The occurrences of endometrial and skin cancers in the proposita’s deceased mother (II.9, obligate carrier) and skin sebaceous cancers and/or multiple colorectal and upper GI adenomas in confirmed carriers of the MSH6 variant alone, all above 50 years of age, are consistent with the MSH6-LS phenotype. This evidence concerns the gene MSH6 and sebaceous adenocarcinoma.