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Blastema predominant kidney Wilms tumor

MedGen UID:
83528
•Concept ID:
C0279609
•
Neoplastic Process
Synonyms: Blastema Predominant Kidney Adenosarcoma; blastema predominant kidney adenosarcoma; Blastema Predominant Kidney Wilms Tumor; blastema predominant kidney Wilms tumor; blastema predominant kidney Wilms' tumor; blastema predominant kidney Wilms' tumour; Blastema Predominant Nephroblastoma; blastema predominant nephroblastoma; Blastema Predominant Renal Adenosarcoma; blastema predominant renal adenosarcoma; Blastema Predominant Renal Wilm's Tumor; blastema predominant renal Wilm's tumor; blastema predominant renal Wilm's tumour; Blastema Predominant Renal Wilms Tumor; blastema predominant renal Wilms tumor; blastema predominant renal Wilms tumour; Blastema Predominant Renal Wilms' Tumor; blastema predominant renal Wilms' tumor; blastema predominant renal Wilms' tumour; blastema predominant Wilms tumor; blastema predominant Wilms tumour
 
Monarch Initiative: MONDO:0003320

Definition

Wilms tumor of the kidney characterized by the predominance of the blastema component. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBlastema predominant kidney Wilms tumor

Recent clinical studies

Etiology

Blastemal NCAM(+)ALDH1(+) Wilms' tumor cancer stem cells correlate with disease progression and poor clinical outcome: A pilot study.
Raved D, Tokatly-Latzer I, Anafi L, Harari-Steinberg O, Barshack I, Dekel B, Pode-Shakked N
Pathol Res Pract 2019 Aug;215(8):152491. Epub 2019 Jun 10 doi: 10.1016/j.prp.2019.152491. PMID: 31202518
Glypican-3 mRNA expression level in Wilms tumor: correlation with histological type, stage, and outcome.
Wari MN, Vallonthaiel AG, Ahmed A, Saxena D, Iyer VK, Mathur SR, Agarwala S, Bakhshi S, Srinivas V, Chattopadhyaya P, Sharma A, Gupta SD, Dinda A
Pediatr Surg Int 2017 Jun;33(6):695-703. Epub 2017 Apr 21 doi: 10.1007/s00383-017-4087-2. PMID: 28432433
Whole-tumor apparent diffusion coefficient measurements in nephroblastoma: Can it identify blastemal predominance?
Littooij AS, Sebire NJ, Olsen ØE
J Magn Reson Imaging 2017 May;45(5):1316-1324. Epub 2016 Oct 11 doi: 10.1002/jmri.25506. PMID: 27726252
B7-h1 as a biomarker for therapy failure in patients with favorable histology Wilms tumor.
Routh JC, Grundy PE, Anderson JR, Retik AB, Kurek KC
J Urol 2013 Apr;189(4):1487-92. Epub 2012 Nov 12 doi: 10.1016/j.juro.2012.11.012. PMID: 23154206
SIX1 protein expression selectively identifies blastemal elements in Wilms tumor.
Sehic D, Karlsson J, Sandstedt B, Gisselsson D
Pediatr Blood Cancer 2012 Jul 15;59(1):62-8. Epub 2011 Dec 16 doi: 10.1002/pbc.24025. PMID: 22180226

Diagnosis

Whole-tumor apparent diffusion coefficient measurements in nephroblastoma: Can it identify blastemal predominance?
Littooij AS, Sebire NJ, Olsen ØE
J Magn Reson Imaging 2017 May;45(5):1316-1324. Epub 2016 Oct 11 doi: 10.1002/jmri.25506. PMID: 27726252
Diagnostic pitfalls of differentiating desmoplastic small round cell tumor (DSRCT) from Wilms tumor (WT): overlapping morphologic and immunohistochemical features.
Arnold MA, Schoenfield L, Limketkai BN, Arnold CA
Am J Surg Pathol 2014 Sep;38(9):1220-6. doi: 10.1097/PAS.0000000000000231. PMID: 24832162
The cytomorphologic spectrum of Wilms tumour on fine needle aspiration: a single institutional experience of 110 cases.
Nayak A, Iyer VK, Agarwala S
Cytopathology 2011 Feb;22(1):50-9. doi: 10.1111/j.1365-2303.2010.00741.x. PMID: 20236293
Molecular evidence of the independent origin of multiple Wilms tumors in a case of WAGR syndrome.
Uccini S, Perotti D, Colarossi C, Stoppacciaro A, Sardella M, Mannarino O, Collini P, Casieri P, Cozzi D, Amoroso L, Spreafico F, Radice P, Dominici C
Pediatr Blood Cancer 2008 Sep;51(3):344-8. doi: 10.1002/pbc.21507. PMID: 18293378
Primary Ewing's sarcoma/primitive neuroectodermal tumor of the kidney: a clinicopathologic and immunohistochemical analysis of 11 cases.
Jimenez RE, Folpe AL, Lapham RL, Ro JY, O'Shea PA, Weiss SW, Amin MB
Am J Surg Pathol 2002 Mar;26(3):320-7. doi: 10.1097/00000478-200203000-00005. PMID: 11859203

Therapy

Whole-tumor apparent diffusion coefficient measurements in nephroblastoma: Can it identify blastemal predominance?
Littooij AS, Sebire NJ, Olsen ØE
J Magn Reson Imaging 2017 May;45(5):1316-1324. Epub 2016 Oct 11 doi: 10.1002/jmri.25506. PMID: 27726252
B7-h1 as a biomarker for therapy failure in patients with favorable histology Wilms tumor.
Routh JC, Grundy PE, Anderson JR, Retik AB, Kurek KC
J Urol 2013 Apr;189(4):1487-92. Epub 2012 Nov 12 doi: 10.1016/j.juro.2012.11.012. PMID: 23154206
SIX1 protein expression selectively identifies blastemal elements in Wilms tumor.
Sehic D, Karlsson J, Sandstedt B, Gisselsson D
Pediatr Blood Cancer 2012 Jul 15;59(1):62-8. Epub 2011 Dec 16 doi: 10.1002/pbc.24025. PMID: 22180226

Prognosis

Blastemal NCAM(+)ALDH1(+) Wilms' tumor cancer stem cells correlate with disease progression and poor clinical outcome: A pilot study.
Raved D, Tokatly-Latzer I, Anafi L, Harari-Steinberg O, Barshack I, Dekel B, Pode-Shakked N
Pathol Res Pract 2019 Aug;215(8):152491. Epub 2019 Jun 10 doi: 10.1016/j.prp.2019.152491. PMID: 31202518
Glypican-3 mRNA expression level in Wilms tumor: correlation with histological type, stage, and outcome.
Wari MN, Vallonthaiel AG, Ahmed A, Saxena D, Iyer VK, Mathur SR, Agarwala S, Bakhshi S, Srinivas V, Chattopadhyaya P, Sharma A, Gupta SD, Dinda A
Pediatr Surg Int 2017 Jun;33(6):695-703. Epub 2017 Apr 21 doi: 10.1007/s00383-017-4087-2. PMID: 28432433
Diagnostic pitfalls of differentiating desmoplastic small round cell tumor (DSRCT) from Wilms tumor (WT): overlapping morphologic and immunohistochemical features.
Arnold MA, Schoenfield L, Limketkai BN, Arnold CA
Am J Surg Pathol 2014 Sep;38(9):1220-6. doi: 10.1097/PAS.0000000000000231. PMID: 24832162
Apoptotic protein expression in favorable-histology Wilms tumor correlates with tumor recurrence.
Agarwal S, Iyer VK, Agarwala S, Mathur SR, Aron M, Datta Gupta S, Verma K
Pediatr Surg Int 2011 Mar;27(3):303-8. Epub 2010 Oct 21 doi: 10.1007/s00383-010-2740-0. PMID: 20963425
Primary Ewing's sarcoma/primitive neuroectodermal tumor of the kidney: a clinicopathologic and immunohistochemical analysis of 11 cases.
Jimenez RE, Folpe AL, Lapham RL, Ro JY, O'Shea PA, Weiss SW, Amin MB
Am J Surg Pathol 2002 Mar;26(3):320-7. doi: 10.1097/00000478-200203000-00005. PMID: 11859203

Clinical prediction guides

Blastemal NCAM(+)ALDH1(+) Wilms' tumor cancer stem cells correlate with disease progression and poor clinical outcome: A pilot study.
Raved D, Tokatly-Latzer I, Anafi L, Harari-Steinberg O, Barshack I, Dekel B, Pode-Shakked N
Pathol Res Pract 2019 Aug;215(8):152491. Epub 2019 Jun 10 doi: 10.1016/j.prp.2019.152491. PMID: 31202518
Diagnostic pitfalls of differentiating desmoplastic small round cell tumor (DSRCT) from Wilms tumor (WT): overlapping morphologic and immunohistochemical features.
Arnold MA, Schoenfield L, Limketkai BN, Arnold CA
Am J Surg Pathol 2014 Sep;38(9):1220-6. doi: 10.1097/PAS.0000000000000231. PMID: 24832162
Primary primitive neuroectodermal tumor of the kidney: a case report.
Erkiliç S, Ozsaraç C, Koçer NE, Erbağci A
Int Urol Nephrol 2006;38(2):199-202. doi: 10.1007/s11255-006-6675-2. PMID: 16868682
Myogenesis in Wilms' tumors is associated with mutations of the WT1 gene and activation of Bcl-2 and the Wnt signaling pathway.
Fukuzawa R, Heathcott RW, Sano M, Morison IM, Yun K, Reeve AE
Pediatr Dev Pathol 2004 Mar-Apr;7(2):125-37. Epub 2004 Mar 4 doi: 10.1007/s10024-003-3023-8. PMID: 14994125
Histologic characterization of renal tumors (nephroblastomas) induced transplacentally in IIIVO/J and WH/J rabbits by N-ethylnitrosourea.
Hard GC, Fox RR
Am J Pathol 1983 Oct;113(1):8-18. PMID: 6312804Free PMC Article

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