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Multiloculated Cystic Renal Tumors of Childhood: Has the Final Word Been Spoken

BACKGROUND: Multicystic renal tumors which include cystic nephroma, cystic partially differentiated nephroblastoma (CPDN) and cystic Wilms tumor has been a diagnostic and therapeutic challenge. Histopathological examination has been the only reliable differentiating method. Management of these tumor...

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Autores principales: Kurian, Jujju Jacob, Jehangir, Susan, Korula, Anila
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5772090/
https://www.ncbi.nlm.nih.gov/pubmed/29386760
http://dx.doi.org/10.4103/jiaps.JIAPS_224_16
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author Kurian, Jujju Jacob
Jehangir, Susan
Korula, Anila
author_facet Kurian, Jujju Jacob
Jehangir, Susan
Korula, Anila
author_sort Kurian, Jujju Jacob
collection PubMed
description BACKGROUND: Multicystic renal tumors which include cystic nephroma, cystic partially differentiated nephroblastoma (CPDN) and cystic Wilms tumor has been a diagnostic and therapeutic challenge. Histopathological examination has been the only reliable differentiating method. Management of these tumors is still riddled with controversy as a definitive preoperative differentiation between the three has not been possible. METHODS: A retrospective evaluation was performed of the treatment strategies employed with nine cases of multicystic renal tumors treated from 2005 to 2015. RESULTS: The median age at presentation was 12 months with all except one being boys. All except two children underwent primary surgery. The median follow-up was 50 months with six children having long-term survival. One child succumbed to the disease process, one died due to an unrelated cause and another was lost to follow-up. Although there was no ambiguity with cases of cystic nephroma (CN) and cystic Wilms tumor, three of the four cases of CPDN had problems. CONCLUSION: Primary surgery for multicystic renal tumors is safe and should be seriously considered as it prevents overtreatment in cases of CN and early stage CPDN. Further studies are needed to fully understand the biological behavior of CPDN.
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spelling pubmed-57720902018-01-31 Multiloculated Cystic Renal Tumors of Childhood: Has the Final Word Been Spoken Kurian, Jujju Jacob Jehangir, Susan Korula, Anila J Indian Assoc Pediatr Surg Original Article BACKGROUND: Multicystic renal tumors which include cystic nephroma, cystic partially differentiated nephroblastoma (CPDN) and cystic Wilms tumor has been a diagnostic and therapeutic challenge. Histopathological examination has been the only reliable differentiating method. Management of these tumors is still riddled with controversy as a definitive preoperative differentiation between the three has not been possible. METHODS: A retrospective evaluation was performed of the treatment strategies employed with nine cases of multicystic renal tumors treated from 2005 to 2015. RESULTS: The median age at presentation was 12 months with all except one being boys. All except two children underwent primary surgery. The median follow-up was 50 months with six children having long-term survival. One child succumbed to the disease process, one died due to an unrelated cause and another was lost to follow-up. Although there was no ambiguity with cases of cystic nephroma (CN) and cystic Wilms tumor, three of the four cases of CPDN had problems. CONCLUSION: Primary surgery for multicystic renal tumors is safe and should be seriously considered as it prevents overtreatment in cases of CN and early stage CPDN. Further studies are needed to fully understand the biological behavior of CPDN. Medknow Publications & Media Pvt Ltd 2018 /pmc/articles/PMC5772090/ /pubmed/29386760 http://dx.doi.org/10.4103/jiaps.JIAPS_224_16 Text en Copyright: © 2017 Journal of Indian Association of Pediatric Surgeons http://creativecommons.org/licenses/by-nc-sa/3.0 This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
spellingShingle Original Article
Kurian, Jujju Jacob
Jehangir, Susan
Korula, Anila
Multiloculated Cystic Renal Tumors of Childhood: Has the Final Word Been Spoken
title Multiloculated Cystic Renal Tumors of Childhood: Has the Final Word Been Spoken
title_full Multiloculated Cystic Renal Tumors of Childhood: Has the Final Word Been Spoken
title_fullStr Multiloculated Cystic Renal Tumors of Childhood: Has the Final Word Been Spoken
title_full_unstemmed Multiloculated Cystic Renal Tumors of Childhood: Has the Final Word Been Spoken
title_short Multiloculated Cystic Renal Tumors of Childhood: Has the Final Word Been Spoken
title_sort multiloculated cystic renal tumors of childhood: has the final word been spoken
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5772090/
https://www.ncbi.nlm.nih.gov/pubmed/29386760
http://dx.doi.org/10.4103/jiaps.JIAPS_224_16
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