For example, there are reports of durable response to nivolumab in two siblings with recurrent glioblastoma carrying a biallelic PMS2 germline variant [40], and of clinical and immunologic response to pembrolizumab in a patient with hypermutated glioblastomas and a heterozygous POLE germline variant affecting the exonuclease domain [31]. Here, POLE is linked to glioblastoma.