title: Current treatment options for dermatofibrosarcoma protuberans.
abstract: Dermatofibrosarcoma protuberans (DFSP) is rare, infiltrating dermal neoplasm, characterized by indolent growth and low probability of metastases. The critical event in DFSP development is the rearrangement of chromosome 17 and 22, leading to transcriptional up-regulation of platelet-derived growth factor, providing an autocrine and/or paracrine stimulus. The cornerstone of treatment for localized DFSP is complete surgical resection with microscopically negative margins. Adjuvant radiotherapy is suggested in cases of positive margins when re-excision is not feasible. The first effective systemic therapy in DFSP introduced into clinical practice was imatinib, demonstrating dramatic activity in advanced cases. Current results indicate that some DFSP patient initially evaluated as unresectable/metastatic or necessitating mutilating surgery turned resectable after imatinib therapy and this rational approach leading to complete remission maybe potentially curative. The clinical experience with other tyrosine kinase inhibitors is limited and imatinib remains the gold standard treatment of locally unresectable/metastatic DFSP. This review summarizes state of the art and perspectives on the DFSP management.
authors.affiliation: Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie Memorial Cancer Center and Institute of Oncology, Roentgena 5, 02-781 Warsaw, Poland.
authors.forename: Piotr
authors.initials: P
authors.lastname: Rutkowski
authors.forename: Maria
authors.initials: M
authors.lastname: Debiec-Rychter
mesh_terms: D000970:Antineoplastic Agents / Q000494:pharmacology / Q000627:therapeutic use*; D003131:Combined Modality Therapy; D018223:Dermatofibrosarcoma / Q000235:genetics / Q000473:pathology / Q000628:therapy*; D006801:Humans; D009362:Neoplasm Metastasis; D010982:Platelet-Derived Growth Factor / Q000235:genetics; D047428:Protein Kinase Inhibitors / Q000494:pharmacology / Q000627:therapeutic use; D018714:Radiotherapy, Adjuvant / Q000379:methods; D012878:Skin Neoplasms / Q000235:genetics / Q000473:pathology / Q000628:therapy*; D015854:Up-Regulation
chemical_list: D000970:Antineoplastic Agents; D010982:Platelet-Derived Growth Factor; D047428:Protein Kinase Inhibitors
keywords: dermatofibrosarcoma protuberans; imatinib; molecular targeted therapy; neoadjuvant therapy; tyrosine kinase inhibitor