designated q) of chromosome 15 at position q25.3 (i.e. 15q25.3). This
71:
2060:
1876:
1802:
1684:
1484:
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1420:
1415:
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764:"Congenital mesoblastic nephroma: Clinical analysis of eight cases and a review of the literature"
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2017:
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1) The classic type occurs in ~39% of patients. Its tissues show interlaced spindle-shaped
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448:
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Based on a survey of >800, surgical removal of the entire involved kidney plus the
186:(4%), and elevated serum levels of the kidney-secreted, hypertension-inducing enzyme,
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1997:
873:
452:
440:
231:
191:
183:
17:
1171:
960:
932:
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579:"Congenital mesoblastic nephroma 50 years after its recognition: A narrative review"
47:
Congenital mesoblastic nephroma, classic type, with typical features. H&E stain.
1934:
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1397:
1370:
1338:
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249:, less infiltration of normal kidney tissue, and multiple areas of hemorrhage and
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2) The cellular type occurs in ~42% of patients. Its tissues show densely packed
2027:
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1533:
1307:
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83:
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Congenital
Mesoblastic nephroma is a malignant tumorous growth of the kidney's
1871:
1720:
916:
444:
219:
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199:
159:
127:
1117:"Childhood Soft Tissue Sarcoma Treatment (PDQ®): Health Professional Version"
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797:
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704:
645:
602:
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1981:
1310:
813:"Molecular mechanisms of ETS transcription factor-mediated tumorigenesis"
779:
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542:
Last author update: 10 December 2020. Last staff update: 29 November 2021
266:
122:
57:
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and the various trisomy chromosomes that populate mesoblastic nephroma.
1393:
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1314:
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327:
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246:
235:
65:
1155:
594:
1629:
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Alavi S, Khoddami M, Yazdi MK, Dehghanian P, Esteghamati S (2013).
1716:
1228:
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187:
198:. The vast majority of patients present with localized (i.e. non-
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gene fusion is notated as t(12;15)(p13;q25) and consists of the
284:
1954:
1276:
516:
Image by Mikael Häggström, MD. Source for typical features:
348:
that distinguish it from the following childhood neoplasms:
1245:
entry in the public domain NCI Dictionary of Cancer Terms
206:
present or V (i.e. tumors in both kidneys) disease (see
1830:
Multiple cutaneous and uterine leiomyomatosis syndrome
817:
Critical
Reviews in Biochemistry and Molecular Biology
518:
Ellen D’Hooghe, M.D., Gordan M. Vujanic, M.D., Ph.D.
413:) indicate that they have different cellular origins.
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1996:
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1321:
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105:
79:
51:
32:
762:Wang ZP, Li K, Dong KR, Xiao XM, Zheng S (2014).
356:at presentation favor a diagnosis of Wilms tumor.
1115:PDQ Pediatric Treatment Editorial Board (2002).
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536:: CS1 maint: multiple names: authors list (
2002:
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70:
38:
29:
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1007:
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787:
138:. It is embryologically derived from the
626:Journal of Pediatric Hematology/Oncology
509:
529:
142:, the same tissue that gives rise to
7:
811:Kar A, Gutierrez-Hartmann A (2013).
1334:Desmoplastic small-round-cell tumor
1123:. National Cancer Institute (US).
489:mammary analog secretory carcinoma
392:, and deletions) in chromosome 22.
25:
2006:Glandular and epithelial neoplasm
1411:Aggressive infantile fibromatosis
520:"Congenital mesoblastic nephroma"
459:, to surgical, radiation, and/or
118:, while rare, is the most common
2046:Clear-cell sarcoma of the kidney
1902:Clear-cell sarcoma of the kidney
1254: This article incorporates
1249:
1121:PDQ Cancer Information Summaries
874:10.1111/j.1365-2559.2006.02400.x
396:Clear cell sarcoma of the kidney
222:(i.e. connective tissue cells).
96:Clear cell sarcoma of the kidney
1670:Intradermal spindle cell lipoma
1379:Dermatofibrosarcoma protuberans
1243:Congenital mesoblastic nephroma
961:10.1016/j.cancergen.2013.06.004
245:cells evidencing high rates of
116:Congenital mesoblastic nephroma
2186:Malignant fibrous histiocytoma
1498:Malignant fibrous histiocytoma
1446:Infantile digital fibromatosis
1431:Familial myxovascular fibromas
1426:Diffuse infantile fibromatosis
1267:U.S. National Cancer Institute
485:gastrointestinal stromal tumor
390:single nucleotide polymorphism
1:
1544:Superficial acral fibromyxoma
1451:Juvenile hyaline fibromatosis
1000:10.1158/1078-0432.CCR-08-2091
481:non-small-cell lung carcinoma
467:that are associated with the
282:of genetic material from the
136:leiomyomatous renal hamartoma
1845:Solitary cutaneous leiomyoma
1835:Multiple cutaneous leiomyoma
1556:Ossifying fibromyxoid tumour
1144:Pediatric Blood & Cancer
829:10.3109/10409238.2013.838202
697:10.1016/j.pathol.2015.11.007
638:10.1097/MPH.0000000000000698
583:Pediatric Blood & Cancer
465:acute lymphoblastic leukemia
360:congenital infantile sarcoma
88:congenital infantile sarcoma
2155:Transitional cell carcinoma
2127:Transitional cell carcinoma
1493:Benign fibrous histiocytoma
278:. This gene results from a
196:Beckwith–Wiedemann syndrome
2223:
2082:Juxtaglomerular cell tumor
1773:Embryonal rhabdomyosarcoma
1349:Connective tissue neoplasm
1262:Dictionary of Cancer Terms
1045:10.1016/j.ejca.2012.04.009
1033:European Journal of Cancer
62:obstetrics and gynaecology
2077:Renal medullary carcinoma
1897:Malignant rhabdoid tumour
1864:Complex mixed and stromal
1793:
1785:Alveolar rhabdomyosarcoma
917:10.1007/s00381-015-2824-1
402:Infantile myofibromatosis
100:Infantile myofibromatosis
46:
37:
1436:Fibroma of tendon sheath
1084:10.3332/ecancer.2013.311
988:Clinical Cancer Research
477:papillary thyroid cancer
140:metanephrogenic blastema
2160:Squamous-cell carcinoma
2132:Squamous-cell carcinoma
2087:Renal medullary fibroma
1466:Oral submucous fibrosis
1406:Aggressive fibromatosis
1322:Not otherwise specified
208:staging of renal cancer
1508:Solitary fibrous tumor
1503:Atypical fibroxanthoma
1256:public domain material
905:Child's Nervous System
386:loss of heterozygosity
346:differential diagnosis
314:. In consequence, the
128:spread to other organs
1882:Mixed MĂĽllerian tumor
1072:ecancermedicalscience
362:is a rare aggressive
234:cells evidencing low
132:fetal renal hamartoma
18:Nephroma, mesoblastic
2207:Urological neoplasia
2041:Mesoblastic nephroma
2013:Renal cell carcinoma
1887:Mesoblastic nephroma
1456:Plantar fibromatosis
1384:Desmoplastic fibroma
780:10.3892/ol.2014.2489
732:10.3892/ol.2014.2489
33:Mesoblastic nephroma
2061:Metanephric adenoma
1877:Pleomorphic adenoma
1685:Spindle cell lipoma
1485:histiocytic sarcoma
1461:Pleomorphic fibroma
1421:Collagenous fibroma
1416:Aponeurotic fibroma
1329:Soft-tissue sarcoma
144:nephroblastomatosis
2137:Inverted papilloma
1840:Neural fibrolipoma
1815:Angiolipoleiomyoma
1778:Sarcoma botryoides
1675:Pleomorphic lipoma
1644:Myxoid liposarcoma
1608:Clear-cell sarcoma
1441:Fibromatosis colli
524:Pathology Outlines
451:inhibitor, either
2194:
2193:
2109:Ureteral neoplasm
2095:
2094:
1948:
1947:
1940:Adenomatoid tumor
1912:Pancreatoblastoma
1858:
1857:
1820:Genital leiomyoma
1680:Lipoblastomatosis
1616:
1615:
1516:
1515:
1471:Pachydermodactyly
1238:
1237:
1156:10.1002/pbc.26593
595:10.1002/pbc.26437
493:colorectal cancer
267:acquired mutation
243:fibrosarcoma-like
194:, and 1 with the
113:
112:
27:Medical condition
16:(Redirected from
2214:
2018:Renal oncocytoma
2003:
1975:
1968:
1961:
1952:
1769:rhabdomyosarcoma
1758:
1734:
1710:
1705:
1665:Chondroid lipoma
1603:Synovial sarcoma
1539:Cutaneous myxoma
1363:
1354:
1297:
1290:
1283:
1274:
1270:
1253:
1252:
1188:
1176:
1175:
1139:
1133:
1132:
1112:
1106:
1105:
1095:
1063:
1057:
1056:
1028:
1022:
1021:
1011:
979:
973:
972:
955:(7–8): 299–303.
943:
937:
936:
900:
894:
893:
857:
851:
850:
840:
808:
802:
801:
791:
774:(5): 2007–2011.
768:Oncology Letters
759:
744:
743:
726:(5): 2007–2011.
720:Oncology letters
715:
709:
708:
679:
658:
657:
621:
615:
614:
574:
543:
541:
535:
527:
514:
316:chimeric protein
170:(i.e. excess of
166:(19% of cases),
109:surgical removal
75:
74:
42:
30:
21:
2222:
2221:
2217:
2216:
2215:
2213:
2212:
2211:
2197:
2196:
2195:
2190:
2174:
2141:
2113:
2091:
2065:
2056:Cystic nephroma
2022:
1992:
1990:genital systems
1979:
1949:
1944:
1921:
1854:
1789:
1756:Skeletal muscle
1754:
1749:
1730:
1725:
1708:
1694:
1612:
1588:
1584:Phyllodes tumor
1566:Fibroepithelial
1560:
1512:
1475:
1388:
1343:
1317:
1301:
1259:
1250:
1239:
1234:
1233:
1199:
1185:
1180:
1179:
1141:
1140:
1136:
1114:
1113:
1109:
1065:
1064:
1060:
1039:(14): 2219–26.
1030:
1029:
1025:
981:
980:
976:
949:Cancer Genetics
945:
944:
940:
902:
901:
897:
859:
858:
854:
810:
809:
805:
761:
760:
747:
717:
716:
712:
681:
680:
661:
623:
622:
618:
576:
575:
546:
528:
517:
515:
511:
506:
449:tyrosine kinase
420:
341:
263:
216:
214:Tumor pathology
156:
69:
28:
23:
22:
15:
12:
11:
5:
2220:
2218:
2210:
2209:
2199:
2198:
2192:
2191:
2189:
2188:
2182:
2180:
2176:
2175:
2173:
2172:
2167:
2165:Adenocarcinoma
2162:
2157:
2151:
2149:
2143:
2142:
2140:
2139:
2134:
2129:
2123:
2121:
2115:
2114:
2112:
2111:
2105:
2103:
2097:
2096:
2093:
2092:
2090:
2089:
2084:
2079:
2073:
2071:
2067:
2066:
2064:
2063:
2058:
2053:
2051:Angiomyolipoma
2048:
2043:
2038:
2032:
2030:
2024:
2023:
2021:
2020:
2015:
2009:
2007:
2000:
1994:
1993:
1980:
1978:
1977:
1970:
1963:
1955:
1946:
1945:
1943:
1942:
1937:
1931:
1929:
1923:
1922:
1920:
1919:
1917:Carcinosarcoma
1914:
1909:
1907:Hepatoblastoma
1904:
1899:
1894:
1889:
1884:
1879:
1874:
1868:
1866:
1860:
1859:
1856:
1855:
1853:
1852:
1847:
1842:
1837:
1832:
1827:
1825:Leiomyosarcoma
1822:
1817:
1812:
1810:Angioleiomyoma
1807:
1806:
1805:
1794:
1791:
1790:
1788:
1787:
1782:
1781:
1780:
1761:
1759:
1751:
1750:
1748:
1747:
1745:leiomyosarcoma
1737:
1735:
1727:
1726:
1724:
1723:
1713:
1711:
1702:
1696:
1695:
1693:
1692:
1687:
1682:
1677:
1672:
1667:
1661:
1660:
1659:
1658:
1656:Angiomyolipoma
1648:
1647:
1646:
1641:
1626:
1624:
1618:
1617:
1614:
1613:
1611:
1610:
1605:
1599:
1597:
1590:
1589:
1587:
1586:
1581:
1576:
1574:Brenner tumour
1570:
1568:
1562:
1561:
1559:
1558:
1553:
1548:
1547:
1546:
1541:
1526:
1524:
1518:
1517:
1514:
1513:
1511:
1510:
1505:
1500:
1495:
1489:
1487:
1477:
1476:
1474:
1473:
1468:
1463:
1458:
1453:
1448:
1443:
1438:
1433:
1428:
1423:
1418:
1413:
1408:
1402:
1400:
1390:
1389:
1387:
1386:
1381:
1375:
1373:
1360:
1351:
1345:
1344:
1342:
1341:
1336:
1331:
1325:
1323:
1319:
1318:
1302:
1300:
1299:
1292:
1285:
1277:
1247:
1246:
1236:
1235:
1232:
1231:
1220:
1209:
1200:
1195:
1194:
1192:
1191:Classification
1184:
1183:External links
1181:
1178:
1177:
1134:
1107:
1058:
1023:
994:(6): 1923–30.
974:
938:
911:(11): 2145–9.
895:
862:Histopathology
852:
803:
745:
710:
659:
632:(2): 103–107.
616:
544:
508:
507:
505:
502:
424:peri-renal fat
419:
416:
415:
414:
399:
393:
382:Rhabdoid tumor
379:
357:
340:
337:
262:
259:
258:
257:
254:
239:
215:
212:
172:amniotic fluid
168:polyhydramnios
155:
152:
111:
110:
107:
103:
102:
92:Rhabdoid tumor
81:
77:
76:
55:
49:
48:
44:
43:
35:
34:
26:
24:
14:
13:
10:
9:
6:
4:
3:
2:
2219:
2208:
2205:
2204:
2202:
2187:
2184:
2183:
2181:
2177:
2171:
2168:
2166:
2163:
2161:
2158:
2156:
2153:
2152:
2150:
2148:
2144:
2138:
2135:
2133:
2130:
2128:
2125:
2124:
2122:
2120:
2116:
2110:
2107:
2106:
2104:
2102:
2098:
2088:
2085:
2083:
2080:
2078:
2075:
2074:
2072:
2068:
2062:
2059:
2057:
2054:
2052:
2049:
2047:
2044:
2042:
2039:
2037:
2034:
2033:
2031:
2029:
2025:
2019:
2016:
2014:
2011:
2010:
2008:
2004:
2001:
1999:
1995:
1991:
1987:
1983:
1976:
1971:
1969:
1964:
1962:
1957:
1956:
1953:
1941:
1938:
1936:
1933:
1932:
1930:
1928:
1924:
1918:
1915:
1913:
1910:
1908:
1905:
1903:
1900:
1898:
1895:
1893:
1890:
1888:
1885:
1883:
1880:
1878:
1875:
1873:
1870:
1869:
1867:
1865:
1861:
1851:
1848:
1846:
1843:
1841:
1838:
1836:
1833:
1831:
1828:
1826:
1823:
1821:
1818:
1816:
1813:
1811:
1808:
1804:
1801:
1800:
1799:
1796:
1795:
1792:
1786:
1783:
1779:
1776:
1775:
1774:
1770:
1766:
1763:
1762:
1760:
1757:
1752:
1746:
1742:
1739:
1738:
1736:
1733:
1732:Smooth muscle
1728:
1722:
1718:
1715:
1714:
1712:
1706:
1703:
1701:
1697:
1691:
1688:
1686:
1683:
1681:
1678:
1676:
1673:
1671:
1668:
1666:
1663:
1662:
1657:
1654:
1653:
1652:
1649:
1645:
1642:
1640:
1637:
1636:
1635:
1631:
1628:
1627:
1625:
1623:
1619:
1609:
1606:
1604:
1601:
1600:
1598:
1595:
1591:
1585:
1582:
1580:
1577:
1575:
1572:
1571:
1569:
1567:
1563:
1557:
1554:
1552:
1549:
1545:
1542:
1540:
1537:
1536:
1535:
1531:
1528:
1527:
1525:
1523:
1519:
1509:
1506:
1504:
1501:
1499:
1496:
1494:
1491:
1490:
1488:
1486:
1482:
1478:
1472:
1469:
1467:
1464:
1462:
1459:
1457:
1454:
1452:
1449:
1447:
1444:
1442:
1439:
1437:
1434:
1432:
1429:
1427:
1424:
1422:
1419:
1417:
1414:
1412:
1409:
1407:
1404:
1403:
1401:
1399:
1395:
1391:
1385:
1382:
1380:
1377:
1376:
1374:
1372:
1368:
1364:
1361:
1359:
1355:
1352:
1350:
1346:
1340:
1337:
1335:
1332:
1330:
1327:
1326:
1324:
1320:
1316:
1312:
1309:
1305:
1298:
1293:
1291:
1286:
1284:
1279:
1278:
1275:
1271:
1268:
1264:
1263:
1257:
1244:
1241:
1240:
1230:
1226:
1225:
1221:
1219:
1215:
1214:
1210:
1207:
1206:
1202:
1201:
1198:
1193:
1189:
1182:
1173:
1169:
1165:
1161:
1157:
1153:
1150:(1): e26593.
1149:
1145:
1138:
1135:
1130:
1126:
1122:
1118:
1111:
1108:
1103:
1099:
1094:
1089:
1085:
1081:
1077:
1073:
1069:
1062:
1059:
1054:
1050:
1046:
1042:
1038:
1034:
1027:
1024:
1019:
1015:
1010:
1005:
1001:
997:
993:
989:
985:
978:
975:
970:
966:
962:
958:
954:
950:
942:
939:
934:
930:
926:
922:
918:
914:
910:
906:
899:
896:
891:
887:
883:
879:
875:
871:
868:(6): 748–53.
867:
863:
856:
853:
848:
844:
839:
834:
830:
826:
823:(6): 522–43.
822:
818:
814:
807:
804:
799:
795:
790:
785:
781:
777:
773:
769:
765:
758:
756:
754:
752:
750:
746:
741:
737:
733:
729:
725:
721:
714:
711:
706:
702:
698:
694:
690:
686:
678:
676:
674:
672:
670:
668:
666:
664:
660:
655:
651:
647:
643:
639:
635:
631:
627:
620:
617:
612:
608:
604:
600:
596:
592:
589:(7): e26437.
588:
584:
580:
573:
571:
569:
567:
565:
563:
561:
559:
557:
555:
553:
551:
549:
545:
539:
533:
525:
521:
513:
510:
503:
501:
498:
494:
490:
486:
482:
478:
474:
470:
466:
462:
458:
454:
453:larotrectinib
450:
446:
442:
441:ALK inhibitor
437:
434:
430:
425:
417:
412:
408:
403:
400:
397:
394:
391:
387:
383:
380:
377:
373:
369:
365:
361:
358:
354:
351:
350:
349:
347:
338:
336:
333:
329:
325:
322:
317:
313:
309:
306:fused to the
305:
301:
297:
293:
292:
287:
286:
281:
280:translocation
277:
274:
273:
268:
260:
255:
252:
248:
244:
240:
237:
233:
232:smooth muscle
229:
228:
227:
225:
221:
213:
211:
209:
205:
201:
197:
193:
192:hydrocephalus
189:
185:
184:hypercalcemia
181:
177:
173:
169:
165:
161:
153:
151:
149:
145:
141:
137:
133:
129:
124:
121:
117:
108:
104:
101:
97:
93:
89:
85:
82:
78:
73:
67:
63:
59:
56:
54:
50:
45:
41:
36:
31:
19:
2040:
2036:Wilms' tumor
1935:Mesothelioma
1892:Wilms' tumor
1886:
1579:Fibroadenoma
1481:Histiocytoma
1398:fibromatosis
1371:fibrosarcoma
1339:Skin sarcoma
1261:
1248:
1222:
1211:
1203:
1147:
1143:
1137:
1120:
1110:
1075:
1071:
1061:
1036:
1032:
1026:
991:
987:
977:
952:
948:
941:
908:
904:
898:
865:
861:
855:
820:
816:
806:
771:
767:
723:
719:
713:
691:(1): 47–50.
688:
684:
629:
625:
619:
586:
582:
523:
512:
496:
468:
461:chemotherapy
438:
421:
411:Beta-catenin
376:Beta-catenin
367:
342:
332:case reports
326:
320:
311:
303:
295:
289:
283:
271:
264:
217:
176:amniotic sac
164:hypertension
157:
154:Presentation
135:
131:
115:
114:
2070:by location
2028:Mixed tumor
1927:Mesothelial
1765:Rhabdomyoma
1639:Myelolipoma
1634:liposarcoma
1551:Angiomyxoma
1534:myxosarcoma
1358:Fibromatous
1308:soft tissue
457:entrectinib
433:actinomycin
429:vincristine
353:Wilms tumor
276:fusion gene
148:Wilms tumor
84:Wilms tumor
1872:Adenomyoma
1721:myosarcoma
1622:Lipomatous
1522:Myxomatous
1304:Connective
504:References
497:ETV6–NTRK3
469:ETV6-NTRK3
445:crizotinib
368:ETV6-NRTK3
321:ETY6-NTRK3
296:ETV6-NTRK3
272:ETV6-NTRK3
224:Histologic
220:mesenchyme
204:metastases
200:metastatic
160:ultrasound
1803:Cutaneous
1798:Leiomyoma
1741:Leiomyoma
1700:Myomatous
1690:Hibernoma
1229:307604008
1224:SNOMED CT
685:Pathology
418:Treatment
407:cyclin D1
372:cyclin D1
339:Diagnosis
180:hematuria
178:) (15%),
106:Treatment
53:Specialty
2201:Category
2170:Melanoma
1594:Synovial
1315:sarcomas
1208:: 8960/1
1172:34088251
1164:28440051
1129:26389361
1102:23634181
1053:22579455
1018:19276269
969:23938179
933:25198570
925:26206116
890:36404121
882:16681692
847:24066765
798:25295083
740:25295083
705:27020209
654:40223322
646:27820132
611:22681362
603:28124468
532:cite web
261:Genetics
123:neoplasm
58:Oncology
2147:Urethra
2119:Bladder
1986:urinary
1984:of the
1709:General
1394:Fibroma
1367:Fibroma
1218:D018201
1093:3634723
1078:: 311.
1009:2668138
838:4086824
789:4186628
473:sarcoma
447:, or a
364:sarcoma
328:Trisomy
247:mitosis
236:mitotic
182:(11%),
174:in the
66:urology
2101:Ureter
1998:Kidney
1982:Tumors
1651:PEComa
1630:Lipoma
1530:Myxoma
1311:tumors
1170:
1162:
1127:
1100:
1090:
1051:
1016:
1006:
967:
931:
923:
888:
880:
845:
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796:
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738:
703:
652:
644:
609:
601:
491:, and
308:3' end
300:5' end
269:, the
120:kidney
68:
2179:Other
1850:STUMP
1717:Myoma
1596:-like
1258:from
1205:ICD-O
1168:S2CID
929:S2CID
886:S2CID
650:S2CID
607:S2CID
312:NTRK3
291:NTRK3
251:cysts
188:renin
80:Types
1988:and
1313:and
1213:MeSH
1160:PMID
1125:PMID
1098:PMID
1049:PMID
1014:PMID
965:PMID
921:PMID
878:PMID
843:PMID
794:PMID
736:PMID
701:PMID
642:PMID
599:PMID
538:link
431:and
409:and
374:and
304:ETV6
285:ETV6
146:and
1152:doi
1088:PMC
1080:doi
1041:doi
1004:PMC
996:doi
957:doi
953:206
913:doi
870:doi
833:PMC
825:doi
784:PMC
776:doi
728:doi
693:doi
634:doi
591:doi
455:or
310:of
302:of
210:).
134:or
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530:{{
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1960:v
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872::
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778::
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253:.
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