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6, 7, 8 However, it is unclear whether H3K27me3 loss is specific for malignant peripheral nerve sheath tumor, as loss of this protein has also been reported recently in some other sarcomas, especially synovial sarcoma and dermatofibrosarcoma protuberans, and in other malignant spindle cell tumors (rare subset of spindle cell melanoma)

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Two other histological features are important for the histological diagnosis: the uniformity of the cell population morphology from side to side of the lesion and the presence of isolated single cells in a pseudo-infiltrative pattern at the base of the nevus, which reaches sometimes into the subcutaneous fat