Bertolli Eduardo, Bretchbuhl Eduard Renè, Camarço William Ricardo, Campagnari Mariane, Molina André Sapata, Baiocchi Glauco, Macedo Mariana Petaccia, Pinto Clovis Antonio, Cunha Isabela Werneck, Neto João Pedreira
World J Surg Oncol. 2014;12(1):399. doi: 10.1186/1477-7819-12-399. Epub 2014 Dec 29.
Dermatofibrosarcoma Protuberans (DFSP) of the vulva is rare and oncologic surgery with free margins may lead to severe functional damage, requiring multidisciplinary approach regarding resection, margin assessment and reconstruction.Case Report: Two cases of DFSP in vulva were treated in a single institution. A 28-year-old patient with an incisional biopsy in the vulvar region revealing DFSP underwent a partial vulvectomy with clitoris preservation. Pathological studies revealed free margins and reconstructive surgery was performed. This patient is disease free in a 40 months follow up. The other, a 57-year-old patient was also referred after an incomplete resection of a DFSP in the vulvar region. A 1-cm margim resection followed by Complete Circumferential and Peripheral Deep Margin Assessment (CCPDMA) was performed. Although the upper lateral margin was positive, it was possible to perform another wide local excision with preservation of the clitoris and primary closure.
DFSP of the vulva requires an accurate evaluation of margins, resections following oncological principles and reconstruction. Although being a very challenging lesion that usually implies difficult surgical management, if treated in a multidisciplinary environment, with surgical oncologists, experienced dermatopathologists and reconstructive surgeons can achieve good results. Even in difficult cases that presents with large lesions and compromising challenging areas, a complete oncologic resection can be performed minimizing functional damage for the patient.
外阴隆突性皮肤纤维肉瘤（DFSP）较为罕见，进行切缘阴性的肿瘤手术可能会导致严重的功能损伤，这就需要在切除、切缘评估和重建方面采取多学科方法。
同一机构治疗了两例外阴DFSP患者。一名28岁患者在外阴区域进行切开活检，结果显示为DFSP，随后接受了保留阴蒂的部分外阴切除术。病理研究显示切缘阴性，并进行了重建手术。该患者在40个月的随访中无疾病复发。另一名57岁患者在对外阴区域的DFSP进行不完全切除后也前来就诊。进行了1厘米切缘切除，随后进行了完全周向和周边深部切缘评估（CCPDMA）。尽管上外侧切缘为阳性，但仍有可能在保留阴蒂的情况下再次进行广泛局部切除并一期缝合。
外阴DFSP需要准确评估切缘，遵循肿瘤学原则进行切除和重建。尽管这是一种极具挑战性的病变，通常意味着手术管理困难，但如果在多学科环境中进行治疗，由外科肿瘤学家、经验丰富的皮肤病理学家和重建外科医生共同协作，能够取得良好的效果。即使是病变较大且累及具有挑战性区域的困难病例，也可以进行完整的肿瘤切除，将对患者的功能损伤降至最低。
