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Perivascular Epithelioid Cell Tumor of the Orbit: A Case Report and Review of the Literature

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Pediatric and Developmental Pathology

Abstract

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 not exclusive to, tuberous sclerosis. To the best of our knowledge, this is the first reported case of a PEComa of the orbit in a child or adult. The main differential diagnoses for this melanin pigment-producing lesion include melanoma and pigmented paraganglioma. The histologic features, immunohistochemical profile, ultrastructural studies, and molecular studies led us to favor a diagnosis of PEComa. The prognosis of this entity is undetermined due largely to the small number of reported cases.

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Acknowledgments

Dr. Greenberg holds the Pediatric Oncology Group of Ontario Chair in Childhood Cancer Control at the University of Toronto, Toronto, Ontario, Canada. The authors thank Dr. J. Srigley, Department of Laboratory Medicine, The Credit Valley Hospital, Mississauga, Ontario, for review of the pathology and editorial comments.

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Authors and Affiliations

  1. Department of Pathology, Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada

    Pratibha Iyengar & Glenn Taylor

  2. Department of Laboratory Medicine and Pathobiology, University of Toronto, 100 College Street, Toronto, ON, M5G 1L5, Canada

    Pratibha Iyengar & Glenn Taylor

  3. Department of Ophthalmology and Vision Sciences, Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada

    Dan D. DeAngelis

  4. Departments of Hematology/Oncology and Pediatrics, Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada

    Mark Greenberg

Authors
  1. Pratibha Iyengar
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  2. Dan D. DeAngelis
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  3. Mark Greenberg
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  4. Glenn Taylor
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Correspondence to Glenn Taylor.

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Iyengar, P., DeAngelis, D.D., Greenberg, M. et al. Perivascular Epithelioid Cell Tumor of the Orbit: A Case Report and Review of the Literature. Pediatr Dev Pathol 8, 98–104 (2005). https://doi.org/10.1007/s10024-004-5055-0

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  • DOI: https://doi.org/10.1007/s10024-004-5055-0

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