Primary Epithelioid Sarcoma of Orbit: A Case Report and Review of the Literature

Epithelioid sarcoma is a rare high-grade malignancy identified by Enzinger in 1970. It accounts for 1% of all reported soft tissue sarcomas and presents most commonly in distal upper extremities in young adults with a male predominance. At this time, there are only 5 previously reported cases of pri...

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Main Authors: Erin A. Kaya, Talmage J. Broadbent, Cheddhi J. Thomas, Aaron E. Wagner, Steve H. Thatcher, Wayne T. Lamoreaux, Robert K. Fairbanks, Christopher M. Lee
Format: Article
Language:English
Published: Wiley 2018-01-01
Series:Case Reports in Oncological Medicine
Online Access:http://dx.doi.org/10.1155/2018/3989716
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author Erin A. Kaya
Talmage J. Broadbent
Cheddhi J. Thomas
Aaron E. Wagner
Steve H. Thatcher
Wayne T. Lamoreaux
Robert K. Fairbanks
Christopher M. Lee
author_facet Erin A. Kaya
Talmage J. Broadbent
Cheddhi J. Thomas
Aaron E. Wagner
Steve H. Thatcher
Wayne T. Lamoreaux
Robert K. Fairbanks
Christopher M. Lee
author_sort Erin A. Kaya
collection DOAJ
description Epithelioid sarcoma is a rare high-grade malignancy identified by Enzinger in 1970. It accounts for 1% of all reported soft tissue sarcomas and presents most commonly in distal upper extremities in young adults with a male predominance. At this time, there are only 5 previously reported cases of primary epithelioid sarcoma of the orbit. We present a primary orbital epithelioid sarcoma case of a patient who underwent orbital exenteration followed by external beam radiation treatment. Because the literature is limited, this is to our knowledge the largest descriptive analysis of cases of orbital epithelioid sarcoma. We also provide a detailed review of all the previously reported primary orbital epithelioid sarcoma cases, as well as a discussion on the use of postoperative radiation therapy for patients with epithelioid sarcoma. Surgical resection followed by adjuvant radiation therapy appears to be a safe option for local treatment of this rare malignancy, but further future studies are needed of this rare clinical situation in order to better understand and optimize treatment for patients with orbital epithelioid sarcoma.
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spelling doaj-art-e7f44661e4d849e9b75121bd8b9d58572025-08-20T02:22:15ZengWileyCase Reports in Oncological Medicine2090-67062090-67142018-01-01201810.1155/2018/39897163989716Primary Epithelioid Sarcoma of Orbit: A Case Report and Review of the LiteratureErin A. Kaya0Talmage J. Broadbent1Cheddhi J. Thomas2Aaron E. Wagner3Steve H. Thatcher4Wayne T. Lamoreaux5Robert K. Fairbanks6Christopher M. Lee7Department of Radiation Oncology, Cancer Care Northwest, Spokane, WA, USANorthwest Eyelid and Orbital Specialists, Spokane, WA, USAIncyte Diagnostics, Spokane, WA, USADepartment of Radiation Oncology, Cancer Care Northwest, Spokane, WA, USADepartment of Radiation Oncology, Cancer Care Northwest, Spokane, WA, USADepartment of Radiation Oncology, Cancer Care Northwest, Spokane, WA, USADepartment of Radiation Oncology, Cancer Care Northwest, Spokane, WA, USADepartment of Radiation Oncology, Cancer Care Northwest, Spokane, WA, USAEpithelioid sarcoma is a rare high-grade malignancy identified by Enzinger in 1970. It accounts for 1% of all reported soft tissue sarcomas and presents most commonly in distal upper extremities in young adults with a male predominance. At this time, there are only 5 previously reported cases of primary epithelioid sarcoma of the orbit. We present a primary orbital epithelioid sarcoma case of a patient who underwent orbital exenteration followed by external beam radiation treatment. Because the literature is limited, this is to our knowledge the largest descriptive analysis of cases of orbital epithelioid sarcoma. We also provide a detailed review of all the previously reported primary orbital epithelioid sarcoma cases, as well as a discussion on the use of postoperative radiation therapy for patients with epithelioid sarcoma. Surgical resection followed by adjuvant radiation therapy appears to be a safe option for local treatment of this rare malignancy, but further future studies are needed of this rare clinical situation in order to better understand and optimize treatment for patients with orbital epithelioid sarcoma.http://dx.doi.org/10.1155/2018/3989716
spellingShingle Erin A. Kaya
Talmage J. Broadbent
Cheddhi J. Thomas
Aaron E. Wagner
Steve H. Thatcher
Wayne T. Lamoreaux
Robert K. Fairbanks
Christopher M. Lee
Primary Epithelioid Sarcoma of Orbit: A Case Report and Review of the Literature
Case Reports in Oncological Medicine
title Primary Epithelioid Sarcoma of Orbit: A Case Report and Review of the Literature
title_full Primary Epithelioid Sarcoma of Orbit: A Case Report and Review of the Literature
title_fullStr Primary Epithelioid Sarcoma of Orbit: A Case Report and Review of the Literature
title_full_unstemmed Primary Epithelioid Sarcoma of Orbit: A Case Report and Review of the Literature
title_short Primary Epithelioid Sarcoma of Orbit: A Case Report and Review of the Literature
title_sort primary epithelioid sarcoma of orbit a case report and review of the literature
url http://dx.doi.org/10.1155/2018/3989716
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