title: Dermatofibrosarcoma protuberans treated at a single institution: a surgical disease with a high cure rate.
abstract: OBJECTIVE
  Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade, cutaneous sarcoma with autocrine overproduction of the platelet-derived growth factor (PDGF) beta-chain from gene rearrangement as a key pathogenetic factor, now susceptible of molecular-targeted therapy. The aim of this retrospective analysis was to explore the outcome of patients with primary or recurrent DFSP.
  
  
  METHODS
  Two hundred eighteen patients surgically treated at the Istituto Nazionale per lo studio e la cura dei Tumori (Milan, Italy) over 20 years were reviewed. Local relapse, distant metastasis, and survival were studied.
  
  
  RESULTS
  One hundred thirty-six patients (62.4%) presented with a primary DFSP, while 82 patients (37.6%) had a recurrent disease. In the primary group, margins were microscopically positive in 11.8%, while in the recurrent group they were positive in 14.6% (P =.613). In the primary group, patients undergoing re-excision after inadequate previous surgery had residual disease in 62% of cases. Reconstructive surgery was needed in 30%, significantly more frequently in patients with a recurrence or a head and neck tumor. The crude cumulative incidence of local relapses was 4% at 10 years, and 2% at 10 years for distant metastases. No significant difference was found between primary and recurrent patients, as well as between positive and negative margins.
  
  
  CONCLUSIONS
  This being one of the largest mono-institutional series of DFSP, we confirm that long-term outcome is excellent, in terms of both local and distant control, after a wide excision with negative margins. Reconstructive surgery is often needed. Novel medical therapies will be of use in a limited subgroup of patients.
authors.affiliation: Department of Surgery, Istituto Nazionale per lo studio e la cura dei Tumori, via Venezian 1, 20133 Milano, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Fiore
authors.forename: Rosalba
authors.initials: R
authors.lastname: Miceli
authors.forename: Chiara
authors.initials: C
authors.lastname: Mussi
authors.forename: Salvatore
authors.initials: S
authors.lastname: Lo Vullo
authors.forename: Luigi
authors.initials: L
authors.lastname: Mariani
authors.forename: Laura
authors.initials: L
authors.lastname: Lozza
authors.forename: Paola
authors.initials: P
authors.lastname: Collini
authors.forename: Patrizia
authors.initials: P
authors.lastname: Olmi
authors.forename: Paolo G
authors.initials: PG
authors.lastname: Casali
authors.forename: Alessandro
authors.initials: A
authors.lastname: Gronchi
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D015331:Cohort Studies; D018223:Dermatofibrosarcoma / Q000473:pathology / Q000601:surgery*; D005121:Extremities / Q000473:pathology; D005260:Female; D005500:Follow-Up Studies; D006258:Head and Neck Neoplasms / Q000556:secondary* / Q000601:surgery*; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000473:pathology / Q000601:surgery*; D009367:Neoplasm Staging; D012189:Retrospective Studies; D012307:Risk Factors; D012878:Skin Neoplasms / Q000556:secondary* / Q000601:surgery*