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


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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  • 02 September 2020

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


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

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Correspondence to Jason Diab.

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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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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).

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  • Craniofacial
  • Neoplasms
  • PEComa