Pleuropulmonary blastoma

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.

Also known as childhood pulmonary blastoma, paediatric pulmonary blastoma, pediatric pulmonary blastoma, pleuropulmonary blastoma, pleuropulmonary blastoma (morphologic abnormality), pulmonary blastoma of childhood — per MONDO

Also identified as

• DOID 4769 per MONDO
• ICD9 162.9 per MONDO
• MESH C537516 per MONDO
• NCIT C5669 per MONDO
• OMIM 601200 per MONDO
• Orphanet 64742 per MONDO
• SCTID 707670009 per MONDO
• UMLS C1266144 per MONDO

Affected Organs