To validate this assumption, we focused on the most frequent variant identified in our study, and one of the two variants here described, and ever reported, to be recurrently found in cancer patients – c.1171_1173del (BubR1∆391) – representing 38.9% of all BUB1B variant carriers and 55.5% of HPC index patients who carry a BUB1B variant. Here, BUB1B is linked to cancer.