Article
The Histological Spectrum of DICER1-Associated Neoplasms.
Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society - 1 Jan 2025
Capozzi Alessia, Jansen Floor A, Smetsers Stephanie E, Bakhuizen Jette J, Hiemcke-Jiwa Laura S, Kranendonk Mariëtte E G, Flucke Uta, Alaggio Rita, de Krijger Ronald R
Abstract excerpt
DICER1 syndrome is a heterogeneous cancer predisposition syndrome, characterized by a large variety of benign and malignant tumor types, and caused by germline heterozygous pathogenic variants in the DICER1 gene, which is essential in miRNA processing and RNA interference. The clinical manifestations are diverse, with pleuropulmonary blastoma, Sertoli-Leydig cell tumor, cystic nephroma, uterine cervical embryonal...
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