title: Response of malignant scalp dermatofibrosarcoma to presurgical targeted growth factor inhibition.
abstract: Dermatofibrosarcoma protuberans (DFSP) is an uncommon, locally aggressive, malignant cutaneous tumor that sparingly presents on the scalp. Dermatofibrosarcomas often result from the formation of a fusion oncogene on translocated or supernumerary ring chromosomes 17 and 22, causing the overexpression of PDGFRbeta driven by the COL1A1 promoter. Because of uncertainty surrounding appropriate treatment of aggressive scalp DFSP, the authors performed an extensive review of the available data from a MEDLINE (Ovid) search to describe the clinical presentation and treatment options for this rare tumor. Their search identified 39 different cases, including the illustrative case presented in this study. Adjuvant therapy for this malignant lesion is not universally established in the literature. In the present case, the authors successfully treated a locally invasive scalp DFSP with presurgical therapy that specifically inhibited the PDGFbeta receptor. Imatinib significantly shrank the DFSP tumor mass, reduced hypervascularity, reduced metabolic activity on PET scanning, and permitted a safe gross-total resection. Although wide excision and Mohs micrographic surgery remain the standard surgical treatments for DFSP, the authors illustrate that presurgical chemotherapeutic treatment by imatinib provides a critical adjunct to traditional therapy.
authors.affiliation: Preston Robert Tisch Brain Tumor Center, Duke Medical Center, Durham, North Carolina 27710, USA.
authors.forename: Austin K
authors.initials: AK
authors.lastname: Mattox
authors.forename: Ankit I
authors.initials: AI
authors.lastname: Mehta
authors.forename: Peter M
authors.initials: PM
authors.lastname: Grossi
authors.forename: Thomas J
authors.initials: TJ
authors.lastname: Cummings
authors.forename: D Cory
authors.initials: DC
authors.lastname: Adamson
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use*; D001549:Benzamides; D002886:Chromosomes, Human, Pair 17 / Q000235:genetics; D002892:Chromosomes, Human, Pair 22 / Q000235:genetics; D024042:Collagen Type I / Q000235:genetics; D000090602:Collagen Type I, alpha 1 Chain; D018223:Dermatofibrosarcoma / Q000188:drug therapy / Q000235:genetics / Q000601:surgery; D004815:Epidermal Growth Factor / Q000037:antagonists & inhibitors*; D006801:Humans; D000068877:Imatinib Mesylate; D008297:Male; D008875:Middle Aged; D019635:Neurosurgical Procedures / Q000379:methods; D010879:Piperazines / Q000627:therapeutic use*; D011743:Pyrimidines / Q000627:therapeutic use*; D020797:Receptor, Platelet-Derived Growth Factor beta / Q000235:genetics; D012535:Scalp; D012878:Skin Neoplasms / Q000188:drug therapy / Q000235:genetics / Q000601:surgery; 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; D004815:Epidermal Growth Factor; D000068877:Imatinib Mesylate; D020797:Receptor, Platelet-Derived Growth Factor beta