# Carcinoma ex pleomorphic adenoma

Source: https://onco.cc/cancers/carcinoma-ex-pleomorphic-adenoma/  
OnCo record `carcinoma-ex-pleomorphic-adenoma` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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).

How 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.

How common: about 0.6 per million a year in the United States (American Journal of Otolaryngology 2019).

Treatment: 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.

## Fields

- Kind: Cancer
- Last checked: 2026-09-24
- Also known as: Carcinoma ex pleomorphic adenoma (CXPA); Malignant mixed tumour of the salivary gland; Carcinoma arising in a pleomorphic adenoma
- Tags: subtype-page; wave4; rare
- 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)

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/carcinoma-ex-pleomorphic-adenoma/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/carcinoma-ex-pleomorphic-adenoma/#overview
- What it is (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/carcinoma-ex-pleomorphic-adenoma/#what-it-is [4 subtypes]
- Finding it (on the hub): How it shows itself, how it is confirmed, what screening exists, and the biomarkers clinicians test for. https://onco.cc/cancers/carcinoma-ex-pleomorphic-adenoma/#finding-it [4 biomarkers]
- Treating it (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/carcinoma-ex-pleomorphic-adenoma/#treating-it [1 setting]
- Evidence (on the hub): Trials recruiting now, the landmark trials, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/carcinoma-ex-pleomorphic-adenoma/#evidence
- The science (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/carcinoma-ex-pleomorphic-adenoma/#science
- Where you are (own page): Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record. https://onco.cc/cancers/carcinoma-ex-pleomorphic-adenoma/where-you-are/
- Living with it (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/carcinoma-ex-pleomorphic-adenoma/#living-with-it [7 questions]
- What is coming (own page): Everything in development, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/carcinoma-ex-pleomorphic-adenoma/coming/
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/carcinoma-ex-pleomorphic-adenoma/data/

## Standard of care

- All stages: Complete excision; postoperative radiotherapy and neck dissection for widely invasive, high-grade or node-positive disease; systemic therapy by the carcinoma component. ([Salivary gland cancers](https://onco.cc/cancers/salivary-gland/), [Salivary duct carcinoma](https://onco.cc/cancers/salivary-duct-carcinoma/))

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Carcinoma_ex_pleomorphic_adenoma
- NCI PDQ: salivary gland cancer treatment: https://www.cancer.gov/types/head-and-neck/patient/adult/salivary-gland-treatment-pdq
- Head and Neck Pathology 2012: carcinoma ex pleomorphic adenoma, comprehensive review: https://doi.org/10.1007/s12105-011-0281-z
- American Journal of Otolaryngology 2019: carcinoma ex pleomorphic adenoma in SEER 1973 to 2015, 619 patients: https://doi.org/10.1016/j.amjoto.2019.102279

## Connected records

- cancers: [Acinic cell carcinoma of the salivary glands](https://onco.cc/cancers/acinic-cell-carcinoma-salivary/), [Adenoid cystic carcinoma](https://onco.cc/cancers/adenoid-cystic-carcinoma/), [Salivary duct carcinoma](https://onco.cc/cancers/salivary-duct-carcinoma/), [Salivary gland cancers](https://onco.cc/cancers/salivary-gland/)

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JSON: https://onco.cc/api/v1/entities/carcinoma-ex-pleomorphic-adenoma.json