Whereas the pineocytomas showed no pathogenic variants in NGS panel sequencing except for a heterozygous PALB2 variant in a single case, hotspot KBTBD4 inframe insertions were found in all 4 PPTID-B samples and 9 of 11 PPTID-A cases. Here, PALB2 is linked to pineal parenchymal tumor of intermediate differentiation.