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Childhood Cystic Nephroma

MedGen UID:
1683122
•Concept ID:
C4763824
•
Neoplastic Process
Synonym: Pediatric Cystic Nephroma

Definition

A cystic nephroma that occurs in very young children. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChildhood Cystic Nephroma

Professional guidelines

PubMed

DICER1-associated Tumors in the Female Genital Tract: Molecular Basis, Clinicopathologic Features, and Differential Diagnosis.
Han LM, Weiel JJ, Longacre TA, Folkins AK
Adv Anat Pathol 2022 Sep 1;29(5):297-308. Epub 2022 Jul 1 doi: 10.1097/PAP.0000000000000351. PMID: 35778792
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

Recent clinical studies

Etiology

DICER1-associated Tumors in the Female Genital Tract: Molecular Basis, Clinicopathologic Features, and Differential Diagnosis.
Han LM, Weiel JJ, Longacre TA, Folkins AK
Adv Anat Pathol 2022 Sep 1;29(5):297-308. Epub 2022 Jul 1 doi: 10.1097/PAP.0000000000000351. PMID: 35778792
DICER1 and Associated Conditions: Identification of At-risk Individuals and Recommended Surveillance Strategies.
Schultz KAP, Williams GM, Kamihara J, Stewart DR, Harris AK, Bauer AJ, Turner J, Shah R, Schneider K, Schneider KW, Carr AG, Harney LA, Baldinger S, Frazier AL, Orbach D, Schneider DT, Malkin D, Dehner LP, Messinger YH, Hill DA
Clin Cancer Res 2018 May 15;24(10):2251-2261. Epub 2018 Jan 17 doi: 10.1158/1078-0432.CCR-17-3089. PMID: 29343557Free PMC Article
PTEN, DICER1, FH, and Their Associated Tumor Susceptibility Syndromes: Clinical Features, Genetics, and Surveillance Recommendations in Childhood.
Schultz KAP, Rednam SP, Kamihara J, Doros L, Achatz MI, Wasserman JD, Diller LR, Brugières L, Druker H, Schneider KA, McGee RB, Foulkes WD
Clin Cancer Res 2017 Jun 15;23(12):e76-e82. doi: 10.1158/1078-0432.CCR-17-0629. PMID: 28620008
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
Pulmonary cysts in early childhood and the risk of malignancy.
Priest JR, Williams GM, Hill DA, Dehner LP, Jaffé A
Pediatr Pulmonol 2009 Jan;44(1):14-30. doi: 10.1002/ppul.20917. PMID: 19061226

Diagnosis

Imaging of DICER1 syndrome.
Guillerman RP, Foulkes WD, Priest JR
Pediatr Radiol 2019 Oct;49(11):1488-1505. Epub 2019 Oct 16 doi: 10.1007/s00247-019-04429-x. PMID: 31620849
DICER1 and Associated Conditions: Identification of At-risk Individuals and Recommended Surveillance Strategies.
Schultz KAP, Williams GM, Kamihara J, Stewart DR, Harris AK, Bauer AJ, Turner J, Shah R, Schneider K, Schneider KW, Carr AG, Harney LA, Baldinger S, Frazier AL, Orbach D, Schneider DT, Malkin D, Dehner LP, Messinger YH, Hill DA
Clin Cancer Res 2018 May 15;24(10):2251-2261. Epub 2018 Jan 17 doi: 10.1158/1078-0432.CCR-17-3089. PMID: 29343557Free PMC Article
Pulmonary cysts in early childhood and the risk of malignancy.
Priest JR, Williams GM, Hill DA, Dehner LP, Jaffé A
Pediatr Pulmonol 2009 Jan;44(1):14-30. doi: 10.1002/ppul.20917. PMID: 19061226
Imaging of renal tumours in infancy and childhood.
Riccabona M
Eur Radiol 2003 Dec;13 Suppl 4:L116-29. doi: 10.1007/s00330-003-2001-x. PMID: 15018176
Renal neoplasms of childhood.
Geller E, Smergel EM, Lowry PA
Radiol Clin North Am 1997 Nov;35(6):1391-413. PMID: 9374996

Therapy

Cystic variant of favorable-histology Wilms' tumor presenting with osteolytic metastasis to the ribs.
Bindhu J, Imtiaz A, Kumar RV, Thejaswini MD
J Postgrad Med 2010 Jan-Mar;56(1):28-30. doi: 10.4103/0022-3859.62436. PMID: 20393247
A 23-year experience with malignant renal tumors in infancy and childhood.
Tröbs RB, Hänsel M, Friedrich T, Bennek J
Eur J Pediatr Surg 2001 Apr;11(2):92-8. doi: 10.1055/s-2001-13793. PMID: 11371043
Pleuropulmonary blastoma: a rare pathology with an even rarer presentation.
Lallier M, Bouchard S, Di Lorenzo M, Youssef S, Blanchard H, Lapierre JG, Vischoff D, Tucci M, Brochu P
J Pediatr Surg 1999 Jul;34(7):1057-9. doi: 10.1016/s0022-3468(99)90563-8. PMID: 10442587
Pleuropulmonary blastoma: a clinicopathologic study of 50 cases.
Priest JR, McDermott MB, Bhatia S, Watterson J, Manivel JC, Dehner LP
Cancer 1997 Jul 1;80(1):147-61. PMID: 9210721
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

Prognosis

DICER1 tumor predisposition syndrome: an evolving story initiated with the pleuropulmonary blastoma.
González IA, Stewart DR, Schultz KAP, Field AP, Hill DA, Dehner LP
Mod Pathol 2022 Jan;35(1):4-22. Epub 2021 Oct 1 doi: 10.1038/s41379-021-00905-8. PMID: 34599283Free PMC Article
Pulmonary cysts in early childhood and the risk of malignancy.
Priest JR, Williams GM, Hill DA, Dehner LP, Jaffé A
Pediatr Pulmonol 2009 Jan;44(1):14-30. doi: 10.1002/ppul.20917. PMID: 19061226
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
Congenital cystic mesoblastic nephroma.
Campagnola S, Fasoli L, Flessati P, Sulpasso M, Balter R, Pea M, Caudana R
Urol Int 1998;61(4):254-6. doi: 10.1159/000030342. PMID: 10364762
Congenital cystic mesoblastic nephroma: a rare cystic renal tumour of childhood. Case report.
Vujanić GM
Scand J Urol Nephrol 1992;26(3):315-7. doi: 10.3109/00365599209180892. PMID: 1332189

Clinical prediction guides

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
Imaging of DICER1 syndrome.
Guillerman RP, Foulkes WD, Priest JR
Pediatr Radiol 2019 Oct;49(11):1488-1505. Epub 2019 Oct 16 doi: 10.1007/s00247-019-04429-x. PMID: 31620849
Pulmonary cysts in early childhood and the risk of malignancy.
Priest JR, Williams GM, Hill DA, Dehner LP, Jaffé A
Pediatr Pulmonol 2009 Jan;44(1):14-30. doi: 10.1002/ppul.20917. PMID: 19061226
Pleuropulmonary blastoma in an adult: an initial case report.
Hill DA, Sadeghi S, Schultz MZ, Burr JS, Dehner LP
Cancer 1999 Jun 1;85(11):2368-74. PMID: 10357407
Pleuropulmonary blastoma: a clinicopathologic study of 50 cases.
Priest JR, McDermott MB, Bhatia S, Watterson J, Manivel JC, Dehner LP
Cancer 1997 Jul 1;80(1):147-61. PMID: 9210721

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