While hallmark somatic mutations have been reported to underlie PA tumorigenesis, including NF1 [3], KRAS [4], PTEN [5], and BRAF [6], heritable genetic contributions impacting risk of PA remain largely unidentified, other than in the context of Neurofibromatosis Type I, where it was shown Neurofibromatosis Type I patients have a higher chance of contracting optic pathway PA, most likely due to NF1 mutations [7]. This evidence concerns the gene PTEN and neurofibromatosis type 1.