Abstract

There are few studies investigating the biomarker signatures and their relations with prognosis of pulmonary artery sarcoma patients. In the present study, we performed a cohort study on 16 cases of pulmonary artery sarcoma in an attempt to identify the potential diagnostic markers and molecular therapeutic targets of that vascular sarcoma. Patients who had undergone pulmonary artery sarcoma tumorectomy over 7 ½ years in our hospital were enrolled in this retrospective study. The resected tumors were analyzed by histopathology, the expression and mutation of Murine Double Minute 2 (MDM2), Epidermal Growth Factor Receptor (EGFR), and Platelet-Derived Growth Factor Alpha (PDGFRA) by immunohistochemistry (IHC) and fluorescence in situ hybridization (FISH). The correlations between patients’ outcomes and tumor histological, IHC and FISH features were statistically analyzed. MDM2 immunostaining was seen mainly in intimal sarcoma. At univariate analysis, MDM2 expression had a positive correlation with shorter progression free survival (PFS). MDM2 and EGFR co-expression also had a positive correlation with shorter PFS and tumor metastasis. This study investigated MDM2, EGFR, and PDGFRA as potential biomarkers for diagnosis of pulmonary artery sarcoma and therapeutic targets.

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