Investigating oncogenic mechanisms in DICER1 syndrome-associated ovarian Sertoli-Leydig cell tumors

DICER1 syndrome is a rare, inherited disorder; individuals with a mutation (change) in the DICER1 gene are at increased risk of a variety of cancerous and non-cancerous (benign) tumors. It affects mostly children and young adults. These cancers can be lethal at advanced stages of disease with no biologically-informed treatment strategies available. Despite the discovery of the gene, DICER1, being attributed to cancer development, the translation of this genomic discovery to the bedside to improve cancer care has been hindered by the lack of relevant models to study the disease. My post-doctoral research project will focus on one such aggressive rare cancer, called ovarian Sertoli-Leydig cell tumor. I will use the unique tools/resources such as a large cohort of patient samples and the first-ever mouse model of DICER1 syndrome-associated cancer to understand the biology of this rare ovarian cancer and identify potential druggable candidates for future therapeutic development.