隆突性皮肤纤维肉瘤远处转移4例临床病理分析

Dermatofibrosarcoma protuberans with distant metastasis:a clinicopathologic analysis of four cases

目的探讨隆突性皮肤纤维肉瘤(dermatofibrosarcoma protuberans,DFSP)远处转移的临床病理学特征和转移的高危因素。方法收集4例转移性DFSP的临床资料,行免疫组化和FISH检测,并复习相关文献。结果4例转移性DFSP,原发灶1例为经典型DFSP,1例为经典型DFSP伴20%黏液样变,2例为纤维肉瘤型隆突性皮肤纤维肉瘤(fibrosarcomatous dermatofibrosarcoma protuberans,FS-DFSP);4例转移灶为FS-DFSP,均发生肺转移,1例合并头部顶叶转移。经典型DFSP组织学表现为梭形肿瘤细胞呈席纹或漩涡状排列,FS-DFSP除有经典型DFSP区域外,还可见纤维肉瘤样区域。原发灶弥漫表达CD34,1例转移灶CD34表达缺失,1例p53表达增加,FS-DFSP核分裂象与Ki-67增殖指数均高于经典型DFSP。结论FS-DFSP与经典DFSP相比,侵袭性更强,转移风险更大。肿物局部切除是经典型DFSP进展为FS-DFSP的高危因素,建议尽早扩大切除,减少肿瘤进展风险。

Purpose To investigate the clinical and pathological features of dermatofibrosarcoma protuberans(DFSP)with distant metastasis,and the high-risk factors for metastasis.Methods The clinical data of 4 cases of metastatic DFSP were collected,immunohistochemical staining and FISH detection of COL1A1-PDGFB were carried out along with review of the literature.Results There were 4 cases of metastatic DFSP.The primary lesion of 1 case was typical DFSP,1 case was typical DFSP with 20%mucoid degeneration,and 2 cases were fibrosarcomatous dermatofibrosarcoma protuberans(FS-DFSP).All metastatic lesions progressed to FS-DFSP.Lung metastasis occurred in all patients,and one case was combined with parietal lobe metastasis of the brain.The typical DFSP histologically showed that spindle-shaped cells arranged in storiform or whorled patterns.In addition to the typical DFSP area,fibrosarcomatoid areas were also noted in FS-DFSP.Immunohistochemical staining showed diffusely immunoreactive for CD34 in all the primary lesions;CD34 expression was lost and p53 expression was increased in 1 metastatic lesion.The mitotic activity and Ki-67 proliferation index of FS-DFSP were higher than typical DFSP.Conclusion FS-DFSP has a higher degree of malignancy,and a higher risk of distant metastasis than typical DFSP.Local resection of tumor is a high risk factor for progression of typical DFSP to FS-DFSP.It is recommended to expand the resection as early as possible to reduce the risk of tumor progression.