Zemni Ines, Sassi Imène, Boujelbene Nedia, Haddad Sabrine, Doghri Raoudha, Chargui Riadh, Rahal Khaled
Department of Surgical Oncology, Salah Azaiez Institute, University of Tunis El Manar, Tunis, Tunisia.
Department of Pathology, Salah Azaiez Institute, University of Tunis El Manar, Tunis, Tunisia.
Pan Afr Med J. 2019 May 22;33:46. doi: 10.11604/pamj.2019.33.46.18861. eCollection 2019.
Dermatofibrosarcoma protuberans (DFSP) is a low-to-intermediate grade sarcoma of dermal origin that rarely presents in the vulva, typically occurring on the trunk of young to middle-aged adults. Even though it has a low potential for distant metastases, it often recurs locally. Therefore, surgical excision is the treatment of choice. We report a case of DFSP with fibrosarcoma transformation involving the vulva treated by large excision with tumor free margins followed by plastic reconstruction. Our aim is to highlight this rare disease and through literature evaluate the safety and efficacy of surgical treatment. Early recognition of this rare entity whose localization and the aggressive nature of the fibrosarcomatous component will be an issue in the surgical management.
隆突性皮肤纤维肉瘤（DFSP）是一种起源于真皮的低至中级别肉瘤，很少出现在外阴部，通常发生在中青年成年人的躯干上。尽管其远处转移的可能性较低，但它经常在局部复发。因此，手术切除是首选的治疗方法。我们报告了一例发生在外阴的伴有纤维肉瘤转化的DFSP病例，该病例通过切除肿瘤周围无瘤边缘的大块组织，随后进行整形重建来治疗。我们的目的是强调这种罕见疾病，并通过文献评估手术治疗的安全性和有效性。早期识别这种罕见实体，其定位以及纤维肉瘤成分的侵袭性在手术管理中将会是一个问题。
