Article
DICER1 and Associated Conditions: Identification of At-risk Individuals and Recommended Surveillance Strategies.
Clinical cancer research : an official journal of the American Association for Cancer Research - 15 May 2018
Schultz Kris Ann P, Williams Gretchen M, Kamihara Junne, Stewart Douglas R, Harris Anne K, Bauer Andrew J, Turner Joyce, Shah Rachana, Schneider Katherine, Schneider Kami Wolfe, Carr Ann Garrity, Harney Laura A, Baldinger Shari, Frazier A Lindsay, Orbach Daniel, Schneider Dominik T, Malkin David, Dehner Louis P, Messinger Yoav H, Hill D Ashley
Abstract excerpt
Pathogenic germline DICER1 variants cause a hereditary cancer predisposition syndrome with a variety of manifestations. In addition to conferring increased cancer risks for pleuropulmonary blastoma (PPB) and ovarian sex cord-stromal tumors, particularly Sertoli-Leydig cell tumor, individuals with pathogenic germline DICER1 variants may also develop lung cysts, cystic nephroma, renal sarcoma and Wilms tumor,...
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