These rearrangements lead to a more severe “contiguous gene syndrome” with higher incidence of neurofibromas and MPNSTs, likely attributable to the involvement of tumor suppressor genes in co-deleted regions (e.g., SUZ12—OMIM *606245, RNF135—OMIM *611358, and ADAP2—OMIM *608635) [37,38,39,40]. This evidence concerns the gene RNF135 and plexiform neurofibroma.