AIM: To investigate the clinicopathological features of orbit primary liposarcoma. METHODS: By means of histological, ultrastructural and immunohistochemical studies, the clinical and pathological features of two cases of orbit primary liposarcoma were analyzed and compared with 6 cases of primary retroperitoneum liposarcoma. RESULTS: Two cases of orbit primary liposarcoma were young persons. The majority of cases of primary retroperitoneum liposarcoma were aged over 40 years. The tumors were located at the retrobulbar region, and involved the wall of eyeball and optic nerve. The tumor cells of case 1 were round, with abundant eosinophilic granules in the cytoplasm, and there were lipoblasts in the tumor cells of two cases. Local recurrence following excision occurred in case 2.Immunohistochemical expression of S100 protein was positive in all cases, and CK (AE1/AE3), desmin, MyoD1, melanoma were negative. Ultrastructure observation showed lipid drops in the tumor cells in case 2 of orbit primary liposarcoma. CONCLUSION: Primary liposarcoma of the orbit may recur.The diagnosis was based on histological,ultrastruc-tural observation and immunohistochemical staining.%目的:探讨眼眶原发性脂肪肉瘤的临床和病理特点.方法:收集2例眼眶原发性脂肪肉瘤和6例腹膜后原发性脂肪肉瘤的临床资料,病理标本光镜取材切片观察,免疫组织化学作CK(AE1/AE3),Desmin,MyoD1,S100-protein,Melanoma标记.眼眶原发性脂肪肉瘤的例2 作透射电镜观察.结果:原发性眼眶脂肪肉瘤的患者年青,原发性腹膜后脂肪肉瘤的患者年龄以40岁以上者为主(5/6).原发性眼眶患者症状显著但肿瘤小,原发性腹膜后患者症状轻,但肿瘤巨大.两组在组织学上分布于脂肪肉瘤的各型.肿瘤易复发,但无转移.结论:眼眶原发性脂肪肉瘤易复发,诊断依靠光镜和电镜观察以及免疫组织化学染色.
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