Toremifene in Desmoid Tumor: Prospective Clinical Trial and Identification of Potential Molecular Targets

Inclusion Criteria: * Adult patients (age \> 18 years) with primary or locally recurrent, sporadic or FAP associated, desmoid fibromatosis * Histologically documented diagnosis of DF * At least one measurable site of disease at CT or MRI scans, which has not been previously embolised or irradiated * Progressive disease demonstrated at contrast-enhanced MRI or CT scan by Response Evaluation Criteria in Solid Tumors (RECIST) * Radiologic or clinical evidence of PD in the previous 6 months. Radiologic PD will be defined according to RECIST * ECOG Performance status: 0-2 * Prior hormonal therapy, chemotherapy, or molecular targeted therapies are allowed * Adequate end organ function, defined as the following: total bilirubin \< 1.5 x ULN, SGOT and SGPT \< 2.5 x UNL (or \< 5 x ULN if hepatic metastases are present), creatinine \< 1.5 x ULN, ANC \> 1.5 x 109/L, platelets \> 100 x 109/L * Female patients of child-bearing potential must have negative pregnancy test within 7 days before initiation of study drug dosing. Post menopausal women must be amenorrheic for at least 12 months to be considered of non-childbearing potential. Male and female patients of reproductive potential must agree to employ an effective barrier method of birth control throughout the study and for up to 3 months following discontinuation of study drug * Life expectancy of at least 6 months * Written, voluntary, informed consent. Exclusion Criteria: * Previous history of deep vein thrombosis * Evidence of prolonged QTc \>480 msec (using Bazetts correction, for which the formula is: QTc = QT/√RR) or history of familial long QT syndrome * Previous arrhythmia * Clinically significant bradycardia * Endometrial hyperplasia * Hepatic insufficiency * Other concurrent hormonal therapy, including hormonal contraceptives * Patient has received any other investigational agents within 28 days of first day of study drug dosing. - Female patients who are pregnant or breast-feeding * Patient has a severe and/or uncontrolled medical disease * Patient has a known diagnosis of human immunodeficiency virus (HIV) infection * Patient received chemotherapy within 4 weeks prior to study entry * Patient had a major surgery within 2 weeks prior to study entry.