Li Yanan, Wang Chuan, Xiang Bo, Chen Siyuan, Li Li, Ji Yi
Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu, 610041, China.
Pediatric Intensive Care Unit, Department of Critical Care Medicine, West China Hospital of Sichuan University, Chengdu, 610041, China.
J Cancer. 2017 May 12;8(7):1319-1323. doi: 10.7150/jca.17988. eCollection 2017.
Dermatofibrosarcoma protuberans (DESP) is a relatively rare and low-grade tumor of the skin and soft tissue. We review the clinical features, pathological findings and management of recurrent DESP with the aim to improve our understanding of this rare tumor. Fifty-seven patients were diagnosed with recurrent DFSP. The mean age at the presentation of DFSP was 30.9 years. The mean age at diagnosis of DFSP was 40.2 years. Chest wall was the dominant location. The histopathological diagnosis was ordinary DFSP (O-DFSP) in 46 cases and fibrosarcomatous DFSP (FS-DFSP) in 11 cases. No differences were noted between FS-DFSP and conventional DFSP in age at presentation and diagnosis, tumor size, as well as CD34 and SMA expression ( > 0.05). However, the time course to recurrence in FS-DFSP group was less than that in O-DFSP group ( = 0.038). All of the patients were treated with Mohs micrographic surgery (MMS). On follow-up, none of the patients had tumor recurrence. Our data demonstrated that the clinical features of recurrent DFSP are non-specific and variable. Our current practice suggests that MMS is very useful treatment choice for recurrent DFSP.
隆突性皮肤纤维肉瘤（DESP）是一种相对罕见的皮肤和软组织低度肿瘤。我们回顾复发性DESP的临床特征、病理表现及治疗方法，旨在提高对这种罕见肿瘤的认识。57例患者被诊断为复发性隆突性皮肤纤维肉瘤（DFSP）。DFSP初诊时的平均年龄为30.9岁。DFSP确诊时的平均年龄为40.2岁。胸壁是主要发病部位。组织病理学诊断为普通型DFSP（O-DFSP）46例，纤维肉瘤型DFSP（FS-DFSP）11例。FS-DFSP与传统DFSP在初诊和确诊年龄、肿瘤大小以及CD34和SMA表达方面无差异（P>0.05）。然而，FS-DFSP组的复发时间短于O-DFSP组（P=0.038）。所有患者均接受了莫氏显微外科手术（MMS）治疗。随访期间，所有患者均无肿瘤复发。我们的数据表明，复发性DFSP的临床特征不具有特异性且具有多样性。我们目前的实践表明，MMS是复发性DFSP非常有用的治疗选择。
