The diagnosis of MPNST has always been challenging because of its histological variation and the absence of a surrogate marker for the biallelic loss of function of NF1. Loss of H3K27me3 expression, largely specific to high‐grade MPNST, recently provided a valuable marker for this disease [28]; however, evidence has accumulated that a significant proportion of MPNSTs do not exhibit this molecular alteration [8, 16]. Here, NF1 is linked to malignant peripheral nerve sheath tumor.