Dermatofibrosarcoma protuberans of the vulva: Case report

Dermatofibrosarcoma protuberans (DFSP) of the vulva is a rare type of locally aggressive cutaneous sarcoma. Approximately 85%-90% are low-grade tumours. Although metastases are rare (<5%), this tumour has a tendency to local recurrence. Five-year survival ranges from 91%-100%. The treatment is multidisciplinary and is based primarily on local resection. Recurrence is potentially directly related to the extent of resection. We report the case of a patient with vulvar localization of DFSP who developed 3 local recurrences over a 5-year period. The patient required surgery and adjuvant therapy with imatinib and external radiotherapy. Currently, she has multiple pulmonary nodules of metastatic lytic lesions in the left L2 vertebral body. (C) 2015 Elsevier Espana, S.L.U. All rights reserved.