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

  • Jason DiabEmail author
  • Tomas O’Neill
  • Lynette Moore
  • Stephen Santoreneos
  • Walter Flapper
Case Report


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.


Craniofacial Neoplasms PEComa 



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

Compliance with ethical standards


There was no funding for this research publication.

Conflict of interest

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

Ethical approval

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

Informed consent

Informed consent has been obtained for this research publication.

Patient consent

The patient provided written consent for the use of her image.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Australian Craniofacial UnitAdelaideAustralia
  2. 2.Department of PathologyWomen and Children’s HospitalAdelaideAustralia
  3. 3.Department of NeurosurgeryRoyal Adelaide HospitalAdelaideAustralia

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