Imatinib in Dermatofibrosarcoma Protuberans (DFSP)

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.

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