A rare case of Pleuropulmonary blastoma type III with Immunohistochemical Study
Here we intend to document a rare case of PPB type III in a 2-year male presenting with an extensive tumor occupying the right hemithorax with immunohistochemical (IHC) study. Pleuropulmonary blastoma (PPB) is a rare variably aggressive, dysodontogenetic, childhood primary intrathoracic malignancy w...
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2023-07-01
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| Series: | Indian Journal of Pathology and Microbiology |
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| Online Access: | https://journals.lww.com/10.4103/ijpm.ijpm_781_21 |
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| author | Siddhi Gaurish Sinai Khandeparkar Maithili Mandar Kulkarni Bageshri P. Gogate Chinmayee Sanjeev Dhavan |
| author_facet | Siddhi Gaurish Sinai Khandeparkar Maithili Mandar Kulkarni Bageshri P. Gogate Chinmayee Sanjeev Dhavan |
| author_sort | Siddhi Gaurish Sinai Khandeparkar |
| collection | DOAJ |
| description | Here we intend to document a rare case of PPB type III in a 2-year male presenting with an extensive tumor occupying the right hemithorax with immunohistochemical (IHC) study.
Pleuropulmonary blastoma (PPB) is a rare variably aggressive, dysodontogenetic, childhood primary intrathoracic malignancy which in up to 25% of cases can be extrapulmonary with attachment to the parietal pleura. It is found in pediatric population under 5 years of age. It was initially proposed as a distinct entity by Manivel et al. in 1988. PPB is a proliferation of primitive mesenchymal cells that initially form air-filled cysts lined by benign-appearing epithelium (type I, cystic). Later on, the mesenchymal cells outgrow the cysts with formation of focal solid areas (type II, solid and cystic) and finally, mainly solid mass (type III, solid PPB). |
| format | Article |
| id | doaj-art-75fa9344ba1b46b094704387ff16cc27 |
| institution | Kabale University |
| issn | 0377-4929 |
| language | English |
| publishDate | 2023-07-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Indian Journal of Pathology and Microbiology |
| spelling | doaj-art-75fa9344ba1b46b094704387ff16cc272025-02-07T13:35:31ZengWolters Kluwer Medknow PublicationsIndian Journal of Pathology and Microbiology0377-49292023-07-0166363263510.4103/ijpm.ijpm_781_21A rare case of Pleuropulmonary blastoma type III with Immunohistochemical StudySiddhi Gaurish Sinai KhandeparkarMaithili Mandar KulkarniBageshri P. GogateChinmayee Sanjeev DhavanHere we intend to document a rare case of PPB type III in a 2-year male presenting with an extensive tumor occupying the right hemithorax with immunohistochemical (IHC) study. Pleuropulmonary blastoma (PPB) is a rare variably aggressive, dysodontogenetic, childhood primary intrathoracic malignancy which in up to 25% of cases can be extrapulmonary with attachment to the parietal pleura. It is found in pediatric population under 5 years of age. It was initially proposed as a distinct entity by Manivel et al. in 1988. PPB is a proliferation of primitive mesenchymal cells that initially form air-filled cysts lined by benign-appearing epithelium (type I, cystic). Later on, the mesenchymal cells outgrow the cysts with formation of focal solid areas (type II, solid and cystic) and finally, mainly solid mass (type III, solid PPB).https://journals.lww.com/10.4103/ijpm.ijpm_781_21lung tumorspediatric malignancyppb |
| spellingShingle | Siddhi Gaurish Sinai Khandeparkar Maithili Mandar Kulkarni Bageshri P. Gogate Chinmayee Sanjeev Dhavan A rare case of Pleuropulmonary blastoma type III with Immunohistochemical Study Indian Journal of Pathology and Microbiology lung tumors pediatric malignancy ppb |
| title | A rare case of Pleuropulmonary blastoma type III with Immunohistochemical Study |
| title_full | A rare case of Pleuropulmonary blastoma type III with Immunohistochemical Study |
| title_fullStr | A rare case of Pleuropulmonary blastoma type III with Immunohistochemical Study |
| title_full_unstemmed | A rare case of Pleuropulmonary blastoma type III with Immunohistochemical Study |
| title_short | A rare case of Pleuropulmonary blastoma type III with Immunohistochemical Study |
| title_sort | rare case of pleuropulmonary blastoma type iii with immunohistochemical study |
| topic | lung tumors pediatric malignancy ppb |
| url | https://journals.lww.com/10.4103/ijpm.ijpm_781_21 |
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