Extrapulmonary pleuropulmonary blastoma in a 3-year-old child: A case report and review of literature
Pleuropulmonary blastomas (PPBs) are very rare, highly aggressive, dysembryonic neoplasms of thoracopulmonary mesenchyme. These have been reported in the pediatric population and account for only 0.5%–1% of all primary malignant lung cancers. They normally arise from lung tissue, however rarely the...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2021-01-01
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| Series: | Journal of Indian Association of Pediatric Surgeons |
| Subjects: | |
| Online Access: | http://www.jiaps.com/article.asp?issn=0971-9261;year=2021;volume=26;issue=5;spage=342;epage=344;aulast=Ali |
| _version_ | 1818722445186564096 |
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| author | Mokarram Ali Muneer Abas Malik Nitin James Peters Manasa Reddy Ram Samujh |
| author_facet | Mokarram Ali Muneer Abas Malik Nitin James Peters Manasa Reddy Ram Samujh |
| author_sort | Mokarram Ali |
| collection | DOAJ |
| description | Pleuropulmonary blastomas (PPBs) are very rare, highly aggressive, dysembryonic neoplasms of thoracopulmonary mesenchyme. These have been reported in the pediatric population and account for only 0.5%–1% of all primary malignant lung cancers. They normally arise from lung tissue, however rarely the parietal pleura may be the tissue of origin (extra pulmonary PPB) which are extremely rare. Common age of presentation is three to 4 years. The prognosis is poor with distant metastasis to central nervous system and bone with survival rate of approximately 42.9% at 5 years. They are managed by aggressive multimodal therapies including surgery and chemotherapy. We report a case of a 3-year-old male child with Type 2 PPB of the left hemithorax, managed by surgical excision of the mass and adjuvant chemotherapy. |
| first_indexed | 2024-12-17T20:54:44Z |
| format | Article |
| id | doaj.art-382611e3a5e94e2793a6d8f9f00534ac |
| institution | Directory Open Access Journal |
| issn | 0971-9261 1998-3891 |
| language | English |
| last_indexed | 2024-12-17T20:54:44Z |
| publishDate | 2021-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Journal of Indian Association of Pediatric Surgeons |
| spelling | doaj.art-382611e3a5e94e2793a6d8f9f00534ac2022-12-21T21:32:54ZengWolters Kluwer Medknow PublicationsJournal of Indian Association of Pediatric Surgeons0971-92611998-38912021-01-0126534234410.4103/jiaps.JIAPS_159_20Extrapulmonary pleuropulmonary blastoma in a 3-year-old child: A case report and review of literatureMokarram AliMuneer Abas MalikNitin James PetersManasa ReddyRam SamujhPleuropulmonary blastomas (PPBs) are very rare, highly aggressive, dysembryonic neoplasms of thoracopulmonary mesenchyme. These have been reported in the pediatric population and account for only 0.5%–1% of all primary malignant lung cancers. They normally arise from lung tissue, however rarely the parietal pleura may be the tissue of origin (extra pulmonary PPB) which are extremely rare. Common age of presentation is three to 4 years. The prognosis is poor with distant metastasis to central nervous system and bone with survival rate of approximately 42.9% at 5 years. They are managed by aggressive multimodal therapies including surgery and chemotherapy. We report a case of a 3-year-old male child with Type 2 PPB of the left hemithorax, managed by surgical excision of the mass and adjuvant chemotherapy.http://www.jiaps.com/article.asp?issn=0971-9261;year=2021;volume=26;issue=5;spage=342;epage=344;aulast=Aliextra pulmonary pleuropulmonary blastomamultimodal therapypoor prognosis |
| spellingShingle | Mokarram Ali Muneer Abas Malik Nitin James Peters Manasa Reddy Ram Samujh Extrapulmonary pleuropulmonary blastoma in a 3-year-old child: A case report and review of literature Journal of Indian Association of Pediatric Surgeons extra pulmonary pleuropulmonary blastoma multimodal therapy poor prognosis |
| title | Extrapulmonary pleuropulmonary blastoma in a 3-year-old child: A case report and review of literature |
| title_full | Extrapulmonary pleuropulmonary blastoma in a 3-year-old child: A case report and review of literature |
| title_fullStr | Extrapulmonary pleuropulmonary blastoma in a 3-year-old child: A case report and review of literature |
| title_full_unstemmed | Extrapulmonary pleuropulmonary blastoma in a 3-year-old child: A case report and review of literature |
| title_short | Extrapulmonary pleuropulmonary blastoma in a 3-year-old child: A case report and review of literature |
| title_sort | extrapulmonary pleuropulmonary blastoma in a 3 year old child a case report and review of literature |
| topic | extra pulmonary pleuropulmonary blastoma multimodal therapy poor prognosis |
| url | http://www.jiaps.com/article.asp?issn=0971-9261;year=2021;volume=26;issue=5;spage=342;epage=344;aulast=Ali |
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