title: [Molecular approach in the treatment of dermatofibrosarcoma protuberans].
abstract: Dermatofibrosarcoma protuberans (DFSP) is a cutaneous low-grade malignancy with a high recurrence rate that rarely generates distant metastases. In most cases this tumor is associated with a chromosomal translocation involving the COL1A1 gene on chromosome 17 and the platelet-derived-growth-factor B gene on chromosome 22, generating a fusion gene that constitutively activates the PDGF receptor (PDG-FR). In the early stages of disease traditional surgery (wide excision) or Mohs micrographic surgery represent the standard of care. When surgical margins are positive, postoperative radiotherapy is a valuable option. Recently it has been shown that inhibiting PDGFR with imatinib can induce a high response rates in case of unresectable or metastatic disease. This targeted agent now represents the therapy of choice of advanced DFSP and it is the fi rst great therapeutic success in this disease after unsuccessful years using cytotoxic drugs. It is likely that a better knowledge of molecular biology of DFSP could, as it was the case for GISTs, may improve treatment results leading to the development of new targeted agents.
authors.affiliation: Istituto Nazionale Tumori Regina Elena Roma, Italia.
authors.forename: S I S
authors.initials: SI
authors.lastname: Fattoruso
authors.forename: P
authors.initials: P
authors.lastname: Visca
authors.forename: M
authors.initials: M
authors.lastname: Lopez
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use; D001549:Benzamides; D002886:Chromosomes, Human, Pair 17; D002892:Chromosomes, Human, Pair 22; D024042:Collagen Type I / Q000235:genetics; D000090602:Collagen Type I, alpha 1 Chain; D018223:Dermatofibrosarcoma / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy*; D019317:Evidence-Based Medicine; D006801:Humans; D000068877:Imatinib Mesylate; D015580:Mohs Surgery / Q000379:methods; D010879:Piperazines / Q000627:therapeutic use; D011379:Prognosis; D011743:Pyrimidines / Q000627:therapeutic use; D018714:Radiotherapy, Adjuvant; D017479:Receptors, Platelet-Derived Growth Factor / Q000235:genetics; D012307:Risk Factors; D017678:Sex Distribution; D012878:Skin Neoplasms / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy*; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents; D001549:Benzamides; D024042:Collagen Type I; D000090602:Collagen Type I, alpha 1 Chain; D010879:Piperazines; D011743:Pyrimidines; D000068877:Imatinib Mesylate; D017479:Receptors, Platelet-Derived Growth Factor