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Annals of Surgical Oncology

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01-10-2007 | Bone and Soft Tissue Sarcomas

Dermatofibrosarcoma Protuberans: Recent Clinical Progress

Author: Grant McArthur, MBBS, BMedSc, PhD, FRACP

Published in: Annals of Surgical Oncology | Issue 10/2007

Abstract

Background

Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous tumor of low malignant grade characterized by a pattern of slow, infiltrative growth and a marked tendency to recur locally after surgical excision. Wide surgical resection is generally accepted as optimal treatment for DFSP. However, despite optimal surgical management, distant metastases may develop in up to 5% of patients. More than 90% of DFSP are characterized by a reciprocal chromosomal translocation, t(17;22). This rearrangement leads to constitutive activation of the platelet-derived growth factor receptor (PDGFR) as a result of deregulated ligand expression, thus providing a rationale for targeted inhibition of PDGFR as a treatment strategy for patients with unresectable locally advanced or metastatic DFSP.

Methods

This article reviews the current understanding of DFSP, with emphasis on molecular-level pathogenetic events and their implications for management, and evidence for the role of tyrosine kinase inhibition in improving the outcomes of patients with unresectable locally advanced or metastatic DFSP.

Results

Surgery with wide margins remains the cornerstone in the management of DFSP. Recently, imatinib, a potent, selective inhibitor of the PDGFR alpha and PDGFR beta protein-tyrosine kinases, has been reported to induce complete or partial remissions in most patients treated for advanced DFSP.

Conclusions

Imatinib is approved for treatment of adult patients with unresectable, recurrent, and/or metastatic DFSP who are not eligible for surgery. Future investigations will determine whether imatinib can also be used in the neoadjuvant setting to reduce tumor volume, thereby allowing resection of very large DFSP that would otherwise not be resectable.

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Imatinib in dermatofibrosarcoma protuberans (DFSP): sponsored by Dermatologic Cooperative Oncology Group. ClinicalTrials.gov identifier: NCT00122473

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Imatinib mesylate in treating patients with locally recurrent or metastatic dermatofibrosarcoma protuberans (DFSP) or transformed fibrosarcomatous DFSP: sponsored by Southwest Oncology Group and National Cancer Institute. ClinicalTrials.gov identifier: NCT00084630

Title: Dermatofibrosarcoma Protuberans: Recent Clinical Progress
Author: Grant McArthur, MBBS, BMedSc, PhD, FRACP
Publication date: 01-10-2007
Publisher: Springer-Verlag
Published in: Annals of Surgical Oncology / Issue 10/2007
Print ISSN: 1068-9265
Electronic ISSN: 1534-4681
DOI: https://doi.org/10.1245/s10434-007-9480-y

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Dermatofibrosarcoma Protuberans: Recent Clinical Progress

Abstract

Background

Methods

Results

Conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Background

Methods

Results

Conclusions

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