Purpose To stuffy the clinicopathologic, radiologic features, pathologic diagnosis and differential diagnosis of small cell osteosarcoma. Methods The clinical, radiologic, pathologic features and follow-up information of 14 cases of small cell osteosarcoma were reviewed, and categorized on the basis of 2002 WHO classification of tumors of bone. Immunohistochemical study using En Vision method for a panel of antibodies was earned out. Results Among the 14 cases studied, the male-to-female ratio was 1 : 1. The age of the patients ranged from 12 to 73 years ( mean age 34 years, median age 29 years ). The most common site of involvement was metaph-ysis of long tubular bone ( 7/14, 50% ). Radiologicaly, most small cell osteosarcoma showed a mixed lytic and mineralized pattern with soft tissue extension. Histologically, the leisons were composed the predominance of small to intermediate-sized round cell or short spindle cells with scant coarse lace-like osteoid. Immunohistochemical study showed the osteosarcoma cells were diffusely positive for vimentin ( 10/10, 100% ), CD99( 7/10, 70% )and BCL-2( 4/10, 40% ), and weakly positive for S-100 ( 2/10, 20% ) and Syn ( 2/ 10, 20% ). Conclusions Small cell osteosarcoma has been described as a rare osteosarcoma, which should be distinguished from the other small cell tumors of bone. Correlations of clinical, radiologic and pathologic features are helpful in the definitive diagnosis of small cell osteosarcoma.%目的 探讨小细胞性骨肉瘤的临床特征、影像学表现、病理诊断及鉴别诊断.方法 收集14例小细胞性骨肉瘤的临床病理资料,按2002年WHO骨肿瘤分类标准重新复片,进行临床、影像学、病理学特征分析并采用EnVision法行免疫组化染色.结果 14例小细胞性骨肉瘤男女比1:1.年龄12～73岁(平均34岁,中位29岁).肿瘤多位于长管状骨干骺端(7/14,50%).影像学表现以溶骨性骨质破坏为主伴钙化,并见软组织肿块.组织学表现为小～中等的圆形或短梭形肿瘤细胞弥漫性分布,伴有灶性纤细的花边状肿瘤性成骨.免疫表型:vimentin(10/10,100%)、CD99(7/10,70%)、BCL-2(4/10,40%)均呈阳性;S-1OO(2/10,20%)和Syn(2/10,20%)均呈弱阳性.结论 小细胞性骨肉瘤为少见、特殊类型的骨肉瘤.小细胞性骨肉瘤的诊断需密切结合临床、影像学及病理特征,需与发生于骨的多种其他类型的小细胞恶性肿瘤鉴别.
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