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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...

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Detalles Bibliográficos
Autores principales: Knight, Samantha, Knight, Tristan, Khan, Amir, Murphy, Andrew J.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2019
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.
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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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