Abstract / Summary
Abstract Dermatofibrosarcoma protuberans (DFSP) is a rare sarcoma with low metastatic potential, yet pulmonary metastasis, when it occurs, carries a dismal prognosis. Although imatinib is approved for advanced DFSP, its efficacy in metastatic disease remains limited and resistance inevitably develops. To characterize the proteomic landscape of DFSP pulmonary metastasis and identify functional drivers of metastatic progression, we performed proteomic profiling on 18 tissue specimens from six patients with paired normal, primary, and metastatic tissues. Through integrated differential expression analysis, weighted gene co-expression network analysis, and expression trend analysis, we identified LMO7 as a progressively upregulated protein along the normal–primary–metastasis continuum, a finding validated by immunohistochemistry and quantitative PCR. Functional assays in patient-derived primary DFSP cells demonstrated that LMO7 knockdown significantly inhibited cell proliferation, migration, and invasion. Despite limitations including small sample size and the use of primary cells with limited passages, our findings identify LMO7 as a progressively upregulated protein during DFSP metastatic progression that may contribute to malignant behavior, supporting its potential as a prognostic biomarker and therapeutic target for metastatic DFSP.