title: Recurrent craniofacial dermatofibrosarcoma protuberans: long-term prognosis after close surgical removal.
abstract: Dermatofibrosarcoma protuberans (DFSP) is a low-grade malignant neoplasm of the dermis that rarely manifests in the craniofacial area. In this retrospective analysis, we investigated the long-term survival of 7 patients with recurrent craniofacial DFSP. This study includes all patients in our department with recurrences of DFSP between 1989 and 2006. All patients were treated by radical surgery with 1-cm free safety margin in every direction and remained in routine long-term follow-up for tumor patients. Two of the 7 patients showed a local recurrence, which was again successfully treated surgically with the same technique. Advanced reconstruction with free full-thickness skin transfers, regional flaps, and forearm flaps, respectively, was required in 5 of the 7 patients. The other 2 patients were reconstructed locally. The long-term prognosis of craniofacial DFSP can be assessed optimistically even if the tumor already reoccurred. All 7 patients included in this study are still alive and so far not suffering from local recurrence. Advanced reconstructive techniques are often required in the management of reoccurring craniofacial DFSP. Late recurrences have been reported; therefore, a long-term follow-up for these patients should be considered.
authors.affiliation: Department of Oral and Maxillofacial Surgery, University Hospital Heidelberg, Germany. oliver.thiele@med.uni-heidelberg.de
authors.forename: Oliver C
authors.initials: OC
authors.lastname: Thiele
authors.forename: Robin
authors.initials: R
authors.lastname: Seeberger
authors.forename: Claire
authors.initials: C
authors.lastname: Bacon
authors.forename: Joachim
authors.initials: J
authors.lastname: Mühling
authors.forename: Kolja
authors.initials: K
authors.lastname: Freier
authors.forename: Christof
authors.initials: C
authors.lastname: Hofele
mesh_terms: D000328:Adult; D018223:Dermatofibrosarcoma / Q000601:surgery*; D005153:Facial Neoplasms / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D005546:Forehead / Q000601:surgery; D006258:Head and Neck Neoplasms / Q000601:surgery*; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D015580:Mohs Surgery; D009364:Neoplasm Recurrence, Local / Q000601:surgery*; D009669:Nose Neoplasms / Q000601:surgery; D009777:Occipital Bone / Q000601:surgery; D009918:Orbital Neoplasms / Q000601:surgery; D011379:Prognosis; D019651:Plastic Surgery Procedures; D012086:Reoperation; D012189:Retrospective Studies; D012888:Skull Neoplasms / Q000601:surgery; D015996:Survival Rate; D016896:Treatment Outcome