We found that seven genes, including connective tissue growth factor (CTGF), Syndecan Binding Protein (SDCBP), Cysteine Rich Protein 61 (CYR61/CCN1), Type III Collagen A 1 Chain (COL3A1), Type I Collagen A 1 Chain (COL1A1), Type V Collagen A 2 Chain (COL5a2), and Serpin Family E Member 1 (SERPINE1), were significantly up-regulated in these tumor samples, which proved that VHL mutation activated Expression of these genes in pheochromocytoma. Here, SERPINE1 is linked to hereditary pheochromocytoma-paraganglioma.