NF1 and malignant peripheral nerve sheath tumor: Although constitutive depletion of PRC2 core proteins is responsible for embryonic lethality in mice, complete genetic loss- or gain-of-function variants in somatic tissues constitute a driver event for several tumour types through a major alteration of transcription regulation and the alteration of RAS, WNT, and NOTCH signalling [32], and can be at the origin of lymphoid and myeloid malignancies [33], as well as MPNST [34], this later being significantly more prevalent in NF1-deleted patients [15].