Carcinoma ex-pleomorphic adenoma (CXPA) is a carcinoma originating from a primary or recurrent benign pleomorphic adenoma [1]. The most common sites of origin are major salivary gland where these represent approximately 3–5% of all salivary gland neoplasms [1]. Due to the rarity of the disease, no level one evidence based treatment algorithm is present. The main evidence is available from retrospective institutional experiences. Surgery followed by post-operative radiotherapy is the mainstay of the treatment [1,2].
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