13例隆突性皮肤纤维肉瘤病理特点观察及文献复习

Literature Review and Observation of Pathology Features on 13 Cases of Fibrosarcoma Juga Sex Skin

目的：探讨隆突性皮肤纤维肉瘤（dermatofibrosarcoma protuberans,DFSP）病理形态学特点、鉴别诊断及组织来源和预后.掌握DFSP的临床病理特点,避免与其他皮肤梭形细胞肿瘤混淆.方法：分析观察13例皮肤隆突性纤维肉瘤的临床资料病理学形态特征、免疫组化结果,结合文献并对照其免疫表型讨论肿瘤的来源和鉴别诊断,使用抗体为CD34、Vimentin、SMA、S-100,采用S-P法.结果：DFSP的类型多样,组织学图像虽各具特点,但突出的病理特征是真皮层中大量梭形细胞聚集呈车辐状或席纹状经典结构,瘤组织常浸润皮下脂肪组织内形成蜂窝状结构.分子标记为：本瘤细胞对波形蛋白（Vimentin）呈强而弥漫性的阳性反应；CD34一般呈强而弥漫性的阳性反应,文献报道阳性率为72～92％[1,2].溶菌酶（Lysozyme）呈局灶性阳性反应；平滑肌肌动蛋白（SMA）在DFSP的表达阳性率为50～95％,但是其表达常不稳定并且常呈局灶性[3-5].结论：DFSP好发于青壮年,男性稍多于女性,病变部位主要是躯干和四肢近侧端的真皮组织,也可发生于头颈部,易局部复发,也常被称为恶性潜能未定的潜在低度恶性肿瘤.极少发生转移,因此不是真正的恶性肿瘤[6,7].

Objective： To explore the promontory sex skin fibrosarcoma （dermatofibrosarcoma protuberans, DFSP） features, differential diagnosis and pathologic morphology tissue source and prognosis. To master the clinical pathological characteristics of DFSP, to avoid confusion with other skin spindle cell tumor.Methods to analyze observation 2 cases of skin juga sex fibrosarcoma clinical material pathology morphological characteristics, immunohistochemical results, combined with literature and control the immune phenotypic discuss the source of the tumor and the differential diagnosis, the use of antibodies for CD34, Vimentin, SMA, S - 100, the S - P method Result DFSP, histological image although with different characteristics,but outstanding pathological characteristics in the dermal layer is a large number of spindle cell gathered a rung shape or mat striate classical structure, tumor tissue often infiltrate subcutaneous adipose tissue formation in the cellular structure. Molecular markers for： the tumor cells of Vimentin （Vimentin） is strong and diffuse positive reaction; CD34 general is strong and diffuse positive reaction, the literature reported positive rate for 72-92%. Lysozyme （Lysozyme） the foci of positive reaction; Smooth muscle actin （SMA） in DFSP expression was fifty - 95%, but the expression often unstable and often with a focal . Conclusi.n DFSP occurs in young adults, more men than women, slightly pathological changes mainly is the trunk and limbs on the near side dermal tissue, can also occur in the head and neck, local recurrence, and is often referred to as potential of malignant potential in low-grade malignant tumor. Rarely shift, therefore is not really a malignant tumor .