The DICER1 familial tumour susceptibility syndrome confers an increased risk most commonly for pleuropulmonary blastoma (PPB) but also ovarian sex cord-stromal tumours; Sertoli-Leydig cell tumor [SLCT], juvenile granulosa cell tumour [JGCT] and gynandroblastomas. Here, DICER1 is linked to Sertoli-Leydig cell tumor.