Pleomorphic salivary gland adenoma (PSA) is the most common salivary gland neoplasm. Fusions involving PLAG1 locus were first discovered in pleomorphic adenoma in 1997. PLAG1 gene rearrangement is the most common genetic abnormality in pleomorphic adenoma, resulting in overexpression of PLAG1 protein. PLAG1 is a zinc finger transcription factor and a proto-oncogene located on chromosome 8q12.