隆突性皮肤纤维肉瘤诊断与治疗

Diagnosis and treatment with the Dermatofibrosarcoma Protuberans

目的：探讨隆突性皮肤纤维肉瘤（DFSP）的诊断和治疗方法。方法：对28例经病理证实不同部位隆突性皮肤纤维肉瘤患者的诊治情况进行回顾性分析。躯干部16例（57％），四肢部8例（28．5％），头颈部4例（14．2）。5例行局部切除术，23例扩大切除术＋植皮或皮瓣转移修复术。16例在术后行放射治疗，平均照射剂量60Gy（50～70Gy）。结果：切除28例标本，CD34阳性26例，阴性2例。术后随访1～5年（平均2．3年），失访2例。复发9例，平均复发时间2．6年（8个月～5年）。28例均未见有远处转移，无死亡。结论：隆突性皮肤纤维肉瘤是一种低度恶性肿瘤，行扩大切除术＋辅助放疗是治疗隆突性皮肤纤维肉瘤的主要治疗方法，手术范围大小及术后辅助放疗与否是影响隆突性皮肤纤维肉瘤的独立预后因素，可有效提高患者生存率及生存质量。

Objective To discuss the diagnosis and treatment of Dermatofibrosarcoma Protuberans （DFSP）. Methods The retrospective analysis of 28 patients with DFSP confirmed pathologically were treated. The tumor of 16 patients located at trunk, 8 at the limbs and 4 at the head and neck.Five patients underwent regional resection and other 23 patients underwent expanded resection.Sixteen patients underwent radiotherapy after operation and the average dosage was 60 Gy （50N70Gy）. Results Twenty-eight specimens were resected.The expression of CD34 of 26 specimens was positive and 2 negative. Twenty-six patients were followed up for 1-5 years （average 2.3 years ）. Tumor recurred in 9 cases. The mean recurrence time was 2.6 years （from 8 months to 5 years）. There were neither distant metastasis nor death in 28cases. Conclusion Dermatofibrosarcoma Protuberans is a malignant tumor with low malignancy.Extensive lumpectomy and postoperation radiotherapy are major treatment methods Of DFSP.Extened excision and postoperation radiotherapy are the independent prognostic factors of DFSP.It is effective treatment in improving survival rate and quality of Dermatofibrosarcoma Protuberans.