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Cystic partially differentiated nephroblastoma

MedGen UID:
220423
Concept ID:
C1266139
Neoplastic Process
Synonyms: Cystic Partially Differentiated Kidney Nephroblastoma; cystic partially differentiated nephroblastoma; Cystic partially differentiated nephroblastoma of kidney; Malignant Cystic Nephroma; Malignant cystic nephroma; malignant cystic nephroma; Malignant Multilocular Cystic Nephroma; Malignant multilocular cystic nephroma; malignant multilocular cystic nephroma
SNOMED CT: Cystic partially differentiated nephroblastoma (128758001); Malignant multilocular cystic nephroma (128759009); Malignant cystic nephroma (128759009); Cystic partially differentiated nephroblastoma of kidney (1148935009); Malignant cystic nephroma (1148852009)
 
Monarch Initiative: MONDO:0030604

Definition

A variant of Wilms tumor of the kidney characterized by the presence of cystic spaces separated by septa. The septa contain immature epithelial cells, immature stromal cells, and blastema cells. Surgical resection is usually curative. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCystic partially differentiated nephroblastoma

Professional guidelines

PubMed

Qureshi SS, Bhagat M, Verma K, Yadav S, Prasad M, Vora T, Chinnaswamy G, Amin N, Smriti V, Baheti A, Laskar S, Khanna N, Ramadwar M, Shah S
J Pediatr Urol 2020 Aug;16(4):475.e1-475.e9. Epub 2020 Jun 5 doi: 10.1016/j.jpurol.2020.05.168. PMID: 32620510
Luithle T, Szavay P, Furtwängler R, Graf N, Fuchs J; SIOP/GPOH Study Group
J Urol 2007 Jan;177(1):294-6. doi: 10.1016/j.juro.2006.09.011. PMID: 17162067
Joshi VV, Beckwith JB
Cancer 1989 Jul 15;64(2):466-79. doi: 10.1002/1097-0142(19890715)64:2<466::aid-cncr2820640221>3.0.co;2-v. PMID: 2544253

Recent clinical studies

Etiology

Das C, Mukhopadhyay M, Maity N, Parvin T, Mukhopadhyay B, Mukhopadhyay B
J Cancer Res Ther 2022 Jan-Mar;18(1):209-213. doi: 10.4103/jcrt.JCRT_436_19. PMID: 35381785
Cajaiba MM, Khanna G, Smith EA, Gellert L, Chi YY, Mullen EA, Hill DA, Geller JI, Dome JS, Perlman EJ
Hum Pathol 2016 Feb;48:81-7. Epub 2015 Oct 28 doi: 10.1016/j.humpath.2015.08.022. PMID: 26772403Free PMC Article
MdZin R, Murch A, Charles A
Pathology 2010 Dec;42(7):643-9. doi: 10.3109/00313025.2010.522171. PMID: 21080873
Luithle T, Szavay P, Furtwängler R, Graf N, Fuchs J; SIOP/GPOH Study Group
J Urol 2007 Jan;177(1):294-6. doi: 10.1016/j.juro.2006.09.011. PMID: 17162067
Blakely ML, Shamberger RC, Norkool P, Beckwith JB, Green DM, Ritchey ML; National Wilms' Tumor Study Group
J Pediatr Surg 2003 Jun;38(6):897-900. doi: 10.1016/s0022-3468(03)00118-0. PMID: 12778388

Diagnosis

Cajaiba MM, Khanna G, Smith EA, Gellert L, Chi YY, Mullen EA, Hill DA, Geller JI, Dome JS, Perlman EJ
Hum Pathol 2016 Feb;48:81-7. Epub 2015 Oct 28 doi: 10.1016/j.humpath.2015.08.022. PMID: 26772403Free PMC Article
Puvaneswary M, Macintosh J, Cassey J
Australas Radiol 2006 Jun;50(3):255-7. doi: 10.1111/j.1440-1673.2006.01574.x. PMID: 16732826
Truong LD, Choi YJ, Shen SS, Ayala G, Amato R, Krishnan B
Adv Anat Pathol 2003 May;10(3):135-59. doi: 10.1097/00125480-200305000-00003. PMID: 12717117
Eble JN, Bonsib SM
Semin Diagn Pathol 1998 Feb;15(1):2-20. PMID: 9503503
Brown JM
J Pathol 1975 Mar;115(3):175-8. doi: 10.1002/path.1711150307. PMID: 168329

Therapy

Baker JM, Viero S, Kim PC, Grant RM
Pediatr Blood Cancer 2008 Jan;50(1):129-31. doi: 10.1002/pbc.20930. PMID: 16786584
Blakely ML, Shamberger RC, Norkool P, Beckwith JB, Green DM, Ritchey ML; National Wilms' Tumor Study Group
J Pediatr Surg 2003 Jun;38(6):897-900. doi: 10.1016/s0022-3468(03)00118-0. PMID: 12778388
Schmidt D, Harms D, Leuschner I
Pathol Res Pract 1992 Feb;188(1-2):1-15. doi: 10.1016/S0344-0338(11)81150-7. PMID: 1317553
Ninane J, Gosseye S, Claus D, Otte JB, Cornu G
Helv Paediatr Acta 1981 Sep;36(4):377-82. PMID: 6268574
Keegan GT, Peterson RF, Stucki WJ, Street L
J Urol 1979 Mar;121(3):362-4. doi: 10.1016/s0022-5347(17)56787-7. PMID: 219264

Prognosis

Qureshi SS, Bhagat M, Verma K, Yadav S, Prasad M, Vora T, Chinnaswamy G, Amin N, Smriti V, Baheti A, Laskar S, Khanna N, Ramadwar M, Shah S
J Pediatr Urol 2020 Aug;16(4):475.e1-475.e9. Epub 2020 Jun 5 doi: 10.1016/j.jpurol.2020.05.168. PMID: 32620510
Khoury JD
Clin Lab Med 2005 Jun;25(2):341-61, vi-vii. doi: 10.1016/j.cll.2005.01.010. PMID: 15848740
Blakely ML, Shamberger RC, Norkool P, Beckwith JB, Green DM, Ritchey ML; National Wilms' Tumor Study Group
J Pediatr Surg 2003 Jun;38(6):897-900. doi: 10.1016/s0022-3468(03)00118-0. PMID: 12778388
Schmidt D, Harms D, Leuschner I
Pathol Res Pract 1992 Feb;188(1-2):1-15. doi: 10.1016/S0344-0338(11)81150-7. PMID: 1317553
Joshi VV
Perspect Pediatr Pathol 1979;5:217-35. PMID: 231757

Clinical prediction guides

Das C, Mukhopadhyay M, Maity N, Parvin T, Mukhopadhyay B, Mukhopadhyay B
J Cancer Res Ther 2022 Jan-Mar;18(1):209-213. doi: 10.4103/jcrt.JCRT_436_19. PMID: 35381785
Qureshi SS, Bhagat M, Verma K, Yadav S, Prasad M, Vora T, Chinnaswamy G, Amin N, Smriti V, Baheti A, Laskar S, Khanna N, Ramadwar M, Shah S
J Pediatr Urol 2020 Aug;16(4):475.e1-475.e9. Epub 2020 Jun 5 doi: 10.1016/j.jpurol.2020.05.168. PMID: 32620510
Tajima S, Waki M
Int J Clin Exp Pathol 2015;8(1):989-94. Epub 2015 Jan 1 PMID: 25755807Free PMC Article
Bahubeshi A, Bal N, Rio Frio T, Hamel N, Pouchet C, Yilmaz A, Bouron-Dal Soglio D, Williams GM, Tischkowitz M, Priest JR, Foulkes WD
J Med Genet 2010 Dec;47(12):863-6. Epub 2010 Oct 29 doi: 10.1136/jmg.2010.081216. PMID: 21036787
Furukawa T, Azakami S, Kurosawa H, Ono Y, Ueda Y, Konno Y
J Pediatr Hematol Oncol 2003 Nov;25(11):896-9. doi: 10.1097/00043426-200311000-00014. PMID: 14608201

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