Orbital alveolar soft part sarcoma: A report of 8 cases and review of the literature

Hei,Yan; Kang,Li; Yang,Xinji; Wang,Yi; Lu,Xiaozhong; Li,Yueyue; Zhang,Wei; Xiao,Lihua

doi:10.3892/ol.2017.7286

Orbital alveolar soft part sarcoma: A report of 8 cases and review of the literature

Authors:
- Yan Hei
- Li Kang
- Xinji Yang
- Yi Wang
- Xiaozhong Lu
- Yueyue Li
- Wei Zhang
- Lihua Xiao
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Affiliations: Institute of Orbital Disease, General Hospital of Chinese People's Armed Police Forces, Beijing 100039, P.R. China
Published online on: October 30, 2017 https://doi.org/10.3892/ol.2017.7286
Pages: 304-314
Copyright: © Hei et al. This is an open access article distributed under the terms of Creative Commons Attribution License.

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Abstract

Alveolar soft part sarcoma (ASPS) is a rare soft tissue neoplasm. The incidence of orbital involvement is the highest in ASPS arising in the head and neck region. However, limited information is available regarding its clinical characteristics. The present study presents the clinical manifestations, imaging findings, pathological features, treatment strategies and prognosis records of 8 cases of orbital ASPS over the last 15 years in a single hospital, along with a review of the literature. Included were 3 male and 5 female patients, with the median age at presentation being 9.5 years. The mean average largest tumour diameter was 3.6 cm. A total of 5 patients underwent surgical excision of the tumour, with 2 undergoing orbital exenteration and 1 undergoing partial orbital exenteration. In total, 6 patients received postoperative radiotherapy and 2 received chemotherapy. Upon follow‑up, 6 patients were doing well with no evidence of recurrence or metastasis. Local recurrence developed in 2 patients, of whom 1 succumbed following withdrawal from treatment. According to the present series and the cases mentioned in the literature, orbital alveolar soft part sarcoma has characteristics distinct from those of alveolar soft part sarcoma which arises in other locations. Orbital alveolar soft part sarcoma presents itself in a younger population with a shorter course of disease, smaller tumour size, improved prognosis, a marked association with the extraocular muscles and with the Ki‑67 proliferation index possibly associated with prognosis of the disease.

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