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1What is known
Myoepithelial cells surround the sweat glands of the skin, as they do the salivary glands and the breast2. The first series of these tumors in the skin came in 2003 and 2004, with 20 and 14 cases23. The earliest paper in this catalog, from 1957, describes a malignant myoepithelioma of the sweat glands4.
In the 14-case series, most tumors were on the limbs, the median age was 22.5, tumors measured 0.5 to 2.5 cm, and one of the 14 was a carcinoma3. In an 18-case series, patients were 34 to 86 years old and one tumor, on the cheek, was a carcinoma that had grown into the fat beneath the skin5.
The carcinoma has a high rate of local recurrence and metastasis in the skin1. A 2025 report describes a tumor in the axilla that had spread to lymph nodes6.
2Related skin tumors
Skin tumors of this family form a spectrum: mixed tumor, which forms ducts and is also called chondroid syringoma; myoepithelioma, which is purely myoepithelial and benign; and myoepithelial carcinoma5. A related malignant tumor, malignant mixed tumor, was counted together with myoepithelial carcinoma in a 101-tumor soft-tissue series, which defined it by malignant cartilage or bone7.
A pathology report may also use the wider term adnexal carcinoma, meaning a cancer of the skin's glands and follicles, with myoepithelial carcinoma given as the specific type8.
3Genetics in the skin
Half of the duct-forming tumors of skin and soft tissue in one study carried PLAG1 rearrangements, as mixed tumors of the salivary glands do9. EWSR1 is rearranged in a subset of cutaneous myoepithelial tumors5, and fusions of EWSR1 with PBX1 or PBX3 are enriched in skin and bone10. A TRPS1::PLAG1 fusion was reported in a primary cutaneous carcinoma in 20261.
A 2026 methylation study found that PLAG1-rearranged tumors were mostly benign, in older adults and in the skin, and were biologically distinct from tumors with EWSR1 or FUS fusions10. Which group a skin tumor belongs to is therefore worth knowing.
4Treatment reports
There is no series large enough to compare treatments in the skin. Across all sites, complete removal with clear margins was associated with better survival11. Mohs micrographic surgery has been reported for a recurrent cutaneous myoepithelioma12.
5Skin cases read in full
The archive holds the full text of 6 papers that each describe a primary myoepithelial carcinoma of the skin or the fat beneath it. A model read each paper in full and recorded what it says13. This has not been checked by a clinician. The other published skin cases are indexed from their abstracts in the case registry.
| Patient and tumor | Treatment | What happened | Follow-up | Source |
|---|---|---|---|---|
| 56-year-old man occipital area of the head, dermis and subcutis, 15 cm | surgery, radiotherapy | 56-year-old man with a 15 cm occipital skin tumor growing for 6 years. Removed with clear margins, then radiotherapy (60 Gy in 30 fractions). Tumor carried a TRPS1::PLAG1 fusion. No recurrence at 26 months. | 26 months. Alive, no evidence of disease | Gillis 2026 |
| 44-year-old man left axilla, 3 cm | surgery, lymph node dissection, chemotherapy, radiotherapy (paclitaxel liposome, cisplatin) | 44-year-old man with left armpit masses for 1 year. Wide excision and lymph node dissection found cancer in 15 of 17 nodes. Six cycles of paclitaxel liposome plus cisplatin and 60 Gy radiotherapy followed; no recurrence reported (follow-up length not stated). | Alive, no evidence of disease | Zhu 2025 |
| 50-year-old woman left nasal wing, dermis, 0.7 cm | surgery, re-excision | 50-year-old woman with a 7 mm nodule on the left nasal wing, first diagnosed as a fibrous papule. It recurred after 6 months and margins were involved. Re-excision achieved clear margins, with no recurrence or metastasis after 6 years. | 72 months. Alive, no evidence of disease | Ben 2025 |
| 81-year-old man left malar cheek, dermis and subcutis, 1 cm | surgery, re-excision | 81-year-old man with a fast-growing cheek nodule of about 1 cm for 2 months, thought to be basal cell carcinoma. Excision left a close deep margin (0.95 mm); wider re-excision was complete. Yearly CT scans unremarkable so far; follow-up length not stated. | Asharaff 2025 | |
| 64-year-old man posterior left thigh, dermis, with partial infiltration into underlying muscle, 20 cm | chemotherapy, surgery, radiotherapy (CA regimen (paper defines it as cytarabine plus aclarubicin, but describes the dose reduction in terms of cisplatin and adriamycin), tegafur/gimeracil/oteracil potassium) | 64-year-old man with a 20 cm ulcerated tumor on the back of the left thigh and groin and pelvic lymph node spread. Chemotherapy gave little shrinkage; resection, radiotherapy and oral tegafur/gimeracil/oteracil followed, with no progression for two years. | 24 months. Alive with disease | Obata 2024 |
| 65-year-old woman left ankle, dermis and subcutis | surgery, re-excision, radiotherapy, targeted therapy (vemurafenib, cobimetinib) | 65-year-old woman with a left ankle tumor. After repeated excisions it recurred locally at 10 months, and radiotherapy failed. Tumor had BRAF V600E; 5 months of vemurafenib plus cobimetinib gave a complete response, with no evidence of disease 18 months after stopping. | Alive, no evidence of disease | Guidry 2020 |
6The literature
Papers naming the disease that mention this form in title or abstract, by year. Assigned by keyword13.
View as table, by decade
| Decade | Papers |
|---|---|
| 1950s | 1 |
| 1980s | 3 |
| 1990s | 3 |
| 2000s | 27 |
| 2010s | 55 |
| 2020s | 51 |
Browse all of them in the directory. See the published cases.
7Most cited papers
8Newest papers
9References
- Gillis T, Howell J, Jafarian F. TRPS1::PLAG1 Fusion in a Primary Cutaneous Myoepithelial Carcinoma: A Case Report and Literature Review. Journal of cutaneous pathology, 2026. PubMed ↩
- Mentzel T, Requena L, Kaddu S et al. Cutaneous myoepithelial neoplasms: clinicopathologic and immunohistochemical study of 20 cases suggesting a continuous spectrum ranging from benign mixed tumor of the skin to cutaneous myoepithelioma and myoepithelial carcinoma. Journal of cutaneous pathology, 2003. PubMed ↩
- Hornick JL, Fletcher CD. Cutaneous myoepithelioma: a clinicopathologic and immunohistochemical study of 14 cases. Human pathology, 2004. PubMed ↩
- DEVENYI I. [Malignant myoepithelioma of the sweat glands]. Zentralblatt fur allgemeine Pathologie u. pathologische Anatomie, 1957. PubMed ↩
- Flucke U, Palmedo G, Blankenhorn N et al. EWSR1 gene rearrangement occurs in a subset of cutaneous myoepithelial tumors: a study of 18 cases. Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc, 2011. PubMed ↩
- Zhu X, Li S. A rare primary cutaneous myoepithelial carcinoma in the axilla accompanied by lymph node metastasis: A case report. Cancer innovation, 2025. PubMed ↩
- 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 ↩
- Saddington P. Today, I'm Supposed to Die. TheAgileVC, 20 March 2025. source ↩
- Antonescu CR, Zhang L, Shao SY et al. Frequent PLAG1 gene rearrangements in skin and soft tissue myoepithelioma with ductal differentiation. Genes, chromosomes & cancer, 2013. PubMed ↩
- 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 ↩
- 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 ↩
- Miller TD, McCalmont T, Tope WD. Recurrent cutaneous myoepithelioma treated using Mohs micrographic surgery: case report and review of the literature. Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.], 2009. 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 ↩