Malignant peripheral nerve sheath tumour arising within neurofibroma. An immunohistochemical analysis in the comparison between benign and malignant components

Aims - To compare the expression of immunohistochemical variables between benign and malignant components of malignant peripheral nerve sheath tumour (MPNST) arising within neurofibroma. Methods - Eight cases of MPNST arising within a neurofibroma,associated with neurofibromatosis type 1 (NF1), were studied. The areas of MPNST and neurofibroma were compared immunohistochemically with regard to the expression of proliferative activity (MIB-1), growth factors, p53, bcl-2, neural cell adhesion molecule (N-CAM), and CD34. Results - The expression of transforming growth factor β1 (TGF-β1), TGF-β receptor type II, hepatocyte growth factor a (HGF-α), c-met, p53, and N-CAM was higher in the areas of MPNST than in the neurofibromatous areas in four, five, five, eight, five, and three of the eight cases, respectively. CD34 expression was lower in the areas of MPNST than in the neurofibroma areas in three of the eight cases. Conclusions - On the basis of these findings, TGF-β1, HGF-α, and p53 might be involved in the malignant transformation of neurofibroma to MPNST.