Love W Elliot, Keiler Susan A, Tamburro Joan E, Honda Kord, Gosain Arun K, Bordeaux Jeremy S
Department of Dermatology, University Hospitals Case Medical Center, Cleveland, Ohio, USA.
J Am Acad Dermatol. 2009 Dec;61(6):1014-23. doi: 10.1016/j.jaad.2009.05.028.
Congenital dermatofibrosarcoma protuberans (DFSP) is a rare tumor with varying clinical presentations that is commonly misdiagnosed. Treatment of congenital DFSP is complicated by delays in diagnosis and its propensity for subclinical spread. Of 61 reported cases, 11 (18%) were treated with Mohs micrographic surgery (MMS) and 46 (75%) were treated with wide local excision (WLE). One case was treated with imatinib, and the remaining 3 did not differentiate between receiving MMS or WLE. In the cases of congenital DFSP treated with MMS the clearance rate was 100% with an average follow-up of 4.3 years. The clearance rate seen with WLE was 89% with an average follow-up period of 1.9 years. The average margins taken during MMS (1.7 cm) were smaller than those taken with WLE (2.8 cm). Fifty percent of cases with available follow-up undergoing WLE required multiple surgeries. Based on superior cure rates with long-term follow-up, smaller surgical margins, and fewer surgical sessions, MMS should be considered as first-line treatment for congenital DFSP.
先天性隆突性皮肤纤维肉瘤（DFSP）是一种临床表现各异的罕见肿瘤，常被误诊。先天性DFSP的治疗因诊断延迟及其亚临床扩散倾向而变得复杂。在61例报告病例中，11例（18%）接受了莫氏显微外科手术（MMS）治疗，46例（75%）接受了广泛局部切除（WLE）治疗。1例接受了伊马替尼治疗，其余3例未区分接受MMS还是WLE治疗。在接受MMS治疗的先天性DFSP病例中，清除率为100%，平均随访4.3年。WLE的清除率为89%，平均随访期为1.9年。MMS期间切除的平均切缘（1.7 cm）小于WLE（2.8 cm）。接受WLE治疗且有随访资料的病例中有50%需要多次手术。基于长期随访的更高治愈率、更小的手术切缘和更少的手术次数，MMS应被视为先天性DFSP的一线治疗方法。
