Document Detail


Response of malignant scalp dermatofibrosarcoma to presurgical targeted growth factor inhibition.
MedLine Citation:
PMID:  19681684     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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:
Austin K Mattox; Ankit I Mehta; Peter M Grossi; Thomas J Cummings; D Cory Adamson
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of neurosurgery     Volume:  112     ISSN:  1933-0693     ISO Abbreviation:  J. Neurosurg.     Publication Date:  2010 May 
Date Detail:
Created Date:  2010-05-03     Completed Date:  2010-05-21     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0253357     Medline TA:  J Neurosurg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  965-77     Citation Subset:  AIM; IM    
Affiliation:
Preston Robert Tisch Brain Tumor Center, Duke Medical Center, Durham, North Carolina 27710, USA.
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MeSH Terms
Descriptor/Qualifier:
Antineoplastic Agents / therapeutic use*
Chromosomes, Human, Pair 17 / genetics
Chromosomes, Human, Pair 22 / genetics
Collagen Type I / genetics
Dermatofibrosarcoma* / drug therapy,  genetics,  surgery
Epidermal Growth Factor / antagonists & inhibitors*
Humans
Male
Middle Aged
Neurosurgical Procedures / methods
Piperazines / therapeutic use*
Pyrimidines / therapeutic use*
Receptor, Platelet-Derived Growth Factor beta / genetics
Scalp
Skin Neoplasms* / drug therapy,  genetics,  surgery
Treatment Outcome
Chemical
Reg. No./Substance:
0/Antineoplastic Agents; 0/Collagen Type I; 0/Piperazines; 0/Pyrimidines; 0/collagen type I, alpha 1 chain; 152459-95-5/imatinib; 62229-50-9/Epidermal Growth Factor; EC 2.7.10.1/Receptor, Platelet-Derived Growth Factor beta

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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