目的探讨乳腺隆突性皮肤纤维肉瘤(DFSP)的临床特征、诊断及治疗方法。方法回顾性分析我院近期收治的2例乳腺DFSP患者的临床资料。结果本组2例分别为一27岁男性与一35岁女性患者。男性DFSP患者出现典型的皮肤紫红色改变并突出皮肤表面,一直被诊断为“血管瘤”;乳腺彩超示肿块边界欠清,形态不规则,以低回声为主,周边见环形强回声带,彩色多普勒血流成像见其内短线状血流信号,术前肿块30 mm×11 mm大小。女性DFSP患者则长期被误诊为乳腺纤维腺瘤;乳腺钼靶仅提示双乳乳腺增生,未见确切肿块组织,术前肿块5 mm×10 mm大小。2例患者均采用传统手术治疗模式,肿瘤细胞均高表达CD34,低表达S-100及细胞角蛋白。分别随访8个月与4个月,术后未常规行放疗,至目前未复发。结论乳腺DFSP是起源于乳腺皮肤区域并可浸润皮下组织的局限性低度恶性肿瘤,以无痛性肿块为主要临床表现,术前各种影像学检查诊断特异性欠佳,确诊有赖于肿块切除术后的组织学病理检测及免疫组织化学分析。彻底的手术切除疗效理想,部分切缘阳性或局部复发的患者可能需进一步综合放疗或伊马替尼靶向治疗。 Objective To investigate the clinical features, diagnosis and treatment of breast hyperplasia dermatofibrosarcoma (DFSP). Methods The clinical data of 2 cases of breast DFSP treated in our hospital recently were retrospectively analyzed. Results The two patients in this group were a 27-year-old male and a 35-year-old female patient, respectively. Typical DFSP patients with a typical skin reddish purple change and highlight the surface of the skin has been diagnosed as “hemangioma ”; breast color Doppler showed less clear boundary of the tumor, irregular in shape, with low echo mainly peripheral see the ring echo , Color Doppler flow imaging to see the short-term blood flow within the signal, the size of the preoperative tumor 30 mm × 11 mm. Female DFSP patients were misdiagnosed as long-term breast fibroadenoma; mammary gland mammography only prompted breast hyperplasia, no clear tumor tissue, size of the preoperative tumor 5 mm × 10 mm. Both of the 2 patients were treated by traditional surgery. CD34, S-100 and cytokeratin were all highly expressed in tumor cells. The patients were followed up for 8 months and 4 months respectively. The patients were not routinely treated with radiotherapy and did not relapse till now. Conclusions The mammary gland DFSP is a localized low-grade malignant tumor that originated in the mammary glands and infiltrating the subcutaneous tissue. The painless mass is the main clinical manifestation. The diagnostic accuracy of preoperative imaging is poor. The diagnosis depends on the tumor resection Postoperative histological examination and immunohistochemical analysis. Complete surgical resection of the ideal effect, partial positive margins or local recurrence patients may need further integrated radiotherapy or imatinib targeted therapy.