{"entity":{"id":"carcinoma-ex-pleomorphic-adenoma","kind":"cancer","name":"Carcinoma ex pleomorphic adenoma","aka":["Carcinoma ex pleomorphic adenoma (CXPA)","Malignant mixed tumour of the salivary gland","Carcinoma arising in a pleomorphic adenoma"],"tldr":"Carcinoma ex pleomorphic adenoma is a salivary gland cancer that grows out of a long-standing benign pleomorphic adenoma, the commonest salivary tumour, usually in the parotid. Its outlook depends on how far the cancer has grown beyond the old adenoma's capsule: cancers still inside it are cured by surgery, while widely invasive ones need radiotherapy and do poorly.","summary":"Carcinoma ex pleomorphic adenoma is a carcinoma arising from a primary or recurrent benign pleomorphic adenoma, most often presenting as a firm parotid mass; it is difficult to diagnose preoperatively, pathological assessment is the gold standard, treatment is ablative surgery often followed by radiotherapy, prognosis is poor overall, and molecular studies show a multistep progression from adenoma (Head and Neck Pathology 2012). The WHO classification grades it by the extent of invasion beyond the adenoma capsule (intracapsular, minimally invasive, widely invasive), which drives prognosis. In 619 SEER patients the parotid was the site in 76.9 percent, two- and five-year disease-specific survival were 90.3 and 80.4 percent, facial nerve sacrifice did not predict survival, and size over 4 cm, multiple positive nodes and distant metastasis raised the risk of death two- to four-fold (American Journal of Otolaryngology 2019).\n\nHow it differs from its parent: the carcinoma component can be of any salivary type (salivary duct carcinoma most often), so treatment follows that component once invasion is established, and the history of a slowly growing lump that suddenly enlarges is the clinical clue.\n\nHow common: about 0.6 per million a year in the United States (American Journal of Otolaryngology 2019).\n\nTreatment: complete excision (parotidectomy) for all forms; postoperative radiotherapy and neck dissection for widely invasive, high-grade or node-positive disease; systemic therapy by the carcinoma component, including HER2- and androgen receptor-directed therapy when the component is salivary duct carcinoma, as on that page.","asOf":"2026-09-24","wikipedia":"https://en.wikipedia.org/wiki/Carcinoma_ex_pleomorphic_adenoma","links":[{"label":"NCI PDQ: salivary gland cancer treatment","url":"https://www.cancer.gov/types/head-and-neck/patient/adult/salivary-gland-treatment-pdq"},{"label":"Head and Neck Pathology 2012: carcinoma ex pleomorphic adenoma, comprehensive review","url":"https://doi.org/10.1007/s12105-011-0281-z"},{"label":"American Journal of Otolaryngology 2019: carcinoma ex pleomorphic adenoma in SEER 1973 to 2015, 619 patients","url":"https://doi.org/10.1016/j.amjoto.2019.102279"}],"tags":["subtype-page","wave4","rare"],"related":["salivary-gland","salivary-duct-carcinoma","acinic-cell-carcinoma-salivary","adenoid-cystic-carcinoma"],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"head and neck","burden":"619 patients in the US SEER registry over 1973 to 2015, 76.9 percent in the parotid; the reported incidence rose from 0.24 to 0.63 per million between 2005 and 2015 (American Journal of Otolaryngology 2019).","subtypes":["Intracapsular carcinoma ex pleomorphic adenoma (non-invasive; excellent outlook)","Minimally invasive carcinoma ex pleomorphic adenoma (invasion under 4 to 6 mm)","Widely invasive carcinoma ex pleomorphic adenoma (the poor-prognosis form)","Carcinoma ex pleomorphic adenoma with a salivary duct carcinoma component (HER2, androgen receptor)"],"biomarkers":["Extent of invasion beyond the adenoma capsule","PLAG1 or HMGA2 rearrangement (inherited from the adenoma)","HER2 and androgen receptor when the component is salivary duct carcinoma","Size over 4 cm and node number (prognostic)"],"standardOfCare":[{"setting":"All stages","approach":"Complete excision; postoperative radiotherapy and neck dissection for widely invasive, high-grade or node-positive disease; systemic therapy by the carcinoma component.","refs":["salivary-gland","salivary-duct-carcinoma"]}],"stateOfArt":[],"history":[],"pipeline":[],"openProblems":[],"parent":"salivary-gland"},"route":"/cancers/carcinoma-ex-pleomorphic-adenoma/","neighbours":{"cancer":[{"id":"acinic-cell-carcinoma-salivary","kind":"cancer","name":"Acinic cell carcinoma of the salivary glands","route":"/cancers/acinic-cell-carcinoma-salivary/"},{"id":"adenoid-cystic-carcinoma","kind":"cancer","name":"Adenoid cystic carcinoma","route":"/cancers/adenoid-cystic-carcinoma/"},{"id":"salivary-duct-carcinoma","kind":"cancer","name":"Salivary duct carcinoma","route":"/cancers/salivary-duct-carcinoma/"},{"id":"salivary-gland","kind":"cancer","name":"Salivary gland cancers","route":"/cancers/salivary-gland/"}]}}