Pleuropulmonary blastoma
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 http://www.orphadata.org. Data version April 2024
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Pleuropulmonary blastoma?
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