Perivascular epithelioid cell tumor of the orbit: a case report and review of the literature.

@article{Iyengar2005PerivascularEC,
  title={Perivascular epithelioid cell tumor of the orbit: a case report and review of the literature.},
  author={Pratibha Iyengar and Dan D Deangelis and Mark Greenberg and Glenn Taylor},
  journal={Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society},
  year={2005},
  volume={8 1},
  pages={98-104}
}
We present a unique case of a perivascular epithelioid cell tumor (PEComa) in the orbit of a 9-year-old female patient. The entity of PEComas has been described only recently. Characteristic histologic features and an immunohistochemical profile of negativity for epithelial markers and positivity for melanogenesis-related markers define the tumors. In children and young adults, this tumor has a predilection for the falciform ligament and ligamentum teres of the liver. It is associated with, but… CONTINUE READING