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.