Loss Lesley, Zeitouni Nathalie C
Department of Dermatology, Roswell Park Cancer Institute, Buffalo, New York 14263, USA.
Dermatol Surg. 2005 Nov;31(11 Pt 1):1428-33. doi: 10.2310/6350.2005.31209.
Dermatofibrosarcoma protuberans is a relatively rare, locally aggressive soft tissue tumor. Less than 5% of these tumors are located on the scalp; however, the recurrence rates for this region are high compared with those of more common locations, such as the trunk and proximal extremities. Standards for the management of dermatofibrosarcoma protuberans in general have been established in the literature, including wide local excision and Mohs micrographic surgery.
To describe optimum treatment options for primary and recurrent dermatofibrosarcoma protuberans of the scalp based on cases from Roswell Park Cancer Institute (RPCI) and from the literature.
Five cases from RPCI and 23 cases from the literature were reviewed. Age, sex, history, tumor size and level of invasion, treatment, reconstruction method, and follow-up were compiled from each case.
Twenty-eight cases of scalp dermatofibrosarcoma protuberans were found; 13 were primary, 8 were recurrent, and 3 had persistent positive margins. Fifty-eight percent had classic histologic findings; seven tumors invaded beyond the periosteum. Twenty tumors were treated by surgical excision, whereas eight tumors were treated with Mohs micrographic surgery, requiring between two and four layers. Advanced surgical reconstruction was used in most cases to close the defects. Four patients experienced one tumor recurrence, whereas three other patients had several local recurrences. One patient died of his disease, and one died during surgical treatment of dermatofibrosarcoma protuberans.
Mohs micrographic surgery or a modified version may be the best treatment option for scalp dermatofibrosarcoma protuberans to ensure complete removal of tumor and minimize recurrence. Surgeons should be prepared for advanced reconstruction following tumor removal.
隆突性皮肤纤维肉瘤是一种相对罕见的、具有局部侵袭性的软组织肿瘤。这些肿瘤中不到5%位于头皮；然而，与躯干和近端肢体等更常见部位相比，该区域的复发率较高。文献中已确立了隆突性皮肤纤维肉瘤的一般治疗标准，包括广泛局部切除和莫氏显微外科手术。
基于罗斯威尔帕克癌症研究所（RPCI）的病例和文献，描述头皮原发性和复发性隆突性皮肤纤维肉瘤的最佳治疗方案。
回顾了RPCI的5例病例和文献中的23例病例。收集了每个病例的年龄、性别、病史、肿瘤大小和侵袭程度、治疗方法、重建方法及随访情况。
共发现28例头皮隆突性皮肤纤维肉瘤；13例为原发性，8例为复发性，3例切缘持续阳性。58%具有典型组织学表现；7个肿瘤侵犯至骨膜外。20个肿瘤采用手术切除治疗，8个肿瘤采用莫氏显微外科手术治疗，需要切2至4层。大多数病例采用了先进的手术重建来闭合缺损。4例患者出现1次肿瘤复发，另外3例患者有多次局部复发。1例患者死于该病，1例在隆突性皮肤纤维肉瘤手术治疗期间死亡。
莫氏显微外科手术或其改良版本可能是头皮隆突性皮肤纤维肉瘤的最佳治疗选择，以确保肿瘤完全切除并使复发风险降至最低。外科医生应在肿瘤切除后做好进行先进重建的准备。
