" /> Pleuropulmonary Blastoma - CISMeF





Preferred Label : Pleuropulmonary Blastoma;

NCIt synonyms : Pulmonary Blastoma of Childhood; PPB;

NCIt definition : A malignant neoplasm affecting the lungs and/or the pleura. Pleuropulmonary blastoma is seen in children. Microscopically, the tumor may show features of chondrosarcoma, leiomyosarcoma, rhabdomyosarcoma, liposarcoma, or undifferentiated sarcoma. In approximately 25% of patients with pleuropulmonary blastoma, there are other lesions or neoplasms that may affect patients or their families, including lung or kidney cysts, and ovarian or testicular neoplasms. Heterozygous germline mutations in DICER1 gene have been identified in families harboring pleuropulmonary blastomas.;

Alternative definition : NICHD: A malignant neoplasm affecting the lungs and/or the pleura. Pleuropulmonary blastoma is seen in children. Microscopically, the tumor may show features of chondrosarcoma, leiomyosarcoma, rhabdomyosarcoma, liposarcoma, or undifferentiated sarcoma. In approximately 25% of patients there is a predisposition for other tumors. Heterozygous germline mutations in DICER1 gene have been identified in families harboring pleuropulmonary blastomas.; NCI-GLOSS: A rare and very aggressive (fast-growing) cancer that forms in tissues of the lung and pleura (a thin layer of tissue that covers the lungs and lines the interior wall of the chest cavity). Pleuropulmonary blastoma is most common in children.;

Neoplastic status : Malignant;

ICD-O code : 8973/3;

Codes from synonyms : CDR0000446557;

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30/04/2025


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