目的：探讨眼眶软组织肿瘤临床病理特征。

方法：回顾性分析我院2003-11/2018-11眼眶软组织肿瘤患者455例的病例资料，观察其临床特点、病理分类和少见肿瘤的病理学特征。

结果：眼眶软组织肿瘤患者455例中，良性肿瘤421例(92.5%)，居于前5位的依次为海绵状血管瘤258例、毛细血管瘤58例、神经纤维瘤16例、纤维瘤15例和神经鞘瘤14例； 中间型27例(5.9%)，其中孤立性纤维性肿瘤(SFT)23例、低度恶性纤维组织细胞瘤2例、低度恶性黏液性神经纤维瘤2例； 恶性7例(1.5%)，其中眼眶恶性SFT者2例，眼眶髓系肉瘤(MS)2例，黏液样脂肪肉瘤(MLS)、梭形细胞未分化肉瘤和外周原始神经外胚叶肿瘤(PNET)各1例。对部分病例进行免疫组化和分子检测，结果发现新抗体如STAT6和分子检测技术的应用能提高诊断准确性。

结论：眼眶软组织肿瘤中，良性肿瘤占大部分，脉管肿瘤中的海绵状血管瘤居于首位，不乏眼眶部位少见软组织肿瘤病理学类型如Erdheim-Chester病(ECD)、砂粒体性骨化性纤维瘤(POF)、平滑肌瘤和黏液瘤等； 中间型和恶性肿瘤少见，眼眶部位SFT、MLS、梭形细胞未分化肉瘤和MS等少见的病理学类型常诊断困难，容易漏诊误诊，新型免疫组化抗体和分子检测技术能提高诊断的准确率。

METHODS: A retrospective analysis of 455 cases of orbital soft tissue tumors in our hospital from 2003-11/2018-11 were performed to observe the clinical features, pathological classification and pathological features of rare tumors.

RESULTS:All 455 patients with orbital soft tissue tumors, 421(92.5%)were benign tumors. The top 5 were 258 cases of cavernous hemangioma, 58 cases of capillary hemangioma, 16 cases of neurofibroma, 15 cases of fibroid, and 14 cases of schwannoma; 27 cases(5.9%)of intermediate type, including 23 cases of solitary fibrous tumor(SFT), 2 cases of low-grade malignant fibrous histiocytoma, 2 cases of low-grade mucinous neurofibroma; 7 cases(1.5%)of malignant type, 2 cases of orbital malignant SFT, 2 cases of orbital myeloid sarcoma(MS), mucinous liposarcoma(MLS), spindle cell undifferentiated sarcoma and peripheral primitive neuroectodermal tumor(PNET)1 case. Immunohistochemistry and molecular detection of some cases revealed that the application of new antibodies such as STAT6 and molecular detection techniques can improve the diagnostic accuracy.

CONCLUSION:Benign tumors account for the majority of orbital soft tissue tumors. Cavernous hemangioma in vascular tumors is the first, and there are few soft tissue tumor pathological types such as Erdheim-Chester disease(ECD)and granulomatous ossifying fibers. Tumors(POF), leiomyomas and myxomas; intermediate and malignant tumors are rare; pathological types such as SFT, MLS, spindle cell undifferentiated sarcoma and MS are often difficult to diagnose, easy to miss diagnosis and misdiagnosis, new immunity histochemical antibodies and molecular detection techniques can improve the accuracy of the diagnosis.