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1How often it comes back
Often. In a 101-tumor soft-tissue series, 42% of malignant tumors recurred locally and 32% metastasized1. A review puts recurrence and metastasis at up to 40 to 50%2, and in one sarcoma unit 13 of 24 patients relapsed3. In salivary tumors, 27% of the patients with follow-up in one series developed distant metastases4, and in another, ten of 25 patients had recurrences, mostly more than one5. In children, nine of 17 whose tumors were completely removed with clear margins still had a local recurrence6.
So a recurrence is the expected course for many patients, and does not by itself mean that treatment failed or that nothing more can be done.
2When
It can be early or very late. Follow-up in the soft-tissue series ran from 4 to 252 months1. In children the deaths came at a median of nine months6, while in the pooled literature median progression-free survival was 48 months7. This is the reason for a long surveillance schedule.
3Where it spreads
The lung is the most common distant site: 83% of distant metastases in one salivary series4. Of patients with major salivary gland tumors and a recorded stage, 14.5% had lymph-node involvement and 6.6% had distant spread at diagnosis8.
Sites of spread named in 83 single-patient reports that describe metastasis, from the case registry. A report can name several.
4What was done
- More surgery. Complete removal with clear margins is the factor most tied to survival in the pooled data7, and re-excision is the usual first answer to a local recurrence that can be removed.
- Radiotherapy. After surgery it reduced local and regional recurrence in the pooled analysis7.
- Implanted radiation. In 27 patients with tumors of the mouth and face treated with radioactive iodine seeds, 14 of them after a recurrence that could not be operated on, recurrence-free survival was 51.9% at three years and overall survival 68.6%. Patients treated at first diagnosis did better than those treated at relapse9.
- Mohs surgery has been reported for a recurrent myoepithelioma of the skin10.
- Drugs. The catalog holds 32 reports of drug treatment for recurrent or metastatic disease. They are set out on the treatments page.
- Trials. The authors of the adult soft-tissue series encourage enrollment3. See trials to ask about.
5In the published cases
Of 362 single-patient reports read for the registry, 52 describe a local recurrence and 83 describe metastasis. Reports are not a sample of patients, so these are not rates. Browse the reports.
6What to ask
- Is this a recurrence where the first tumor was, a new tumor, or spread? How do we know?
- Can it be removed with a clear margin?
- Should the new tissue be tested for fusions, if the first was not?
- Is radiotherapy an option here, including implanted radiation?
- Is there a trial open to me now?
- What will the surveillance schedule be after this?
7References
- 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 ↩
- 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 ↩
- 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 ↩
- 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 ↩
- 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 ↩
- 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 ↩
- 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 ↩
- Luo Y. Myoepithelial carcinoma of major salivary glands: Analysis of population-based clinicopathologic and prognostic features. Translational oncology, 2022. PubMed ↩
- Zheng L, Lv X, Shi Y et al. (125)I interstitial brachytherapy for the treatment of myoepithelial carcinoma of the oral and maxillofacial region. Brachytherapy, 2016. 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 ↩