Open Access

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
  • View Affiliations

  • 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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January 2018
Volume 15 Issue 1

Print ISSN: 1792-1074
Online ISSN:1792-1082

2016 Impact Factor: 1.39
Ranked #68/217 Oncology
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APA
Hei, Y., Kang, L., Yang, X., Wang, Y., Lu, X., Li, Y. ... Xiao, L. (2018). Orbital alveolar soft part sarcoma: A report of 8 cases and review of the literature. Oncology Letters, 15, 304-314. https://doi.org/10.3892/ol.2017.7286
MLA
Hei, Y., Kang, L., Yang, X., Wang, Y., Lu, X., Li, Y., Zhang, W., Xiao, L."Orbital alveolar soft part sarcoma: A report of 8 cases and review of the literature". Oncology Letters 15.1 (2018): 304-314.
Chicago
Hei, Y., Kang, L., Yang, X., Wang, Y., Lu, X., Li, Y., Zhang, W., Xiao, L."Orbital alveolar soft part sarcoma: A report of 8 cases and review of the literature". Oncology Letters 15, no. 1 (2018): 304-314. https://doi.org/10.3892/ol.2017.7286