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An index case of primary osseous PEComa in a paediatric craniofacial skeleton

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A Correction to this article was published on 02 September 2020

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Abstract

Perivascular epithelioid cell tumours, known as PEComas, are a family of tumours with immunoreactive melanocytic and smooth muscle markers. They are a rare heterogeneous group in adulthood and childhood with primary osseous PEComas representing an even rarer subgroup. The clinical behaviour is not well understood and the treatment options vary. We present an index case of a primary osseous PEComa of the craniofacial skeleton in a 3-year-old girl.

Level of evidence: Level V, diagnostic; therapeutic; risk/prognostic study.

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Change history

  • 02 September 2020

    The correction should include: We would like to acknowledge the contributions of Dr. Vani P Atluri [1] to the article.

References

  1. Thyway KH, Fisher C (2015) PEComa: morphology and genetics of a complex tumour family. Ann Diagn Pathol 19:359–368

    Article  Google Scholar 

  2. Malinowska I, Kwiatkowski DJ, Weiss S, Martignoni G, Netto G, Argani P (2012) Perivascular epithelioid cell tumors (PEComas) harboring TFE3 gene rearrangements lack the TSC2 alterations characteristic of conventional PEComas: further evidence for a biological distinction. Am J Surg Pathol 36(5):783–784

    Article  Google Scholar 

  3. Chang KL, Folpe AL (2001) Diagnostic utility of microphthalmia transcription factor in malignant melanoma and other tumors. Adv Anat Pathol 8(5):273–275

    Article  CAS  Google Scholar 

  4. Folpe AL, Kwiatkowski DJ (2010) Perivascular epithelioid cell neoplasms: pathology and pathogenesis. Hum Pathol 41:1–15

    Article  CAS  Google Scholar 

  5. Yamashita K, Fletcher C (2010) PEComa presenting in bone: clinicopathologic analysis of 6 cases and literature review. AM J Surg Pathol 34(11):1623–1629

    Google Scholar 

  6. Bleeker JS, Quevedo JF, Folpe AL (2012) “Malignant” perivascular epithelioid cell neoplasm: Risk stratification and treatment strategies. Sarcoma. https://doi.org/10.1155/2012/541626

  7. Benson C, Vitfell-Rasmussen J, Maco M et al (2014) A retrospective study of patients with malignant PEComa receiving treatment with sirolimus or temsirolimus: The Royal Marsden Hospital experience. Anticancer Res 34:3663–3668

    PubMed  Google Scholar 

  8. Varan A, Bayhan T, Kiratli H, Özoğul E, Kösemehmetoğlu K, Bulut E, Akyüz C (2017) An orbital perivascular epithelioid cell tumor in a 7-year-old boy: case report and review of the literature. JAAPOS 21(4):325–328

    Google Scholar 

Download references

Acknowledgements

We would like to express our gratitude to the members of the Australian Craniofacial Unit.

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Authors and Affiliations

  1. Australian Craniofacial Unit, 72 King William St., Adelaide, 5006, Australia

    Jason Diab, Tomas O’Neill & Walter Flapper

  2. Department of Pathology, Women and Children’s Hospital, Adelaide, Australia

    Lynette Moore

  3. Department of Neurosurgery, Royal Adelaide Hospital, Adelaide, Australia

    Stephen Santoreneos

Authors
  1. Jason Diab
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  2. Tomas O’Neill
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  3. Lynette Moore
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  4. Stephen Santoreneos
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  5. Walter Flapper
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Corresponding author

Correspondence to Jason Diab.

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There was no funding for this research publication.

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Jason Diab, Tomas O’Neill, Lynette Moore, Stephen Santoreneos, and Walter Flapper declare that they have no conflict of interest.

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The human research and ethics committee has approved this research publication with no restrictions.

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Diab, J., O’Neill, T., Moore, L. et al. An index case of primary osseous PEComa in a paediatric craniofacial skeleton. Eur J Plast Surg 42, 629–632 (2019). https://doi.org/10.1007/s00238-019-01544-8

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  • DOI: https://doi.org/10.1007/s00238-019-01544-8

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