On this page
1What is known
The reference series is 29 soft-tissue carcinomas in patients from newborn to 17 years old, with a median age of nine. Fourteen were in the limbs, six in the trunk, five in internal organs and four in the head and neck2.
Of 23 children followed, nine had local recurrences, 12 developed metastases and 10 died of the disease, at a median of nine months after diagnosis2.
2Genetics
The childhood disease is defined by gene fusions3. EWSR1::KLF15 tumors occur mostly in children under five, and EWSR1::POU5F1 was first found in a child and then in other children and young adults45. In a pooled analysis of 185 fusion-positive cases, age under 25 predicted worse progression-free survival4.
3Treatment reports
Evidence is at the level of single patients. One report describes a child treated with ifosfamide, cisplatin and etoposide, then ifosfamide, vincristine and etoposide, with radiotherapy to the surgical bed6. Another used RNA expression analysis to choose a targeted drug for a four-year-old7.
cureMEC, the patient organization, reports building preclinical models of the fusion-positive disease3.
4The literature
Papers naming the disease that mention this form in title or abstract, by year. Assigned by keyword8.
View as table, by decade
| Decade | Papers |
|---|---|
| 1990s | 1 |
| 2000s | 20 |
| 2010s | 29 |
| 2020s | 48 |
Browse all of them in the directory. See the published cases.
5Most cited papers
6Newest papers
7References
- 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 ↩
- Gleason BC, Fletcher CD. Myoepithelial carcinoma of soft tissue in children: an aggressive neoplasm analyzed in a series of 29 cases. The American journal of surgical pathology, 2007. PubMed ↩
- Jamie D. Barber, Barbara Van Hare, Naomi Natale et al. Abstract A004: Establishing the First Integrated Research Infrastructure and Preclinical Models for EWSR1 Fusion-Positive Myoepithelial Carcinoma. Cancer Research, 2026. DOI ↩
- 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 ↩
- 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 ↩
- Miccio JA, Oladeru OT, Yang J et al. Myoepithelial Carcinoma: The Role of Radiation Therapy. A Case Report and Analysis of Data From the Surveillance, Epidemiology, and End Results (SEER) Registry. Journal of pediatric hematology/oncology, 2016. PubMed ↩
- Vasquez YA, Sanders L, Beale HC et al. Comparative analysis of RNA expression identifies effective targeted drug in myoepithelial carcinoma. NPJ precision oncology, 2025. PubMed ↩
- Catalog compiled for this page: PubMed, Europe PMC, OpenAlex and Semantic Scholar records merged and de-duplicated, retrieved 11 November 2026. Method described on this page. method ↩