DICER1 mutations in familial pleuropulmonary blastoma.

  title={DICER1 mutations in familial pleuropulmonary blastoma.},
  author={Dana Ashley Hill and Jennifer L. Ivanovich and John R. Priest and Christina A. Gurnett and Louis P. Dehner and David Michael Desruisseau and Jason A. Jarzembowski and Kathryn Wikenheiser-brokamp and Brian K. Suarez and Alison J. Whelan and Gretchen Williams and Dawn Bracamontes and Yoav H Messinger and Paul J Goodfellow},
  volume={325 5943},
Pleuropulmonary blastoma (PPB) is a rare pediatric lung tumor that is often part of an inherited cancer syndrome. PPBs consist of mesenchymal cells that are susceptible to malignant transformation and cysts lined by epithelial cells. In a subset of patients, overgrowth of the cysts by mesenchymal cells leads to sarcoma formation. Here, we show that 11 multiplex PPB families harbor heterozygous germline mutations in DICER1, a gene encoding an endoribonuclease critical to the generation of small… CONTINUE READING
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