Dermatofibrosarcoma protuberans: treatment results of 35 cases

Background and purpose: This study evaluates the treatment results of patie nts with dermatofibrosarcoma protuberans. Patients and methods: Between August 1987 and July 1998, 35 consecutive pat ients with pathologically proved dermatofibrosarcoma protuberans received s urgery with or without radiation therapy. Their treatment results were anal yzed retrospectively. Results: The patient ages ranged from 5 to 67 years (median 37 years). Ther e were 24 males and 11 females. The anatomic sites of tumor were: trunk in 21, extremity in eight, and head and neck region in six. The maximal dimens ion of tumor ranged from 1.5 to 25 cm. Surgery techniques included local ex cision and wide excision with or without graft or Rap. Adjuvant radiation t herapy was given to 11 patients, with a dose ranging from 46 to 68 Gy (one pre-operative, ten post-operative). At a median follow-up of 50 months (ran ge 11-131 months), there were 11 patients (nine patients without radiation therapy) who developed local failure. Salvage therapy (excision with or wit hout radiation therapy) was given to all of them, and ten achieved disease control. Some patients had treatment-related moderate cosmetic or functiona l problems. Conclusions: Dermatofibrosarcoma protuberans is a malignancy of a high cure rate, and adjuvant radiation therapy can reliably decrease the local recur rence rate and prevent mutilation and functional deficit caused by repeated surgery. (C) 2000 Elsevier Science Ireland Ltd. All rights reserved.