Pleuropulmonary blastoma

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A rare respiratory tumor characterized by an aggressive malignant dysontogenetic neoplasm of intrathoracic (pulmonary pleural or combined) mesenchyme occurring in young children. Three subtypes can be distinguished type 1 being purely cystic type 2 cystic and solid and type 3 purely solid. Type 1 lesions may progress to the more malignant types 2 and 3 which are associated with central nervous system and bone metastasis. The tumor is often part of pleuropulmonary blastoma family tumor and dysplasia syndrome. It can also be associated with multilocular cystic nephroma or other neoplasms. Patients usually present with dyspnea or other respiratory problems and sometimes pneumothorax.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on Data version June 2024

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My Faulty Gene is a nonprofit organization which provides information and assistance to any individual whose family medical history suggests genetic testing might be helpful in identifying an increased risk of disease due to a genetic mutation. We believe that everyone in need of genetic testing should have access to it.

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My Little Sunshine is a non-profit foundation dedicated to educating people about the importance of fertility preservation and making fertility resources accessible to all.


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