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. The gene discussed is MSH2; the disease is childhood malignant neoplasm.