title: [Dermatofibrosarcoma protuberans].
abstract: Dermatofibrosarcoma protuberans (DFSP) is a soft tissue neoplasm of intermediate malignancy that is initially localized to the skin from where it can invade deep structures (fat, fascia, muscle and bone). It is the most frequent fibrohistiocytic tumor, comprising approximately 1.8 % of all soft tissue sarcomas and 0.1 % of all cancers. It has an estimated incidence of 0.8-5 cases per one million persons per year. Treatment of localized disease consists in complete surgical excision of the lesion by conventional surgery with wide margins (>3 cm) or by micrographic Mohs surgery. Although the cases of metastatic DFSP do not reach 5 % of the total, almost all of them appear after previous local relapses. The prognosis for metastatic cases is very poor with a survival of less than 2 years following detection of metastatic disease. Patients with locally advanced DFSP are not candidates for an initial radical surgical therapy therefore neoadyuvant treatment is required prior to surgery in order to reduce tumor burden. In this regard, chemotherapy and radiotherapy have not been highly efficacious so it is necessary to consider new alternatives. The demonstration of the oncogenic power of the translocation COL1A1-PDGFB in DFSP has allowed the successful introduction of drug therapy with antagonists of the PDGFB receptor for metastatic or locally advanced cases.
authors.affiliation: Servicio de Dermatología, Instituto Valenciano de Oncología, Valencia, España. osanmartinj@gmail.com
authors.forename: O
authors.initials: O
authors.lastname: Sanmartín
authors.forename: B
authors.initials: B
authors.lastname: Llombart
authors.forename: J A
authors.initials: JA
authors.lastname: López-Guerrero
authors.forename: C
authors.initials: C
authors.lastname: Serra
authors.forename: C
authors.initials: C
authors.lastname: Requena
authors.forename: C
authors.initials: C
authors.lastname: Guillén
mesh_terms: D018952:Antigens, CD34 / Q000032:analysis; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001549:Benzamides; D014408:Biomarkers, Tumor / Q000032:analysis; D017024:Chemotherapy, Adjuvant; D002886:Chromosomes, Human, Pair 17 / Q000648:ultrastructure; D002892:Chromosomes, Human, Pair 22 / Q000235:genetics / Q000648:ultrastructure; D003131:Combined Modality Therapy; D018223:Dermatofibrosarcoma / Q000737:chemistry / Q000145:classification / Q000188:drug therapy / Q000235:genetics / Q000473:pathology / Q000601:surgery; D015195:Drug Design; D006801:Humans; D000068877:Imatinib Mesylate; D015580:Mohs Surgery; D020360:Neoadjuvant Therapy; D009361:Neoplasm Invasiveness; D009363:Neoplasm Proteins / Q000032:analysis / Q000235:genetics; D009364:Neoplasm Recurrence, Local; D015514:Oncogene Proteins, Fusion / Q000032:analysis / Q000235:genetics; D010879:Piperazines / Q000627:therapeutic use; D011379:Prognosis; D011743:Pyrimidines / Q000627:therapeutic use; D020797:Receptor, Platelet-Derived Growth Factor beta / Q000037:antagonists & inhibitors; D012303:Ring Chromosomes; D012509:Sarcoma / Q000737:chemistry / Q000188:drug therapy / Q000235:genetics / Q000473:pathology / Q000601:surgery; D012878:Skin Neoplasms / Q000737:chemistry / Q000188:drug therapy / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D014178:Translocation, Genetic
chemical_list: D018952:Antigens, CD34; D000970:Antineoplastic Agents; D001549:Benzamides; D014408:Biomarkers, Tumor; C120892:COLIA1-PDGFB fusion protein, human; D009363:Neoplasm Proteins; D015514:Oncogene Proteins, Fusion; D010879:Piperazines; D011743:Pyrimidines; D000068877:Imatinib Mesylate; D020797:Receptor, Platelet-Derived Growth Factor beta