Imatinib Mesylate in Treating Patients With Locally Advanced or Metastatic Dermatofibrosarcoma Protuberans or Giant Cell Fibroblastoma

Study Overview
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Brief Summary
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RATIONALE: Imatinib mesylate may stop the growth of tumor cells by blocking the enzymes necessary for their growth. PURPOSE: This phase II trial is studying how well imatinib mesylate works in treating patients with locally advanced or metastatic dermatofibrosarcoma protuberans or giant cell fibroblastoma.

Detailed Description
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OBJECTIVES: Primary Determine the therapeutic activity of imatinib mesylate in patients with locally advanced or metastatic dermatofibrosarcoma protuberans or giant cell fibroblastoma. Determine the progression-free rate at 14 weeks in patients treated with this drug. Secondary Determine objective response rate, progression-free survival, and overall survival in patients treated with this drug. Determine the duration of response in patients treated with this drug. OUTLINE: This is an open-label, non-randomized, multicenter study. Patients receive oral imatinib mesylate twice daily for at least 14 weeks in the absence of disease progression or unacceptable toxicity. Patients with stable disease after 14 weeks receive imatinib mesylate for 12 additional weeks. Patients with a partial or complete response at 14 weeks undergo surgical resection if possible. If surgical resection of all remaining tumor is not possible OR if complete resection is not achieved (section margins positive), patients continue to receive imatinib mesylate in the absence of disease progression Patients are followed monthly for 6 months, every 3 months for 6 months, every 6 months for 2 years, and then annually thereafter. PROJECTED ACCRUAL: A total of 44 patients will be accrued for this study within 2 years.

Official Title
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Phase II Study of Glivec (Imatinib) in Locally Advanced and/or Metastatic Soft Tissue Sarcomas Expressing the t(17;22)(q22;q13) Translocation Resulting in a COL1A1/PDGF-beta Fusion Protein i.e. DermatoFibroSarcoma Protuberans (DFSP) and Giant Cell Fibroblastoma (GCF)

Conditions
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Sarcoma

Intervention / Treatment
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* Drug: imatinib mesylate
* Procedure: conventional surgery


Participation Criteria
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Eligibility Criteria
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DISEASE CHARACTERISTICS: Histologically confirmed dermatofibrosarcoma protuberans or giant cell fibroblastoma Locally advanced or metastatic disease Measurable disease Not amenable to surgery, radiotherapy, or combined modality therapy with curative intent Documented progressive disease within the past 3 months Previously irradiated lesions must show disease progression Tumor expressing COL1A1/PDGF-beta by fluorescence in situ hybridization Translocation t(17;22)(q22;q13) No prior chemotherapy OR previously treated with 1, and only 1, line of combination chemotherapy with ifosfamide and doxorubicin OR 2 lines of single-agent therapy OR relapsed within 6 months after adjuvant chemotherapy PATIENT CHARACTERISTICS: Age 18 and over Performance status WHO 0-2 Life expectancy Not specified Hematopoietic Absolute neutrophil count ≥ 2,000/mm^3 Platelet count ≥ 100,000/mm^3 Hemoglobin ≥ 9 mg/dL* NOTE: *Transfusion allowed Hepatic SGOT or SGPT ≤ 2.5 times upper limit of normal (ULN) (5 times ULN if hepatic metastases are present) Bilirubin ≤ 1.5 times ULN No uncontrolled hepatic disease Renal Creatinine ≤ 1.5 times ULN No uncontrolled renal disease Other Not pregnant or nursing Negative pregnancy test Fertile patients must use effective contraception during and for 3 months after study participation HIV negative No uncontrolled diabetes No active or uncontrolled infection No concurrent severe or uncontrolled medical disease No medical, psychological, familial, sociological, or geographical condition that would preclude study participation, compliance, or giving informed consent No other malignancy within the past 5 years except adequately treated basal cell or squamous cell skin cancer, carcinoma in situ of the cervix, or adequately treated stage I or II cancer currently in complete remission PRIOR CONCURRENT THERAPY: Biologic therapy More than 28 days since prior biologic therapy No concurrent filgrastim (G-CSF) or sargramostim (GM-CSF) No concurrent anticancer biologic agents Chemotherapy See Disease Characteristics More than 28 days since prior chemotherapy No concurrent chemotherapy Endocrine therapy Not specified Radiotherapy See Disease Characteristics At least 6 months since prior radiotherapy No concurrent radiotherapy Concurrent palliative radiotherapy allowed provided radiotherapy will not be administered to a target lesion Surgery Not specified Other More than 28 days since prior investigational drugs No concurrent therapeutic anticoagulation therapy with warfarin Concurrent low-molecular weight heparin or mini-dose warfarin for prophylaxis of central venous catheter thrombosis allowed No other concurrent anticancer agents No other concurrent investigational drugs No other concurrent cytostatic agents No other concurrent tyrosine kinase inhibitors

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
Masking: None (Open Label)


Arms and Interventions

| Intervention/Treatment |
| --- |
|Drug: imatinib mesylate|nan|
|Procedure: conventional surgery|nan|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Progression-free rate at 14 weeks |  |  | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Response rate as assessed by RECIST criteria |  |  | 
| Progression-free survival |  |  | 
| Overall survival |  |  | 
| Duration of response |  |  | 
| Toxicity as assessed by CTC 3.0 |  |  | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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recurrent adult soft tissue sarcoma, stage III adult soft tissue sarcoma, stage IV adult soft tissue sarcoma, adult fibrosarcoma, adult malignant fibrous histiocytoma