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Stromal predominant kidney Wilms tumor

MedGen UID:
83529
•Concept ID:
C0279610
•
Neoplastic Process
Synonyms: Stromal Predominant Kidney Adenosarcoma; stromal predominant kidney adenosarcoma; Stromal Predominant Kidney Wilms Tumor; stromal predominant kidney Wilms tumor; stromal predominant kidney Wilms' tumor; stromal predominant kidney Wilms' tumour; Stromal Predominant Nephroblastoma; stromal predominant nephroblastoma; Stromal Predominant Renal Adenosarcoma; stromal predominant renal adenosarcoma; Stromal Predominant Renal Wilm's Tumor; stromal predominant renal Wilm's tumor; stromal predominant renal Wilm's tumour; Stromal Predominant Renal Wilms Tumor; stromal predominant renal Wilms tumor; stromal predominant renal Wilms tumour; Stromal Predominant Renal Wilms' Tumor; stromal predominant renal Wilms' tumor; stromal predominant renal Wilms' tumour; stromal predominant Wilms tumor; stromal predominant Wilms tumour
 
Monarch Initiative: MONDO:0006432

Definition

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

Term Hierarchy

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

Professional guidelines

PubMed

Response of bilateral Wilms tumor to chemotherapy suggests histologic subtype and guides treatment.
Duncan C, Sarvode Mothi S, Santiago TC, Coggins JA, Graetz DE, Bishop MW, Mullen EA, Murphy AJ, Green DM, Krasin MJ, Davidoff AM
J Natl Cancer Inst 2024 Aug 1;116(8):1230-1237. doi: 10.1093/jnci/djae072. PMID: 38539045Free PMC Article
An eHealth decision-support tool to prioritize referral practices for genetic evaluation of patients with Wilms tumor.
Cullinan N, Villani A, Mourad S, Somers GR, Reichman L, van Engelen K, Stephens D, Weksberg R, Foulkes WD, Malkin D, Grant R, Goudie C
Int J Cancer 2020 Feb 15;146(4):1010-1017. Epub 2019 Jul 30 doi: 10.1002/ijc.32561. PMID: 31286500
Twenty-four new cases of WT1 germline mutations and review of the literature: genotype/phenotype correlations for Wilms tumor development.
Royer-Pokora B, Beier M, Henzler M, Alam R, Schumacher V, Weirich A, Huff V
Am J Med Genet A 2004 Jun 15;127A(3):249-57. doi: 10.1002/ajmg.a.30015. PMID: 15150775

Recent clinical studies

Etiology

Response of bilateral Wilms tumor to chemotherapy suggests histologic subtype and guides treatment.
Duncan C, Sarvode Mothi S, Santiago TC, Coggins JA, Graetz DE, Bishop MW, Mullen EA, Murphy AJ, Green DM, Krasin MJ, Davidoff AM
J Natl Cancer Inst 2024 Aug 1;116(8):1230-1237. doi: 10.1093/jnci/djae072. PMID: 38539045Free PMC Article
"Teratoid" Wilms Tumor: The Extreme End of Heterologous Element Differentiation, Not a Separate Entity.
D'Hooghe E, Mifsud W, Vujanić GM
Am J Surg Pathol 2019 Nov;43(11):1583-1590. doi: 10.1097/PAS.0000000000001335. PMID: 31600178
Wilms tumor: an update.
Al-Hussain T, Ali A, Akhtar M
Adv Anat Pathol 2014 May;21(3):166-73. doi: 10.1097/PAP.0000000000000017. PMID: 24713986
Stromal and epithelial predominant Wilms tumours have an excellent outcome: the SIOP 93 01 experience.
Verschuur AC, Vujanic GM, Van Tinteren H, Jones KP, de Kraker J, Sandstedt B
Pediatr Blood Cancer 2010 Aug;55(2):233-8. doi: 10.1002/pbc.22496. PMID: 20582946
An immunohistochemical study comparing clear cell sarcoma of the kidney and Wilms' tumor.
Looi LM, Cheah PL
Pathology 1993 Apr;25(2):106-9. doi: 10.3109/00313029309084780. PMID: 8396229

Diagnosis

Response of bilateral Wilms tumor to chemotherapy suggests histologic subtype and guides treatment.
Duncan C, Sarvode Mothi S, Santiago TC, Coggins JA, Graetz DE, Bishop MW, Mullen EA, Murphy AJ, Green DM, Krasin MJ, Davidoff AM
J Natl Cancer Inst 2024 Aug 1;116(8):1230-1237. doi: 10.1093/jnci/djae072. PMID: 38539045Free PMC Article
Absence of the TRIP13 c.1060C>T Mutation in Wilms Tumor Patients From Pakistan.
Rashid MU, Naeemi H, Qazi AQ, Muhammad N, Ahmad M, Akhtar N, Rahman N
J Pediatr Hematol Oncol 2020 Apr;42(3):e128-e131. doi: 10.1097/MPH.0000000000001602. PMID: 31574018
"Teratoid" Wilms Tumor: The Extreme End of Heterologous Element Differentiation, Not a Separate Entity.
D'Hooghe E, Mifsud W, Vujanić GM
Am J Surg Pathol 2019 Nov;43(11):1583-1590. doi: 10.1097/PAS.0000000000001335. PMID: 31600178
Adult extrarenal Wilms' tumor mimicking mixed epithelial and stromal tumor in the retroperitoneum: a case report with immunohistochemical study and review of the literature.
Kadota K, Haba R, Kushida Y, Katsuki N, Hayashi T, Miyai Y, Bando K, Taoka R, Kakehi Y
Pathol Oncol Res 2009 Dec;15(4):665-9. Epub 2009 Apr 24 doi: 10.1007/s12253-009-9169-6. PMID: 19390992
An immunohistochemical study comparing clear cell sarcoma of the kidney and Wilms' tumor.
Looi LM, Cheah PL
Pathology 1993 Apr;25(2):106-9. doi: 10.3109/00313029309084780. PMID: 8396229

Therapy

Response of bilateral Wilms tumor to chemotherapy suggests histologic subtype and guides treatment.
Duncan C, Sarvode Mothi S, Santiago TC, Coggins JA, Graetz DE, Bishop MW, Mullen EA, Murphy AJ, Green DM, Krasin MJ, Davidoff AM
J Natl Cancer Inst 2024 Aug 1;116(8):1230-1237. doi: 10.1093/jnci/djae072. PMID: 38539045Free PMC Article
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
Stromal and epithelial predominant Wilms tumours have an excellent outcome: the SIOP 93 01 experience.
Verschuur AC, Vujanic GM, Van Tinteren H, Jones KP, de Kraker J, Sandstedt B
Pediatr Blood Cancer 2010 Aug;55(2):233-8. doi: 10.1002/pbc.22496. PMID: 20582946
Results of the SIOP 93-01/GPOH trial and study for the treatment of patients with unilateral nonmetastatic Wilms Tumor.
Reinhard H, Semler O, Bürger D, Bode U, Flentje M, Göbel U, Gutjahr P, Leuschner I, Maass E, Niggli F, Scheel-Walter HG, Stöckle M, Thüroff JW, Tröger J, Weirich A, von Schweinitz D, Zoubek A, Graf N
Klin Padiatr 2004 May-Jun;216(3):132-40. doi: 10.1055/s-2004-822625. PMID: 15175957
Serum hyaluronan and its association with unfavorable histology and aggressiveness of heterotransplanted Wilms' tumor.
Lovvorn HN 3rd, Savani RC, Ruchelli E, Cass DL, Adzick NS
J Pediatr Surg 2000 Jul;35(7):1070-8. doi: 10.1053/jpsu.2000.7774. PMID: 10917299

Prognosis

Response of bilateral Wilms tumor to chemotherapy suggests histologic subtype and guides treatment.
Duncan C, Sarvode Mothi S, Santiago TC, Coggins JA, Graetz DE, Bishop MW, Mullen EA, Murphy AJ, Green DM, Krasin MJ, Davidoff AM
J Natl Cancer Inst 2024 Aug 1;116(8):1230-1237. doi: 10.1093/jnci/djae072. PMID: 38539045Free PMC Article
"Teratoid" Wilms Tumor: The Extreme End of Heterologous Element Differentiation, Not a Separate Entity.
D'Hooghe E, Mifsud W, Vujanić GM
Am J Surg Pathol 2019 Nov;43(11):1583-1590. doi: 10.1097/PAS.0000000000001335. PMID: 31600178
Wilms tumor: an update.
Al-Hussain T, Ali A, Akhtar M
Adv Anat Pathol 2014 May;21(3):166-73. doi: 10.1097/PAP.0000000000000017. PMID: 24713986
Stromal and epithelial predominant Wilms tumours have an excellent outcome: the SIOP 93 01 experience.
Verschuur AC, Vujanic GM, Van Tinteren H, Jones KP, de Kraker J, Sandstedt B
Pediatr Blood Cancer 2010 Aug;55(2):233-8. doi: 10.1002/pbc.22496. PMID: 20582946
Serum hyaluronan and its association with unfavorable histology and aggressiveness of heterotransplanted Wilms' tumor.
Lovvorn HN 3rd, Savani RC, Ruchelli E, Cass DL, Adzick NS
J Pediatr Surg 2000 Jul;35(7):1070-8. doi: 10.1053/jpsu.2000.7774. PMID: 10917299

Clinical prediction guides

Response of bilateral Wilms tumor to chemotherapy suggests histologic subtype and guides treatment.
Duncan C, Sarvode Mothi S, Santiago TC, Coggins JA, Graetz DE, Bishop MW, Mullen EA, Murphy AJ, Green DM, Krasin MJ, Davidoff AM
J Natl Cancer Inst 2024 Aug 1;116(8):1230-1237. doi: 10.1093/jnci/djae072. PMID: 38539045Free PMC Article
Correlations between histological characterizations and methylation statuses of tumour suppressor genes in Wilms' tumours.
Lai YC, Lu MY, Wang WC, Hou TC, Kuo CY
Int J Exp Pathol 2022 Jun;103(3):121-128. Epub 2022 Apr 18 doi: 10.1111/iep.12442. PMID: 35436013Free PMC Article
A case report of infantile cystic nephroblastoma.
Kurose N, Takenaka M, Yamashita M, Shimaguchi C, Nakano M, Britni B, Guo X, Futatsuya C, Shioya A, Yamada S
Diagn Pathol 2018 Oct 27;13(1):84. doi: 10.1186/s13000-018-0761-5. PMID: 30368245Free PMC Article
Nephron Progenitor But Not Stromal Progenitor Cells Give Rise to Wilms Tumors in Mouse Models with β-Catenin Activation or Wt1 Ablation and Igf2 Upregulation.
Huang L, Mokkapati S, Hu Q, Ruteshouser EC, Hicks MJ, Huff V
Neoplasia 2016 Feb;18(2):71-81. doi: 10.1016/j.neo.2015.12.001. PMID: 26936393Free PMC Article
Serum hyaluronan and its association with unfavorable histology and aggressiveness of heterotransplanted Wilms' tumor.
Lovvorn HN 3rd, Savani RC, Ruchelli E, Cass DL, Adzick NS
J Pediatr Surg 2000 Jul;35(7):1070-8. doi: 10.1053/jpsu.2000.7774. PMID: 10917299

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