Associated Genetic Biomarkers
NCI 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. 
Pleuropulmonary blastomas most frequently harbor alterations in DICER1, TP53, RECQL4, OGG1, and MUS81 .
DICER1fs, DICER1 Mutation, TP53 c.217-c.1178 Missense, TP53 R273C, and TP53 Mutation are the most common alterations in pleuropulmonary blastoma .
There is 1 clinical trial for pleuropulmonary blastoma, of which 1 is open and 0 are completed or closed. Of the trial that contains pleuropulmonary blastoma as an inclusion criterion, 1 is phase 2 (1 open).
NCAM1 is the most frequent gene inclusion criterion for pleuropulmonary blastoma clinical trials .
Lorvotuzumab mertansine is the most common intervention in pleuropulmonary blastoma clinical trials.
2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.