Imatinib in Dermatofibrosarcoma Protuberans (DFSP)

Study Overview
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Brief Summary
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The purpose of this study is to determine whether imatinib is effective in the treatment of primary and recurrent dermatofibrosarcoma protuberans (DFSP).

Detailed Description
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This study is aimed to investigate the efficacy of imatinib (Glivec) in the treatment of primary and locally relapsed dermatofibrosarcoma protuberans (DFSP). DFSP is a cutaneous neoplasm well known for its overexpression of the platelet-derived growth factor (PDGF). Herein, imatinib provides a systemic treatment option that offers the possibility of a reduction of the wide surgical margins used today in surgery of primary DFSP, or even of a complete avoidance of surgical treatment in this disease. Since imatinib exerts its function via interference with protein tyrosine kinase activities, it inhibits the platelet-derived growth factor receptor (PDGF-R) signaling cascade that plays a crucial role in the pathogenesis and tumor growth of DFSP. Since imatinib has been shown to shrink metastatic lesions of DFSP, there is a strong rationale to expect that it also decreases cell proliferation and tumor growth in primary DFSP.

Official Title
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Open-label Trial of Glivec® (Imatinib Mesylate) in Patients With Primary or Recurrent Dermatofibrosarcoma Protuberans

Conditions
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Dermatofibrosarcoma

Intervention / Treatment
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* Drug: Imatinib (Glivec)


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Histological diagnosis of primary or recurrent dermatofibrosarcoma protuberans Measurable tumor parameters (by magnetic resonance imaging [MRI]) Patient >/= 18 years of age ECOG performance status < 3 Adequate organ function Patients must be able to swallow capsules Female patients of childbearing potential must have negative pregnancy test Written, voluntary, informed consent; must include investigational use of tumor tissue biopsies. Exclusion Criteria: Any evidence of distant metastases Patient has received any other investigational agents within 28 days of first day of study drug dosing Patient is < 5 years free of another primary malignancy except basal cell skin cancer or cervical carcinoma in situ Grade III/IV cardiac problems as defined by the New York Heart Association Severe and/or uncontrolled medical disease Known diagnosis of human immunodeficiency virus (HIV) infection

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Treatment
Allocation: Non-Randomized
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Intervention/Treatment |
| --- |
|Drug: Imatinib (Glivec)|nan|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Tumor response at 6 and 12 weeks |  |  | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Rate of relapse within the first 2 years |  |  | 
| Association of tumor response with cytogenetic and receptor expression status |  |  | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Dermatofibrosarcoma, Imatinib, Tyrosine Kinase Inhibitor