In:
Journal of Neurosurgery, Journal of Neurosurgery Publishing Group (JNSPG), Vol. 112, No. 5 ( 2010-05), p. 965-977
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 PDGFRβ 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 PDGFβ 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.
Type of Medium:
Online Resource
ISSN:
0022-3085
,
1933-0693
DOI:
10.3171/2009.7.JNS09522
Language:
Unknown
Publisher:
Journal of Neurosurgery Publishing Group (JNSPG)
Publication Date:
2010
detail.hit.zdb_id:
2026156-1
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