Genomic and Expression Profiling of Benign and Malignant Nerve Sheath Profiling of Benign and Malignant Nerve Sheath

摘要

The goal of the study is to identify genes that will serve as molecular markers for progression of neurofibroma to MPNST, and to identify potential therapeutic targets. miRNA expression profiling was performed on 6 cases of MPNSTs, and 7 cases of synovial sarcomas. By using unsupervised hierarchical clustering most tumors were grouped together according to tumor type. Subsequent analyses using Significance Analysis of Microarrays (SAM) identified miRNAs that differentiate between MPNSTs and synovial sarcoma (SS). To develop a cell line model for MPNSTs, global gene expression profiles for cell lines established from 3 primary MPNST and SS tumor tissues was carried out and their expression profiles were compared with other sarcomas. A large tissue microarray (TMA) containing about 200 nerve sheath tumors was used to test for EGFR expression by IHC. Neoplasms in which the majority of samples showed high expression by IHC included MPNST (83% of NF1- associated and 77% of sporadic), 73% of plexiform neurofibroma, 100% diffuse neurofibroma and 93% of SS.