Article
The co-occurrence of an ovarian Sertoli-Leydig cell tumor with a thyroid carcinoma is highly suggestive of a DICER1 syndrome.
Virchows Archiv : an international journal of pathology - 1 May 2016
Durieux Emeline, Descotes Françoise, Mauduit Claire, Decaussin Myriam, Guyetant Serge, Devouassoux-Shisheboran Mojgan
Abstract excerpt
The DICER1 gene encodes an endoribonuclease involved in the production of mature microRNAs which regulates gene expression through several mechanisms. Carriers of germline DICER1 mutations are predisposed to a rare cancer syndrome, the DICER1 syndrome. Pleuropulmonary blastoma is the most frequent lesion seen in this syndrome. Thyroid abnormalities are also a common finding, essentially concerning multinodular...
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