Hei Yan, Kang Li, Yang Xinji, Wang Yi, Lu Xiaozhong, Li Yueyue, Zhang Wei, Xiao Lihua
Institute of Orbital Disease, General Hospital of Chinese People's Armed Police Forces, Beijing 100039, P.R. China.
Oncol Lett. 2018 Jan;15(1):304-314. doi: 10.3892/ol.2017.7286. Epub 2017 Oct 30.
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.
肺泡软组织肉瘤（ASPS）是一种罕见的软组织肿瘤。在头颈部发生的ASPS中，眼眶受累的发生率最高。然而，关于其临床特征的信息有限。本研究呈现了一家医院过去15年中8例眼眶ASPS的临床表现、影像学表现、病理特征、治疗策略及预后记录，并对文献进行了综述。其中包括3例男性和5例女性患者，就诊时的中位年龄为9.5岁。肿瘤平均最大直径为3.6厘米。共有5例患者接受了肿瘤手术切除，2例行眼眶内容剜除术，1例行部分眼眶内容剜除术。总共6例患者接受了术后放疗，2例接受了化疗。随访时，6例患者情况良好，无复发或转移迹象。2例患者出现局部复发，其中1例在停止治疗后死亡。根据本系列病例及文献中提及的病例，眼眶肺泡软组织肉瘤具有与其他部位发生的肺泡软组织肉瘤不同的特征。眼眶肺泡软组织肉瘤多见于较年轻人群，病程较短，肿瘤较小，预后较好，与眼外肌有明显关联，且Ki-67增殖指数可能与疾病预后相关。
