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Non-cutaneous syncytial myoepitheliomas are identical to cutaneous counterparts: a clinicopathologic study of 24 tumors occurring at diverse locations

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Abstract

Aims

Cutaneous syncytial myoepithelioma (CSM) is a rare myoepithelioma variant of skin, characterized by intradermal syncytial growth of spindle cells with a distinct immunophenotype of EMA and S100 positivity and infrequent keratin expression. While CSM was first described as a cutaneous tumor, singular non-cutaneous cases have since been reported in bone. We aimed to investigate the clinicopathological features of this variant across all anatomic sites through a large multi-institutional study.

Methods and results

We complied a total of 24 myoepitheliomas with syncytial growth from our files. The tumors occurred in 12 male and 12 female patients (M:F = 1:1), with a median age of 31 years (range, 9–69 years). While the majority of tumors (75%, n = 18) occurred in skin, a significant subset (25%, n = 6) arose in non-cutaneous sites, including bone (n = 3), bronchus/trachea (n = 2), and interosseous membrane of tibia/fibula (n = 1). Tumor size ranged from 0.4 to 5.9 cm. Clinical follow-up (7 patients; range 14–202 months; median 56.5 months) showed a single local recurrence 8 years after incomplete skin excision but no metastases; all patients were alive at the time of last follow-up without evidence of disease. Histologically, all tumors were pink at low-power and characterized by a syncytial growth of bland ovoid, spindled, or histiocytoid cells with eosinophilic cytoplasm and prominent perivascular lymphoplasmacytic inflammation. One-third displayed adipocytic metaplasia (8/24). Rare cytologic atypia was seen but was not associated with increased mitotic activity. All tumors expressed S100, SMA, and/or EMA. Keratin expression was absent in most cases. Molecular analysis was performed in 16 cases, all showing EWSR1-rearrangments. In total, 15/15 (100%) harbored an EWSR1::PBX3 fusion, whereas 1 case EWSR1 FISH was the only molecular study performed.

Conclusion

Syncytial myoepithelioma is a rare but recognizable morphologic variant of myoepithelioma which may have a predilection for skin but also occurs in diverse non-cutaneous sites. Our series provides evidence supporting a reappraisal of the term “cutaneous syncytial myoepithelioma,” as 25% of patients in our series presented with non-cutaneous tumors; thus, we propose the term “syncytial myoepithelioma” to aid pathologist recognition and avoidance of potentially confusing terminology when referring to non-cutaneous examples. The behavior of syncytial myoepithelioma, whether it arises in cutaneous or non-cutaneous sites, is indolent and perhaps benign with a small capacity for local recurrence.

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Data availability

The data supporting the findings of this study are available upon request from the corresponding author.

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Acknowledgements

The authors thank Norman Barker, MS, MA, RBP for his assistance with preparing figures for this article.

Author information

Authors and Affiliations

  1. Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA

    Sintawat Wangsiricharoen

  2. Department of Pathology, Emory University School of Medicine, Atlanta, GA, USA

    Sandra G. Gjeorgjievski & Armita Bahrami

  3. Department of Pathology, The Mayo Clinic, Rochester, MN, USA

    Jorge Torres-Mora

  4. Department of Pathology, Johns Hopkins University School of Medicine, 401 N Broadway, Weinberg Building 2245, Baltimore, MD, 21231, USA

    Ying S. Zou & John M. Gross

  5. Department of Pathology, Faculty of Medicine in Plzen, Charles University, Pilsen, Czech Republic

    Michael Michal

  6. Bioptical Laboratory, Ltd., Pilsen, Czech Republic

    Michael Michal

  7. Department of Pathology, Stanford University School of Medicine, Stanford, CA, USA

    Gregory W. Charville

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Contributions

SW reviewed all cases, analyzed data, and wrote the first draft of the manuscript. SGG, AB, JTM, YSZ, MM, and GWC contributed to data collection. JMG designed the study, reviewed all cases, and reviewed and edited the manuscript. All authors edited the manuscript and read and approved the final manuscript.

Corresponding author

Correspondence to John M. Gross.

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The authors declare the compliance of ethical standards in research. This study was approved by the Johns Hopkins Institutional Review Board (IRB00379801, 3/21/2023).

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The data are not publicly available due to privacy or ethical restrictions.

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The authors declare no competing interests.

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Wangsiricharoen, S., Gjeorgjievski, S.G., Bahrami, A. et al. Non-cutaneous syncytial myoepitheliomas are identical to cutaneous counterparts: a clinicopathologic study of 24 tumors occurring at diverse locations. Virchows Arch 483, 665–675 (2023). https://doi.org/10.1007/s00428-023-03609-3

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