Perilobar Nephroblastomatosis: Natural History and Management

Nephroblastomatosis (NB) has been considered as a precursor of Wilms tumor (WT). The natural history of NB seems to present significant variation as some lesions may regress spontaneously, while others may grow and expand or relapse and develop into WT later in childhood. Although, most investigator...

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Detalles Bibliográficos
Autores principales: Stabouli, S., Printza, N., Dotis, J., Matis, A., Koliouskas, D., Gombakis, N., Papachristou, F.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2014
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4120796/
https://www.ncbi.nlm.nih.gov/pubmed/25114825
http://dx.doi.org/10.1155/2014/756819
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author Stabouli, S.
Printza, N.
Dotis, J.
Matis, A.
Koliouskas, D.
Gombakis, N.
Papachristou, F.
author_facet Stabouli, S.
Printza, N.
Dotis, J.
Matis, A.
Koliouskas, D.
Gombakis, N.
Papachristou, F.
author_sort Stabouli, S.
collection PubMed
description Nephroblastomatosis (NB) has been considered as a precursor of Wilms tumor (WT). The natural history of NB seems to present significant variation as some lesions may regress spontaneously, while others may grow and expand or relapse and develop into WT later in childhood. Although, most investigators suggest adjutant chemotherapy, the effect and duration of treatment are not well established. Children with diffuse perilobar NB, Beckwith-Wiedemann syndrome, and hemihypertrophy seem to particularly benefit from treatment. We discuss our experience on two cases of NB and we review the literature for the management of this rare condition.
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spelling pubmed-41207962014-08-11 Perilobar Nephroblastomatosis: Natural History and Management Stabouli, S. Printza, N. Dotis, J. Matis, A. Koliouskas, D. Gombakis, N. Papachristou, F. Case Rep Pediatr Case Report Nephroblastomatosis (NB) has been considered as a precursor of Wilms tumor (WT). The natural history of NB seems to present significant variation as some lesions may regress spontaneously, while others may grow and expand or relapse and develop into WT later in childhood. Although, most investigators suggest adjutant chemotherapy, the effect and duration of treatment are not well established. Children with diffuse perilobar NB, Beckwith-Wiedemann syndrome, and hemihypertrophy seem to particularly benefit from treatment. We discuss our experience on two cases of NB and we review the literature for the management of this rare condition. Hindawi Publishing Corporation 2014 2014-07-09 /pmc/articles/PMC4120796/ /pubmed/25114825 http://dx.doi.org/10.1155/2014/756819 Text en Copyright © 2014 S. Stabouli et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Stabouli, S.
Printza, N.
Dotis, J.
Matis, A.
Koliouskas, D.
Gombakis, N.
Papachristou, F.
Perilobar Nephroblastomatosis: Natural History and Management
title Perilobar Nephroblastomatosis: Natural History and Management
title_full Perilobar Nephroblastomatosis: Natural History and Management
title_fullStr Perilobar Nephroblastomatosis: Natural History and Management
title_full_unstemmed Perilobar Nephroblastomatosis: Natural History and Management
title_short Perilobar Nephroblastomatosis: Natural History and Management
title_sort perilobar nephroblastomatosis: natural history and management
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4120796/
https://www.ncbi.nlm.nih.gov/pubmed/25114825
http://dx.doi.org/10.1155/2014/756819
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AT koliouskasd perilobarnephroblastomatosisnaturalhistoryandmanagement
AT gombakisn perilobarnephroblastomatosisnaturalhistoryandmanagement
AT papachristouf perilobarnephroblastomatosisnaturalhistoryandmanagement

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