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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

Incidence, treatment, and outcomes of primary and recurrent Non-Wilms renal tumors in children: Report of 109 patients treated at a single institution.
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
Treatment of cystic nephroma and cystic partially differentiated nephroblastoma--a report from the SIOP/GPOH study group.
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
Multilocular cyst of the kidney (cystic nephroma) and cystic, partially differentiated nephroblastoma. Terminology and criteria for diagnosis.
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

Spectrum of pediatric kidney tumours with special references to WT1 immunostain at a tertiary care hospital.
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
Incidence, treatment, and outcomes of primary and recurrent Non-Wilms renal tumors in children: Report of 109 patients treated at a single institution.
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
Pediatric cystic nephromas: distinctive features and frequent DICER1 mutations.
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
Treatment of cystic nephroma and cystic partially differentiated nephroblastoma--a report from the SIOP/GPOH study group.
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
Cystic partially differentiated nephroblastoma, embryonal rhabdomyosarcoma, and multiple congenital anomalies associated with variegated mosaic aneuploidy and premature centromere division: a case report.
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

Diagnosis

Cystic partially differentiated nephroblastoma with ureteric extension.
Kumar VR, Rajamani G, Raman ML
Indian J Pathol Microbiol 2010 Oct-Dec;53(4):831-2. doi: 10.4103/0377-4929.72067. PMID: 21045434
Nephron-sparing surgery for multilocular cyst of the kidney in a child.
Okada T, Yoshida H, Matsunaga T, Kouchi K, Ohtsuka Y, Saitou T, Horie H, Ohnuma N
J Pediatr Surg 2003 Nov;38(11):1689-92. doi: 10.1016/s0022-3468(03)00589-x. PMID: 14614728
Renal cystic neoplasms and renal neoplasms associated with cystic renal diseases: pathogenetic and molecular links.
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
Cystic partially differentiated nephroblastoma. A case report.
Tiryaki T, Hücümenoğlu S, Livanelioğlu Z, Atayurt H
Urol Int 2003;70(3):223-6. doi: 10.1159/000068754. PMID: 12660462
Clinical quiz. Cystic partially differentiated nephroblastoma (CPDN).
Kingwatanakul P, Warady BA
Pediatr Nephrol 1996 Oct;10(5):675-6. doi: 10.1007/BF03035425. PMID: 8897583

Therapy

Stage III cystic partially differentiated nephroblastoma recurring after nephrectomy and chemotherapy.
Baker JM, Viero S, Kim PC, Grant RM
Pediatr Blood Cancer 2008 Jan;50(1):129-31. doi: 10.1002/pbc.20930. PMID: 16786584
Outcome of children with cystic partially differentiated nephroblastoma treated with or without chemotherapy.
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
Malignant renal tumors of childhood.
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
Cystic partially differentiated nephroblastoma.
Ninane J, Gosseye S, Claus D, Otte JB, Cornu G
Helv Paediatr Acta 1981 Sep;36(4):377-82. PMID: 6268574
Case report: cystic partially differentiated nephroblastoma (Wilms tumor).
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

Incidence, treatment, and outcomes of primary and recurrent Non-Wilms renal tumors in children: Report of 109 patients treated at a single institution.
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
Germline DICER1 mutations and familial cystic nephroma.
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
Nephroblastic neoplasms.
Khoury JD
Clin Lab Med 2005 Jun;25(2):341-61, vi-vii. doi: 10.1016/j.cll.2005.01.010. PMID: 15848740
Outcome of children with cystic partially differentiated nephroblastoma treated with or without chemotherapy.
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
Pathologic delineation of the papillonodular type of cystic partially differentiated nephroblastoma. A review of 11 cases.
Joshi VV, Beckwith JB
Cancer 1990 Oct 1;66(7):1568-77. doi: 10.1002/1097-0142(19901001)66:7<1568::aid-cncr2820660723>3.0.co;2-c. PMID: 2169993

Clinical prediction guides

Spectrum of pediatric kidney tumours with special references to WT1 immunostain at a tertiary care hospital.
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
Cystic partially differentiated nephroblastoma in an adult: a case imitating the process of normal nephrogenesis along with corresponding WT1 expression.
Tajima S, Waki M
Int J Clin Exp Pathol 2015;8(1):989-94. Epub 2015 Jan 1 PMID: 25755807Free PMC Article
Germline DICER1 mutations and familial cystic nephroma.
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
Cystic partially differentiated nephroblastoma, embryonal rhabdomyosarcoma, and multiple congenital anomalies associated with variegated mosaic aneuploidy and premature centromere division: a case report.
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
Hyperdiploidy including trisomy 8 in a cystic partially differentiated nephroblastoma.
Timmons CF, McGavran L, Unterkircher L, Beckwith JB, Wilson HL
Cancer Genet Cytogenet 1989 Aug;41(1):79-85. doi: 10.1016/0165-4608(89)90110-6. PMID: 2548706

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