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Perilobar nephroblastomatosis

MedGen UID:
1640586
•Concept ID:
C4551836
•
Neoplastic Process
HPO: HP:0011796

Definition

Abnormally persistent foci of embryonal immature blastema located in the superficial cortical region (perilobar). [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPerilobar nephroblastomatosis

Professional guidelines

PubMed

Alterations of miRNA Expression in Diffuse Hyperplastic Perilobar Nephroblastomatosis: Mapping the Way to Understanding Wilms' Tumor Development and Differential Diagnosis.
Csók Á, Micsik T, Magyar Z, Tornóczky T, Kuthi L, Nishi Y, Szirák K, Csóka M, Ottóffy G, Soltész B, Balogh I, Buglyó G
Int J Mol Sci 2023 May 15;24(10) doi: 10.3390/ijms24108793. PMID: 37240139Free PMC Article

Recent clinical studies

Etiology

ASO Video Abstract: Kidney Preservation and Wilms Tumor Development in Children with Diffuse Hyperplastic Perilobar Nephroblastomatosis-A Report from the Children's Oncology Group Study AREN0534.
Ehrlich PF, Tornwall B, Chintagumpala MM, Chi YY, Hoffer FA, Perlman EJ, Kalapurakal JA, Warwick A, Shamberger RC, Khanna G, Hamilton TE, Gow KW, Paulino AC, Gratias EJ, Mullen EA, Geller JI, Fernandez CV, Dome JS
Ann Surg Oncol 2022 May;29(5):3262-3263. doi: 10.1245/s10434-021-11311-4. PMID: 35076770
Kidney Preservation and Wilms Tumor Development in Children with Diffuse Hyperplastic Perilobar Nephroblastomatosis: A Report from the Children's Oncology Group Study AREN0534.
Ehrlich PF, Tornwall B, Chintagumpala MM, Chi YY, Hoffer FA, Perlman EJ, Kalapurakal JA, Warwick A, Shamberger RC, Khanna G, Hamilton TE, Gow KW, Paulino AC, Gratias EJ, Mullen EA, Geller JI, Fernandez CV, Dome JS
Ann Surg Oncol 2022 May;29(5):3252-3261. Epub 2022 Jan 24 doi: 10.1245/s10434-021-11266-6. PMID: 35072864Free PMC Article
Imaging Characteristics of Nephrogenic Rests Versus Small Wilms Tumors: A Report From the Children's Oncology Group Study AREN03B2.
Sandberg JK, Chi YY, Smith EA, Servaes S, Hoffer FA, Mullen EA, Perlman EJ, Tornwall B, Ehrlich PF, Geller JI, Grundy PE, Fernandez CV, Dome JS, Khanna G
AJR Am J Roentgenol 2020 May;214(5):987-994. Epub 2020 Mar 11 doi: 10.2214/AJR.19.22301. PMID: 32160052Free PMC Article
Hyperplastic perilobar nephroblastomatosis: long-term survival of 52 patients.
Perlman EJ, Faria P, Soares A, Hoffer F, Sredni S, Ritchey M, Shamberger RC, Green D, Beckwith JB; National Wilms Tumor Study Group
Pediatr Blood Cancer 2006 Feb;46(2):203-21. doi: 10.1002/pbc.20386. PMID: 15816029
Pediatric renal tumors: practical updates for the pathologist.
Perlman EJ
Pediatr Dev Pathol 2005 May-Jun;8(3):320-38. Epub 2005 Jul 14 doi: 10.1007/s10024-005-1156-7. PMID: 16010493

Diagnosis

Alterations of miRNA Expression in Diffuse Hyperplastic Perilobar Nephroblastomatosis: Mapping the Way to Understanding Wilms' Tumor Development and Differential Diagnosis.
Csók Á, Micsik T, Magyar Z, Tornóczky T, Kuthi L, Nishi Y, Szirák K, Csóka M, Ottóffy G, Soltész B, Balogh I, Buglyó G
Int J Mol Sci 2023 May 15;24(10) doi: 10.3390/ijms24108793. PMID: 37240139Free PMC Article
Kidney Preservation and Wilms Tumor Development in Children with Diffuse Hyperplastic Perilobar Nephroblastomatosis: A Report from the Children's Oncology Group Study AREN0534.
Ehrlich PF, Tornwall B, Chintagumpala MM, Chi YY, Hoffer FA, Perlman EJ, Kalapurakal JA, Warwick A, Shamberger RC, Khanna G, Hamilton TE, Gow KW, Paulino AC, Gratias EJ, Mullen EA, Geller JI, Fernandez CV, Dome JS
Ann Surg Oncol 2022 May;29(5):3252-3261. Epub 2022 Jan 24 doi: 10.1245/s10434-021-11266-6. PMID: 35072864Free PMC Article
Imaging Characteristics of Nephrogenic Rests Versus Small Wilms Tumors: A Report From the Children's Oncology Group Study AREN03B2.
Sandberg JK, Chi YY, Smith EA, Servaes S, Hoffer FA, Mullen EA, Perlman EJ, Tornwall B, Ehrlich PF, Geller JI, Grundy PE, Fernandez CV, Dome JS, Khanna G
AJR Am J Roentgenol 2020 May;214(5):987-994. Epub 2020 Mar 11 doi: 10.2214/AJR.19.22301. PMID: 32160052Free PMC Article
Case report: Diffuse hyperplastic perilobar nephroblastomatosis complicated by a unilateral Wilms tumour: diagnosis, treatment and follow-up.
Gao B, Nzekwu E, Cook AJ, Spaner SJ
BMC Res Notes 2018 Jun 19;11(1):396. doi: 10.1186/s13104-018-3502-7. PMID: 29914549Free PMC Article
Hyperplastic perilobar nephroblastomatosis: long-term survival of 52 patients.
Perlman EJ, Faria P, Soares A, Hoffer F, Sredni S, Ritchey M, Shamberger RC, Green D, Beckwith JB; National Wilms Tumor Study Group
Pediatr Blood Cancer 2006 Feb;46(2):203-21. doi: 10.1002/pbc.20386. PMID: 15816029

Therapy

Kidney Preservation and Wilms Tumor Development in Children with Diffuse Hyperplastic Perilobar Nephroblastomatosis: A Report from the Children's Oncology Group Study AREN0534.
Ehrlich PF, Tornwall B, Chintagumpala MM, Chi YY, Hoffer FA, Perlman EJ, Kalapurakal JA, Warwick A, Shamberger RC, Khanna G, Hamilton TE, Gow KW, Paulino AC, Gratias EJ, Mullen EA, Geller JI, Fernandez CV, Dome JS
Ann Surg Oncol 2022 May;29(5):3252-3261. Epub 2022 Jan 24 doi: 10.1245/s10434-021-11266-6. PMID: 35072864Free PMC Article
Laparoscopic nephron-sparing resection of synchronous Wilms tumors in a case of hyperplastic perilobar nephroblastomatosis.
Rauth TP, Slone J, Crane G, Correa H, Friedman DL, Lovvorn HN 3rd
J Pediatr Surg 2011 May;46(5):983-8. doi: 10.1016/j.jpedsurg.2011.01.025. PMID: 21616266Free PMC Article

Prognosis

Case report: Diffuse hyperplastic perilobar nephroblastomatosis complicated by a unilateral Wilms tumour: diagnosis, treatment and follow-up.
Gao B, Nzekwu E, Cook AJ, Spaner SJ
BMC Res Notes 2018 Jun 19;11(1):396. doi: 10.1186/s13104-018-3502-7. PMID: 29914549Free PMC Article
Laparoscopic nephron-sparing resection of synchronous Wilms tumors in a case of hyperplastic perilobar nephroblastomatosis.
Rauth TP, Slone J, Crane G, Correa H, Friedman DL, Lovvorn HN 3rd
J Pediatr Surg 2011 May;46(5):983-8. doi: 10.1016/j.jpedsurg.2011.01.025. PMID: 21616266Free PMC Article
Hyperplastic perilobar nephroblastomatosis: long-term survival of 52 patients.
Perlman EJ, Faria P, Soares A, Hoffer F, Sredni S, Ritchey M, Shamberger RC, Green D, Beckwith JB; National Wilms Tumor Study Group
Pediatr Blood Cancer 2006 Feb;46(2):203-21. doi: 10.1002/pbc.20386. PMID: 15816029
Mosaic duplication 1(q11q44) in an infant with nephroblastomatosis and mineralization of extraplacental membranes.
Christiansen LR, Lage JM, Wolff DJ, Pai GS, Harley RA
Pediatr Dev Pathol 2005 Jan-Feb;8(1):115-23. Epub 2004 Dec 6 doi: 10.1007/s10024-003-9098-4. PMID: 15803218
Epidemiological and clinical correlations with genetic characteristics of Wilms' tumor: results of the Brazilian Wilms' Tumor Study Group.
Franco EL, de Camargo B, Saba L, Marques LA
Int J Cancer 1991 Jul 9;48(5):641-6. doi: 10.1002/ijc.2910480502. PMID: 1649136

Clinical prediction guides

Alterations of miRNA Expression in Diffuse Hyperplastic Perilobar Nephroblastomatosis: Mapping the Way to Understanding Wilms' Tumor Development and Differential Diagnosis.
Csók Á, Micsik T, Magyar Z, Tornóczky T, Kuthi L, Nishi Y, Szirák K, Csóka M, Ottóffy G, Soltész B, Balogh I, Buglyó G
Int J Mol Sci 2023 May 15;24(10) doi: 10.3390/ijms24108793. PMID: 37240139Free PMC Article
Kidney Preservation and Wilms Tumor Development in Children with Diffuse Hyperplastic Perilobar Nephroblastomatosis: A Report from the Children's Oncology Group Study AREN0534.
Ehrlich PF, Tornwall B, Chintagumpala MM, Chi YY, Hoffer FA, Perlman EJ, Kalapurakal JA, Warwick A, Shamberger RC, Khanna G, Hamilton TE, Gow KW, Paulino AC, Gratias EJ, Mullen EA, Geller JI, Fernandez CV, Dome JS
Ann Surg Oncol 2022 May;29(5):3252-3261. Epub 2022 Jan 24 doi: 10.1245/s10434-021-11266-6. PMID: 35072864Free PMC Article
Panlobar nephroblastomatosis with cystic dysplasia: an unusual case with diffuse renal involvement studied by immunohistochemistry.
Gaulier A, Boccon-Gibod L, Sabatier P, Lucas G
Pediatr Pathol 1993 Nov-Dec;13(6):741-9. doi: 10.3109/15513819309048261. PMID: 8108294
Epidemiological and clinical correlations with genetic characteristics of Wilms' tumor: results of the Brazilian Wilms' Tumor Study Group.
Franco EL, de Camargo B, Saba L, Marques LA
Int J Cancer 1991 Jul 9;48(5):641-6. doi: 10.1002/ijc.2910480502. PMID: 1649136

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