Sulindac and Tamoxifen in Treating Patients With Desmoid Tumor

Inclusion Criteria

• Histologically confirmed desmoid tumor, meeting 1 of the following criteria:
• Newly diagnosed disease
• Not previously treated
• Not amenable to complete surgical resection and/or radiotherapy
• If surgical resection was attempted, there must be gross residual disease measurable by MRI
• Radiographically documented recurrent or progressive disease
• No prior chemotherapy or radiotherapy for the present recurrence
• Tumors that progressed on prior chemotherapy are allowed provided patients have not received chemotherapy for this recurrence
• Measurable disease by gadolinium-enhanced MRI
• No other fibroblastic lesions or fibromatoses
• Lipofibromatosis or desmoplastic fibroma of the bone allowed
• Performance status - Karnofsky Score 50-100% (patients over age 16)
• Performance status - Lansky Score 50-100% (patients age 16 and under)
• At least 8 weeks
• Absolute neutrophil count at least 1,000/mm^3
• Platelet count at least 100, 000/mm^3 (transfusion independent)
• Hemoglobin at least 10.0 g/dL (transfusion allowed)
• No hemophilia
• No von Willebrand disease
• No other clinically significant bleeding diathesis
• Bilirubin no greater than 1.5 times upper limit of normal (ULN)
• Alanine aminotransferase (ALT) less than 2.5 times ULN
• Creatinine adjusted according to age as follows:
• No greater than 0.4 mg/dL (≤ 5 months)
• No greater than 0.5 mg/dL (6 months -11 months)
• No greater than 0.6 mg/dL (1 year-23 months)
• No greater than 0.8 mg/dL (2 years-5 years)
• No greater than 1.0 mg/dL (6 years-9 years)
• No greater than 1.2 mg/dL (10 years-12 years)
• No greater than 1.4 mg/dL (13 years and over [female])
• No greater than 1.5 mg/dL (13 years to 15 years [male])
• No greater than 1.7 mg/dL (16 years and over [male])
• Creatinine clearance or radioisotope glomerular filtration rate at least 70 mL/min
• No prior deep venous thrombosis
• Electrocardiogram (EKG) normal
• Chest x-ray normal
• No prior significant gastrointestinal hemorrhage
• No prior peptic ulcer disease
• Not pregnant or nursing
• Fertile patients must use effective nonhormonal contraception
• No evidence of active graft-versus-host disease
• No allergy to aspirin
• Recovered from prior immunotherapy
• At least 7 days since prior anticancer biologic agents
• At least 6 months since prior allogeneic stem cell transplantation
• More than 1 week since prior growth factors
• No concurrent immunomodulating agents
• No prior nonsteroidal anti-inflammatory drugs (NSAIDs) for desmoid tumor
• More than 2 weeks since prior myelosuppressive chemotherapy (4 weeks for nitrosoureas) and recovered
• No concurrent anticancer chemotherapy
• No prior estrogen antagonists for desmoid tumor
• No concurrent hormonal contraceptives
• No concurrent steroids except for non tumor indications (e.g., asthma or severe allergic reactions)
• No concurrent NSAIDs for desmoid tumor
• Occasional NSAIDs for musculoskeletal or other pain are allowed
• Recovered from prior radiotherapy
• No concurrent adjuvant radiotherapy
• No concurrent participation in another COG therapeutic study