AIMS: @entity241 is a rare condition which is frequently misdiagnosed at presentation, resulting in a high incidence of local recurrence due to inadequate resection. The archives of the Department of Orthopaedics at the University of Florida were analysed to investigate the natural history and results of treatment for this @entity5 . METHODS: Between 1975 and 1996, 35 cases of @entity241 were treated at the University of Florida. Of these, one was treated primarily, five were treated for local recurrence, 17 had @entity5 bed excisions following inadequate primary excisions elsewhere and 12 had @entity5 bed excisions following inadequate resection of local recurrences elsewhere. The data were analysed to assess the impact of age, gender, duration of symptoms, @entity5 site and size, surgical margin, number of operations and adjuvant treatments on survival and local recurrence outcomes. RESULTS: Complete follow-up was available for 34 @entity1 . Mean follow-up was 58 months (range 12-144 months). Thirty-three @entity1 remain alive and disease-free. One @entity1 died of unrelated causes. The margins obtained were wide in 28 @entity1 , marginal in six and intralesional in one. Of the seven @entity1 with inadequate surgical margins, four received adjuvant radiation therapy and remain disease-free. No @entity1 with an adequate margin developed a local recurrence, but there were three local recurrences in the @entity1 with an inadequate margin who did not receive adjuvant radiation therapy (local recurrence rate: 8%). No @entity1 developed lymphatic or distant @entity3 . Local recurrences were more likely to be classified Stage IB (17/17) than primary @entity5 (1/18) (P<0.001). Local recurrence was more likely where the surgical margin was less than 2.5 cm from the lesion. CONCLUSIONS: @entity241 is a low-grade @entity5 that has a high potential for local recurrence unless it can be completely excised. The overall rate of local recurrence in referred @entity1 in this series was 20/35 cases (57%). All occurred after inadequate margins at previous surgery in other institutions. Revision surgery in these @entity1 showed a local recurrence rate of 8%. To avoid extensive surgery for recurrences, initial treatment should be by wide excision incorporating the underlying deep fascia and a cuff of 2.5-3 cm of normal skin tissue. Radiation therapy provides a useful adjunct where adequate margins cannot be obtained.

Revision surgery in XXXX of the trunk and extremities.

@entity5 :: ('MESH:D009369', 'Disease') :: ['tumours', 'tumour'] @entity1 :: ('9606', 'Species') :: ['patients', 'patient'] @entity241 :: ('MESH:D018223', 'Disease') :: ['Dermatofibrosarcoma protuberans', 'DFSP'] @entity3 :: ('MESH:D009362', 'Disease') :: ['metastasis']
@entity241 :: (MESH:D018223,Disease) :: ['dermatofibrosarcoma protuberans']
