隆突性皮肤纤维肉瘤的临床病理及免疫组织化学研究

A clinico pathological and immunohistochemical study on dermatofibrosarcoma protuberans

目的 探讨隆突性皮肤纤维肉瘤的诊断、鉴别诊断、复发原因及其组织起源。方法 对 2 6例隆突性皮肤纤维肉瘤的临床和病理资料进行分析和随访 ,全部标本进行HE、Foot网状纤维染色 ,并采用免疫组织化学ABC法对Vi mentin、AACT、S 10 0、Keratin、NSE和Desmin 6种抗体标记结果进行观察。结果 该肿瘤属低度恶性 ,生长缓慢 ,很少转移 ,但易复发 ;在组织学上具有特征性结构 :瘤细胞围绕胶原纤维或毛细血管作典型的辐射状排列。肿瘤细胞呈梭形、异型性不明显、核分裂少见。免疫组化结果 :2 6例全部对Vimentin呈阳性反应 ,6例对AACT呈阳性反应 ,5例对S -10 0呈阳性反应 ,2 6例全部对Keratin、Desmin和NSE呈阴性反应。结论 隆突性皮肤纤维肉瘤在诊断上 ,主要根据HE染色观察 ,形态上典型 ,结合其临床和病理特征即可确诊 ,对隆突性皮肤纤维肉瘤手术范围不宜过于保守 ,局部要彻底切除干净 ,则可减少其复发机会 ,该肿瘤可能起源于真皮内的具有多种分化潜能的间叶细胞。

Objective To inquire into the diagnosis,differential diagnosis,recurrent factors and the origin of its tissue of dermatofibrosarconma protuberans(DFSP).Methods The clinical and pathological data from 26 cases of DFSP were analyzed.All the cases were followed up.The sections were stained with HE and Foot reticulum method.The expression of 6 antibodies such as Vimentin,AACT,S 100,Keratin,NSE and Desmin were observed immunohistochemically .Results DFSP,with low malignancy,slow growth,rare metastasis,was easy to relapse.The morphologic feature of DFSP was that the tumor cells surrounded collagen fibers or capillaries,typically arranging in spokeform.The tumor cells were in spindel from with no distinctive atypia and less mitoses. 26 cases showed positive to vimentin, 6 cases showed positive to AACT,5 cases showed positive to S 100. All cases were negative to Kenation,desmin and NSE.Conclusions The diagnosis of DTSP is mostly based on to the observation of HE staining as well as the clinical and pathological features if the pattern is typical. The natural history of DFSP is characterized by an extremely high tendency of local recurrence due to limited resection.It is clear that wide local excision of this lesion can markedly reduce the risk of recurrence.DFSP probaly originates from the pluripotential mesoblastema in the dermis. [