Dr.AKSHAY NAIR
Aim:To describe the clinical,radiological,histopathological (HPE) and immunohistochemistry (IHC) characteristics of an orbital Perivascular Epithelioid Cell Tumor(PEComa).
Methods & Results: A 9-year-old female presented with a 1 year history of a large painless superior orbital mass and progressive proptosis (OD).On imaging, the mass was well defined, contrast-enhancing with no intracranial extension.Excision biopsy was done and HPE showed uniform epithelioid cells in nests separated by thin fibrovascular septae.Tumor cells had eosinophilic-clear cytoplasm & vesicular nuclei.IHC was positive for HMB-45, negative for Desmin, S-100,SMA, MyoD1,MITF,Vimentin,CD10, CD31 & CD 34.Ki-67 index was 5%.Based on its morphological & IHC pattern, a diagnosis of orbital PEComa was made.
Conclusion: PEComas are rare mesenchymal neoplasms with only 5 previously reported cases.They have typical histologic features & IHC profile.For benign PEComas, complete excision and close follow up is recommended.
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