Temporal order of RNase IIIb and loss-of-function mutations during development determines phenotype in pleuropulmonary blastoma / DICER1 syndrome: a unique variant of the two-hit tumor suppression model [version 2; referees: 2 approved]

Pleuropulmonary blastoma (PPB) is the most frequent pediatric lung tumor and often the first indication of a pleiotropic cancer predisposition, DICER1 syndrome, comprising a range of other individually rare, benign and malignant tumors of childhood and early adulthood. The genetics of DICER1-associa...

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Bibliographic Details
Main Authors: Mark Brenneman, Amanda Field, Jiandong Yang, Gretchen Williams, Leslie Doros, Christopher Rossi, Kris Ann Schultz, Avi Rosenberg, Jennifer Ivanovich, Joyce Turner, Heather Gordish-Dressman, Douglas Stewart, Weiying Yu, Anne Harris, Peter Schoettler, Paul Goodfellow, Louis Dehner, Yoav Messinger, D. Ashley Hill
Format: Article
Language:English
Published: F1000 Research Ltd 2018-01-01
Series:F1000Research
Subjects:
Online Access:https://f1000research.com/articles/4-214/v2