Thus, it is possible that childhood medulloblastoma is specifically associated with the germline POLE p.(E277G) variant in the digenic CPS case of Michaeli et al. [55], who also presented with CMMRD-like skin CALMs (as did all of his family members who carried this POLE variant) and had an MSS tumor suggesting a strong mutational effect of the POLE variant E277G [58]. Here, POLE is linked to neoplasm.