The following criteria were used for the histological diagnosis of MPNST – a) gross fusiform tumors in relation to nerves, b) microscopic features of spindle cell with fascicular pattern and varying degrees of mitosis, necrosis and tumor calcification, c) presence of associated benign neurofibroma or schwannanian cells, and d) positive immunohistochemical staining for S-100 protein, neuron specific enolase (NSE) and others like actin, cytokeratin (CK), smooth muscle actins (SMA), desmin, and vimentin to differentiate from other spindle cell sarcomas. This evidence concerns the gene DES and malignant peripheral nerve sheath tumor.