pleuropulmonary blastoma
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.
MONDO:0005933
pulmonary blastoma
is_a
MONDO:0006517
childhood malignant neoplasm
is_a
MONDO:0100216
DICER1-related tumor predisposition
is_a
PPB
related
PPB familial tumour and dysplasia syndrome
related
childhood pulmonary blastoma
exact
paediatric pulmonary blastoma
exact
pediatric pulmonary blastoma
exact
pleuropulmonary blastoma
exact
pleuropulmonary blastoma (morphologic abnormality)
exact
pulmonary blastoma of childhood
exact
SPCC188.13c