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Current Management of Pleuropulmonary Blastoma: A Surgical Perspective
Pleuropulmonary blastomas (PPB) are pediatric, embryonal cancers of the lung parenchyma and pleural surfaces and are among the most common DICER1—related disorders. These tumors undergo evolution through several forms, allowing division into types I, Ir, II, and III, with correlates to the age of di...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6721434/ https://www.ncbi.nlm.nih.gov/pubmed/31349569 http://dx.doi.org/10.3390/children6080086 |
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author | Knight, Samantha Knight, Tristan Khan, Amir Murphy, Andrew J. |
author_facet | Knight, Samantha Knight, Tristan Khan, Amir Murphy, Andrew J. |
author_sort | Knight, Samantha |
collection | PubMed |
description | Pleuropulmonary blastomas (PPB) are pediatric, embryonal cancers of the lung parenchyma and pleural surfaces and are among the most common DICER1—related disorders. These tumors undergo evolution through several forms, allowing division into types I, Ir, II, and III, with correlates to the age of diagnosis and prognosis. We sought to provide a comprehensive review of the relevant literature describing the characteristics of these tumors and their multidisciplinary treatment, with an emphasis on surgical management. We describe the complementary roles of chemotherapy and surgery in the successful management of this disease. We discuss the timing of surgery and options for surgical approaches. We address the differentiation of PPB from congenital pulmonary airway malformation and the role of DICER1 testing for children with pulmonary cysts. |
format | Online Article Text |
id | pubmed-6721434 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-67214342019-09-10 Current Management of Pleuropulmonary Blastoma: A Surgical Perspective Knight, Samantha Knight, Tristan Khan, Amir Murphy, Andrew J. Children (Basel) Review Pleuropulmonary blastomas (PPB) are pediatric, embryonal cancers of the lung parenchyma and pleural surfaces and are among the most common DICER1—related disorders. These tumors undergo evolution through several forms, allowing division into types I, Ir, II, and III, with correlates to the age of diagnosis and prognosis. We sought to provide a comprehensive review of the relevant literature describing the characteristics of these tumors and their multidisciplinary treatment, with an emphasis on surgical management. We describe the complementary roles of chemotherapy and surgery in the successful management of this disease. We discuss the timing of surgery and options for surgical approaches. We address the differentiation of PPB from congenital pulmonary airway malformation and the role of DICER1 testing for children with pulmonary cysts. MDPI 2019-07-25 /pmc/articles/PMC6721434/ /pubmed/31349569 http://dx.doi.org/10.3390/children6080086 Text en © 2019 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Knight, Samantha Knight, Tristan Khan, Amir Murphy, Andrew J. Current Management of Pleuropulmonary Blastoma: A Surgical Perspective |
title | Current Management of Pleuropulmonary Blastoma: A Surgical Perspective |
title_full | Current Management of Pleuropulmonary Blastoma: A Surgical Perspective |
title_fullStr | Current Management of Pleuropulmonary Blastoma: A Surgical Perspective |
title_full_unstemmed | Current Management of Pleuropulmonary Blastoma: A Surgical Perspective |
title_short | Current Management of Pleuropulmonary Blastoma: A Surgical Perspective |
title_sort | current management of pleuropulmonary blastoma: a surgical perspective |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6721434/ https://www.ncbi.nlm.nih.gov/pubmed/31349569 http://dx.doi.org/10.3390/children6080086 |
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