Of clinical relevance is the fact that cancer surveillance and risk-reducing guidelines now exist for 13 of the 15 hereditary cancer syndromes caused by non-BRCA1/2 genes, and would alter management for almost 70% (22/32) of the non-BRCA1/2 mutation carriers.[8, 15] This is particularly so for high penetrance genes like TP53 (n = 6) and MLH/MSH2 (n = 3) which predispose affected individuals to a wide spectrum of malignancies ranging from childhood cancers to colon, adrenal and endocrine cancers in adulthood, and warrant earlier screening strategies and intervention. Here, BRCA1 is linked to malignant endocrine neoplasm.