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...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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MDPI AG
2019-07-01
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| Series: | Children |
| Subjects: | |
| Online Access: | https://www.mdpi.com/2227-9067/6/8/86 |
| _version_ | 1818615264115163136 |
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| author | Samantha Knight Tristan Knight Amir Khan Andrew J. Murphy |
| author_facet | Samantha Knight Tristan Knight Amir Khan Andrew J. Murphy |
| author_sort | Samantha Knight |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-12-16T16:31:08Z |
| format | Article |
| id | doaj.art-30a705dfe9bd46a19c5e8fde928b5131 |
| institution | Directory Open Access Journal |
| issn | 2227-9067 |
| language | English |
| last_indexed | 2024-12-16T16:31:08Z |
| publishDate | 2019-07-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Children |
| spelling | doaj.art-30a705dfe9bd46a19c5e8fde928b51312022-12-21T22:24:35ZengMDPI AGChildren2227-90672019-07-01688610.3390/children6080086children6080086Current Management of Pleuropulmonary Blastoma: A Surgical PerspectiveSamantha Knight0Tristan Knight1Amir Khan2Andrew J. Murphy3Southern Illinois University School of Medicine, Division of Surgery, Department of General Surgery, Springfield, IL 62702, USADivision of Pediatric Hematology and Oncology, Department of Pediatrics, Children’s Hospital of Michigan, Detroit, MI 48201, USASouthern Illinois University School of Medicine, Division of Surgery, Department of General Surgery, Springfield, IL 62702, USADepartment of Surgery, St. Jude Children’s Research Hospital, Memphis, TN 38105, USAPleuropulmonary 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.https://www.mdpi.com/2227-9067/6/8/86pleuropulmonary blastomacongenital pulmonary airway malformationscongenital cystic adenomatoid malformationsDICER1multidisciplinary managementsurgery |
| spellingShingle | Samantha Knight Tristan Knight Amir Khan Andrew J. Murphy Current Management of Pleuropulmonary Blastoma: A Surgical Perspective Children pleuropulmonary blastoma congenital pulmonary airway malformations congenital cystic adenomatoid malformations DICER1 multidisciplinary management surgery |
| 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 | pleuropulmonary blastoma congenital pulmonary airway malformations congenital cystic adenomatoid malformations DICER1 multidisciplinary management surgery |
| url | https://www.mdpi.com/2227-9067/6/8/86 |
| work_keys_str_mv | AT samanthaknight currentmanagementofpleuropulmonaryblastomaasurgicalperspective AT tristanknight currentmanagementofpleuropulmonaryblastomaasurgicalperspective AT amirkhan currentmanagementofpleuropulmonaryblastomaasurgicalperspective AT andrewjmurphy currentmanagementofpleuropulmonaryblastomaasurgicalperspective |