Differential sensitivity to imatinib of 2 patients with metastatic sarcoma arising from dermatofibrosarcoma protuberans

Maki, R. G., Awan, R. A., Dixon, R. H., Jhanwar, S., Antonescu, C. R. (August 2002) Differential sensitivity to imatinib of 2 patients with metastatic sarcoma arising from dermatofibrosarcoma protuberans. Int J Cancer, 100 (6). pp. 623-6. ISSN 0020-7136 (Print)0020-7136 (Linking)

URL: https://www.ncbi.nlm.nih.gov/pubmed/12209598
DOI: 10.1002/ijc.10535


Dermatofibrosarcoma protuberans (DFSP) is a rare superficial sarcoma usually affecting the trunk, with significant risk of local recurrence. It is characterized by the presence of ring chromosomes or chromosomal translocations fusing the promoter of the collagen gene COL1A1 to the platelet-derived growth factor beta-chain gene PDGFB, increasing the production of PDGF locally and promoting autocrine or paracrine tumor growth. Fewer than 5% of patients with DFSP develop metastatic sarcoma, with a poor subsequent prognosis. Imatinib (STI-571) was developed as an inhibitor of the PDGF receptor tyrosine kinase and has proven clinical activity against chronic myelogenous leukemia (expressing bcr-abl) and gastrointestinal stromal tumors (expressing c-kit). We describe 2 patients with metastatic and unresectable metastases from DFSP treated with imatinib. After confirmation of negative CD117 status of 2 sarcomas arising from DFSP, patients were given imatinib 400 mg po qd and assessed at regular intervals for their tolerance and response to therapy. One patient had a transient response, then progressed rapidly and died of disease. Another patient showed a partial response to therapy after 2 months, with resolution of superior vena cava syndrome and shrinking of metastatic lung lesions. His response is ongoing after 6 months of therapy. These clinical data confirm findings from models of DFSP and support the use of imatinib in the rare setting of metastatic DFSP. Imatinib may be useful for patients with locally advanced DFSP, when other options for local therapy are limited.

Item Type: Paper
Uncontrolled Keywords: Adult Antineoplastic Agents/*pharmacology Benzamides Dermatofibrosarcoma/*drug therapy/pathology Humans Imatinib Mesylate Immunohistochemistry Male Piperazines/*pharmacology Platelet-Derived Growth Factor/metabolism Pyrimidines/*pharmacology Recurrence Sarcoma/*drug therapy/secondary Skin Neoplasms/*drug therapy Time Factors Tomography, X-Ray Computed Treatment Outcome
Subjects: diseases & disorders > cancer > drugs and therapies
diseases & disorders > cancer > metastasis
diseases & disorders > cancer > cancer types > sarcoma
CSHL Authors:
Communities: CSHL labs > Maki lab
Depositing User: Matt Covey
Date: 20 August 2002
Date Deposited: 26 Oct 2016 21:08
Last Modified: 26 Oct 2016 21:08
Related URLs:
URI: https://repository.cshl.edu/id/eprint/33644

Actions (login required)

Administrator's edit/view item Administrator's edit/view item
CSHL HomeAbout CSHLResearchEducationNews & FeaturesCampus & Public EventsCareersGiving