Deletion of Yy1 in mouse lung epithelium unveils molecular mechanisms governing pleuropulmonary blastoma pathogenesis
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چکیده
منابع مشابه
Pleuropulmonary Blastoma in Children: A Case Report
Pleuropulmonary Blastoma [PPB] is a very rare, highly aggressive and malignant tumor that originates from either the lungs or pleura. It occurs mainly in children aged less than five or six years. It has poor prognosis with three different subtypes: cystic [type I], combined cystic and solid [type II] and solid [type III]. PPB is treated with aggressive multimodal therapies including surgery an...
متن کاملBiallelic DICER1 mutations in sporadic pleuropulmonary blastoma.
Pleuropulmonary blastoma (PPB) is a rare pediatric malignancy whose pathogens are poorly understood. Recent reports suggest that germline mutations in the microRNA-processing enzyme DICER1 may contribute to PPB development. To investigate the genetic basis of this cancer, we performed whole-exome sequencing or targeted deep sequencing of multiple cases of PPB. We found biallelic DICER1 mutation...
متن کاملPleuropulmonary Blastoma: Report of 2 Cases
Pleuropulmonary blastoma (PPB) is a rare, malignant intrathoracic tumor that occurs mostly in children before 5 years old. It consists about 15% of all primary pediatric pulmonary tumors and shows various mesenchymal components. Its presentations are nonspecific and common symptoms include fever, cough, chest pain, respiratory distress, and pulmonary infection. PPB should be considered in the ...
متن کاملType II- Pleuropulmonary Blastoma: A case report
Pleuropulmonary blastoma is a rare and aggressive neoplasm typically presents in young children, younger than 5 years, as a pulmonary and/or pleural-based tumor. We reported a case of type-II pleuropulmonary blastoma (PPB) in an 8 month old infant who presented to Al-Khansa'a Maternity Hospital in Western Mosul with a history of repeated attacks of shortness of breath and signs of pulmonary inf...
متن کاملFibroblast Growth Factor 9 Regulation by MicroRNAs Controls Lung Development and Links DICER1 Loss to the Pathogenesis of Pleuropulmonary Blastoma
Pleuropulmonary Blastoma (PPB) is the primary neoplastic manifestation of a pediatric cancer predisposition syndrome that is associated with several diseases including cystic nephroma, Wilms tumor, neuroblastoma, rhabdomyosarcoma, medulloblastoma, and ovarian Sertoli-Leydig cell tumor. The primary pathology of PPB, epithelial cysts with stromal hyperplasia and risk for progression to a complex ...
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ژورنال
عنوان ژورنال: Disease Models & Mechanisms
سال: 2020
ISSN: 1754-8411,1754-8403
DOI: 10.1242/dmm.045989