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Myoepithelial carcinoma / Printable summary

A summary to hand to a new doctor

Most doctors will not have seen this disease. One page on the disease, one page on you.

Updated 11 November 2026. Not medical advice.

On this page
  1. The disease, in one page
  2. This patient
  3. References
Back to the first-steps guide

1The disease, in one page

For a clinician who has not seen it before. Figures are from the sources numbered below.

Definition
Malignant tumor showing exclusively myoepithelial differentiation1. Older papers call it malignant myoepithelioma2. ICD-O-3 morphology 89823. Listed apart from epithelial-myoepithelial carcinoma in standard vocabularies4.
Incidence
About 0.013 per 100,000 person-years (SEER 1973 to 2012, 368 patients)5.
Sites
Major salivary glands most often, then minor salivary glands, skin and soft tissue, breast6.
Pathology
Co-expression of epithelial markers (keratins, EMA) with S-100; calponin in most, GFAP in about half and p63 in under a quarter of soft-tissue tumors; a subset lose INI178.
Genetics
EWSR1 rearranged in 45% of non-salivary tumors (partners PBX1, PBX3, POU5F1, ZNF444, ATF1, KLF15)91011. PLAG1 fusions in most fusion-positive salivary cases (FGFR1, TGFBR3)12.
Behavior
Soft tissue: local recurrence 42%, metastasis 32% of malignant tumors8. Salivary: distant metastasis 27%, mostly lung13.
Survival
All sites, National Cancer Database (n=336): 79.4% at 2 years, 66.5% at 5, 52.0% at 103. Pooled literature (n=691): median overall survival 167 months6.
Treatment evidence
Complete resection with clear margins associated with better survival; adjuvant radiotherapy reduced locoregional recurrence6. Limited chemotherapy data14. No established systemic standard15.
Prognostic factors
Size over 5 cm, more than 10 mitoses per 10 HPF6; necrosis, vascular invasion, origin in pleomorphic adenoma (salivary)13; fusion type and age under 25 (fusion-positive)11.

2This patient

Fill in before the appointment. Bring the reports themselves as well.

Name and date of birth
Date of diagnosis
Site of the first tumor
Diagnosis line from the pathology report
Second pathology opinion (where, when)
Stains reported positive
Margin status after each surgery
Fusion and INI1 testing (test, result)
Size, grade, mitotic rate
Scans done (type, date, finding)
Surgeries (date, site, surgeon)
Radiotherapy or drug treatment
Recurrences or new tumors (date, site)
Current surveillance schedule
Treating doctors and centers
Questions for this visit

3References

  1. Savera AT, Sloman A, Huvos AG et al. Myoepithelial carcinoma of the salivary glands: a clinicopathologic study of 25 patients. The American journal of surgical pathology, 2000. PubMed ↩
  2. Crissman JD, Wirman JA, Harris A. Malignant myoepithelioma of the parotid gland. Cancer, 1977. PubMed ↩
  3. Nikhita Tandon, Eman Alameldeen, Grace S. Saglimbeni et al. Myoepithelial carcinoma: A National Cancer Database study of demographic and socioeconomic factors. Journal of Clinical Oncology, 2026. DOI ↩
  4. NCI Thesaurus, OncoTree, MONDO, the Disease Ontology, MeSH and Wikidata, retrieved 11 November 2026. source ↩
  5. Xiaofei Zhang, Jiyi Hu, Jiade Jay Lu et al. Incidence patterns for myoepithelial carcinoma: a Surveillance, Epidemiology, and End Results (SEER) study. Translational Cancer Research, 2017. DOI ↩
  6. Giridhar P, Gupta P, Mallick S et al. Impact of adjuvant therapy on survival in patients with myoepithelial carcinoma: A systematic review and individual patient data analysis of 691 patients. Radiotherapy and oncology : journal of the European Society for Therapeutic Radiology and Oncology, 2019. PubMed ↩
  7. Jo VY, Fletcher CD. Myoepithelial neoplasms of soft tissue: an updated review of the clinicopathologic, immunophenotypic, and genetic features. Head and neck pathology, 2015. PubMed ↩
  8. Hornick JL, Fletcher CD. Myoepithelial tumors of soft tissue: a clinicopathologic and immunohistochemical study of 101 cases with evaluation of prognostic parameters. The American journal of surgical pathology, 2003. PubMed ↩
  9. Antonescu CR, Zhang L, Chang NE et al. EWSR1-POU5F1 fusion in soft tissue myoepithelial tumors. A molecular analysis of sixty-six cases, including soft tissue, bone, and visceral lesions, showing common involvement of the EWSR1 gene. Genes, chromosomes & cancer, 2010. PubMed ↩
  10. Flucke U, Mentzel T, Verdijk MA et al. EWSR1-ATF1 chimeric transcript in a myoepithelial tumor of soft tissue: a case report. Human pathology, 2012. PubMed ↩
  11. Michal M, Malik F, Mansour B et al. FET-Rearranged Myoepithelial Tumors Are Clinically Heterogeneous and Epigenetically Distinct from PLAG1-Rearranged Adnexal and Salivary Gland Myoepithelial Tumors. Clinical cancer research : an official journal of the American Association for Cancer Research, 2026. PubMed ↩
  12. Dalin MG, Katabi N, Persson M et al. Multi-dimensional genomic analysis of myoepithelial carcinoma identifies prevalent oncogenic gene fusions. Nature communications, 2017. PubMed ↩
  13. Kong M, Drill EN, Morris L et al. Prognostic factors in myoepithelial carcinoma of salivary glands: a clinicopathologic study of 48 cases. The American journal of surgical pathology, 2015. PubMed ↩
  14. Chamberlain F, Cojocaru E, Scaranti M et al. Adult soft tissue myoepithelial carcinoma: treatment outcomes and efficacy of chemotherapy. Medical oncology (Northwood, London, England), 2019. PubMed ↩
  15. Jamie D. Barber, Naomi Natale, Michael Casaus. Abstract 6355: Building the first comprehensive research ecosystem and preclinical model platform for myoepithelial carcinoma. Cancer Research, 2026. DOI ↩