隆突性皮肤纤维肉瘤的治疗进展

Treatment of dermatofibrosarcoma protuberans

隆突性皮肤纤维肉瘤是一种罕见的交界恶性，潜在低度恶性肿瘤，局部复发率高，远处转移率低。隆突性皮肤纤维肉瘤好发年龄20～50岁，好发部位为躯干，典型临床表现为在萎缩性斑块基础上出现直径大小不一的多发硬性结节，组织学特征由单一编席样或车辐状排列的梭形细胞组成，呈浸润性生长。隆突性皮肤纤维肉瘤的标准治疗方式是局部扩大切除，外科切缘通常要求为2～3cm。Mohs手术以及改良Mohs手术有良好的治疗前景，术后可适当辅以放射治疗。近年来，隆突性皮肤纤维肉瘤发病机制得到进一步阐明，90％的隆突性皮肤纤维肉瘤患者发生染色体17和22重排，靶向药物方面已批准伊马替尼可用于治疗不能切除和（或）转移和（或）复发性隆突性皮肤纤维肉瘤患者。

Dermatofibrosarcoma protuberans （DFSP） is a rare borderline malignant or potentially low- grade malignant neoplasm with a high local recurrence rate and a low risk of metastasis. DFSP usually occurs on the trunk in people aged 20 - 50 years, and is characterized by multiple indurated nodules varying in size and arising on atrophic plaques. Histologically, the tumor consists of uniform spindle cells which grow infiltratively and are arranged in a storiform or cartwheel pattern. The standard therapy of DFSP is wide local excision with a margin of 2 - 3 cm. Mobs＇ surgery and modified Mohs＇ surgery have shown a good prospect in the treatment of DFSP. Radiation therapy can serve as an adjuvant treatment after surgery. In recent years, the pathogenesis of DFSP has been further clarified and studies have shown that chromosomes 17 and 22 are rearranged in 90% of patients with DFSP. As a targeted drug, imatinib has been approved for the treatment of DFSP in patients with unresectable, metastatic and/or recurrent DFSP.