Add like
Add dislike
Add to saved papers

Giant Perivascular Epithelioid Cell Tumor of the Orbit: A Clinicopathological Analysis and Review of the Literature.

Aim: To describe and review the clinical, radiological, and histopathological characteristics of an orbital perivascular epithelioid cell tumor (PEComa).

Methods: A systematic review of clinical records, radiological investigations, microscopic features, and immunohistochemical characteristics was done.

Results: A 9-year-old female child presented with a year-long history of a large orbital mass associated with painless, progressive proptosis of the right eye. Radiologically, a well-defined orbital mass was seen with no intracranial extension. Excision was performed and histopathological examination showed uniform epithelioid cells in nests separated by thin fibrovascular septae. The tumor cells stained positively for Human Melanoma Black-45, but neg-atively for desmin, S-100, smooth muscle actin, MyoD1, microphthalmia-associated transcription factor, vimentin, CD10, CD31, and CD34 with a low proliferation index of 5-7%. Based on the tumor's morphological and immuno-histochemical characteristics, a diagnosis of giant orbital PEComa was made. No recurrence was seen at the last follow-up.

Conclusions: PEComas are uncommon mesenchymal neoplasms that have typical histological features, with an immunohistochemical profile of negativity for epithelial markers and positivity for melanocytic markers. For benign PEComas, complete excision is advised. However, since PEComas elsewhere in the body have been known to be malignant, a close follow-up of such cases is recommended.

Full text links

We have located links that may give you full text access.
Can't access the paper?
Try logging in through your university/institutional subscription. For a smoother one-click institutional access experience, please use our mobile app.

For the best experience, use the Read mobile app

Mobile app image

Get seemless 1-tap access through your institution/university

For the best experience, use the Read mobile app

All material on this website is protected by copyright, Copyright © 1994-2024 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

By using this service, you agree to our terms of use and privacy policy.

Your Privacy Choices Toggle icon

You can now claim free CME credits for this literature searchClaim now

Get seemless 1-tap access through your institution/university

For the best experience, use the Read mobile app