The upper and lower age limits for surveillance are based on the typical age of onset of DICER1-associated tumors, covering approximately 90–95% of reported cases [1, 3, 5, 12, 17, 23, 24], in accordance with practice regarding other cancer predisposition syndromes, e.g. BRCA1/BRCA2-associated familial breast and ovarian cancer [25]. Here, BRCA2 is linked to ovarian carcinoma.