外阴隆突性皮肤纤维肉瘤一例

Dermatofibrosarcoma Protuberans of Vulva:A Case Report

隆突性皮肤纤维肉瘤(dermatofibrosarcoma protuberans,DFSP)是一种少见的真皮间质肿瘤,而发生于外阴的DFSP在临床上更为罕见,病理学检查结合免疫组织化学染色是明确诊断的主要方法,常以手术治疗为主。本文报告1例外阴DFSP,该患者既往于当地医院行外阴肿物切除术,术后病理检查提示DFSP,遂转往上级医院行补充治疗。经手术切除残余病灶后送病理及免疫组织化学检查,再次明确诊断为外阴DFSP,术后给予补充化疗。通过探讨该病的病因及发病机制、临床表现、病理分型、诊断、鉴别诊断、治疗、预后及随访情况,以加深临床对该病的认识。

Dermatofibrosarcoma protuberans(DFSP)is a rare dermal stromal tumor,and DFSP occurring in the vulva is even rarer in clinical practice.Pathological examination combined with immunohistochemical staining is the main method of diagnosis,and surgery is usually the main treatment.We reported a case of vulvar DFSP.After resection of vulvar mass in a local hospital,the postoperative examination indicated DFSP,so the patient was transferred to our hospital for the supplementary treatment.After surgical resection,the residual lesion was examined for pathology and immunohistochemistry.The diagnosis of vulva DFSP was confirmed again,and the supplementary chemotherapy was given after surgery.By discussing the etiology,pathogenesis,clinical manifestations,pathological types,diagnosis and dfferential diagnosis,treatment,prognosis and follow-up of the disease,we can deepen the understanding of this disease.