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Childhood multilocular cystic kidney neoplasm

MedGen UID:
232064
•Concept ID:
C1332983
•
Neoplastic Process
Synonyms: benign multilocular cystic renal tumor; benign multilocular cystic renal tumour; Childhood Multilocular Cystic Kidney Neoplasm; childhood multilocular cystic kidney neoplasm; Childhood Multilocular Cystic Kidney Tumor; childhood multilocular cystic kidney tumor; childhood multilocular cystic kidney tumour; Childhood Multilocular Cystic Renal Neoplasm; childhood multilocular cystic renal neoplasm; Childhood Multilocular Cystic Renal Tumor; childhood multilocular cystic renal tumor; childhood multilocular cystic renal tumour; paediatric multilocular cystic kidney neoplasm; paediatric multilocular cystic kidney tumour; paediatric multilocular cystic renal neoplasm; paediatric multilocular cystic renal tumour; Pediatric Multilocular Cystic Kidney Neoplasm; pediatric multilocular cystic kidney neoplasm; Pediatric Multilocular Cystic Kidney Tumor; pediatric multilocular cystic kidney tumor; Pediatric Multilocular Cystic Renal Neoplasm; pediatric multilocular cystic renal neoplasm; Pediatric Multilocular Cystic Renal Tumor; pediatric multilocular cystic renal tumor
 
Monarch Initiative: MONDO:0004356

Definition

A cystic neoplasm which arises from the kidney and occurs in children. It includes the cystic partially differentiated nephroblastoma and cases in which nephroblastomatous elements are not present. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChildhood multilocular cystic kidney neoplasm

Recent clinical studies

Etiology

DICER1 mutations in childhood cystic nephroma and its relationship to DICER1-renal sarcoma.
Doros LA, Rossi CT, Yang J, Field A, Williams GM, Messinger Y, Cajaiba MM, Perlman EJ, A Schultz K, Cathro HP, Legallo RD, LaFortune KA, Chikwava KR, Faria P, Geller JI, Dome JS, Mullen EA, Gratias EJ, Dehner LP, Hill DA
Mod Pathol 2014 Sep;27(9):1267-80. Epub 2014 Jan 31 doi: 10.1038/modpathol.2013.242. PMID: 24481001Free PMC Article

Diagnosis

Uretheral invagination of multilocular cystic nephroma; a case report of a new pathologic variant.
Ozturk H, Karaaslan S
Int J Clin Exp Pathol 2014;7(8):5271-9. Epub 2014 Jul 15 PMID: 25197409Free PMC Article
Renal neoplasms of childhood.
Geller E, Smergel EM, Lowry PA
Radiol Clin North Am 1997 Nov;35(6):1391-413. PMID: 9374996
Multilocular cystic renal tumor in children: radiologic-pathologic correlation.
Agrons GA, Wagner BJ, Davidson AJ, Suarez ES
Radiographics 1995 May;15(3):653-69. doi: 10.1148/radiographics.15.3.7624570. PMID: 7624570
N-myc oncogene expression in histopathologically unrelated bilateral pediatric renal tumors.
Nisen PD, Rich MA, Gloster E, Valderrama E, Saric O, Shende A, Lanzkowsky P, Alt FW
Cancer 1988 May 1;61(9):1821-6. doi: 10.1002/1097-0142(19880501)61:9<1821::aid-cncr2820610917>3.0.co;2-t. PMID: 2833341
Multilocular cystic nephroma: report of three cases.
Garrett A, Carty H, Pilling D
Clin Radiol 1987 Jan;38(1):55-7. doi: 10.1016/s0009-9260(87)80404-x. PMID: 3028697

Prognosis

Non-Wilms' renal tumors in children.
Broecker B
Urol Clin North Am 2000 Aug;27(3):463-9, ix. doi: 10.1016/s0094-0143(05)70094-x. PMID: 10985146
Multilocular cystic renal tumor in children: radiologic-pathologic correlation.
Agrons GA, Wagner BJ, Davidson AJ, Suarez ES
Radiographics 1995 May;15(3):653-69. doi: 10.1148/radiographics.15.3.7624570. PMID: 7624570
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
N-myc oncogene expression in histopathologically unrelated bilateral pediatric renal tumors.
Nisen PD, Rich MA, Gloster E, Valderrama E, Saric O, Shende A, Lanzkowsky P, Alt FW
Cancer 1988 May 1;61(9):1821-6. doi: 10.1002/1097-0142(19880501)61:9<1821::aid-cncr2820610917>3.0.co;2-t. PMID: 2833341

Clinical prediction guides

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
N-myc oncogene expression in histopathologically unrelated bilateral pediatric renal tumors.
Nisen PD, Rich MA, Gloster E, Valderrama E, Saric O, Shende A, Lanzkowsky P, Alt FW
Cancer 1988 May 1;61(9):1821-6. doi: 10.1002/1097-0142(19880501)61:9<1821::aid-cncr2820610917>3.0.co;2-t. PMID: 2833341

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