隆突性皮肤纤维肉瘤50例临床病理分析

Clinical and pathological analysis of 50 patients with dermatofibrosarcoma protuberans

目的：探讨隆突性皮肤纤维肉瘤（DFSP）的诊断及鉴别诊断要点。方法回顾性分析中国医学科学院皮肤病研究所1998—2014年诊治的50例 DFSP 患者的临床表现、组织病理学、免疫组化特点及治疗和预后。结果50例患者发病年龄（29.5±15.9）岁，平均病程9.6年。皮损位于躯干部33例，占66.0％；其次为四肢和头颈部。典型皮损表现为，萎缩性斑片/斑块13例，占26.0％；在此基础上多发性大小不一结节30例，占60.0％；正常皮肤上单发或多发结节7例，占14.0％。组织病理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，肿瘤细胞表达 CD34和波形蛋白。20例患者皮损切除后原位复发，复发率43.5％，未见远处转移和死亡患者。结论 DFSP 皮损形态变化多样，容易误诊，组织病理及免疫组化检查可确诊。DFSP 切除后局部复发率高，可多次复发，但罕见淋巴结和远处转移。

Objective To investigate the diagnosis and differential diagnosis of dermatofibrosarcoma protuberans （DFSP）. Methods Totally, 50 patients with DFSP visiting the Institute of Dermatology, Chinese Academy of Medical Sciences from 1998 to 2014 were enrolled. The clinical manifestations, histopathological and immunohistochemical features, treatment and prognosis of DFSP were retrospectively reviewed. Results The average age at onset of DFSP was （29.5 ± 15.9）years in the 50 patients, with a mean disease duration of 9.57 years. Skin lesions most frequently occurred on the trunk（n = 33, 66.0%）, followed by the extremities, head and neck. DFSP was characterized by atrophic patches or plaques in 13 cases （26.0%）, multiple nodules varying in size and arising on atrophic plaques or patches in 30 cases （60.0%）, single or multiple nodules arising on normal skin in 7 cases （14.0%）. Histologically, the tumor consisted of uniform infiltrative spindle cells arranged in a storiform or cartwheel pattern. In addition, the tumor cells expressed CD34 and vimentin. Twenty patients experienced recurrence at the primary site after resection of skin lesions with a recurrence rate of 43.5%. No distant metastasis or death occurred in these patients. Conclusions DFSP usually has various skin manifestations, is easily misdiagnosed, and can be confirmed based on histopathological and immunohistochemical findings. Local recurrence of DFSP is common, and may occur for many times after surgical excision, but lymphatic and distant metastases are rare.