Inclusion criteria

          1. Patients with histologically proven primary or recurrent desmoid tumor with currently
             bi-dimensionally measurable tumor by WHO criteria.

          2. Patients with disease that is either unresectable or for which the patient refuses
             surgery but is currently progressing, as defined by:

               -  20% increase in tumor volume within 6 months OR

               -  Recurrent disease within 1 year of surgery OR

               -  Desmoid related symptoms as documented by a PRO questionnaire and documentation
                  that symptoms are related to desmoid and not prior therapies.

          3. Willingness to provide tumor biopsies prior to treatment and while on treatment

          4. Patients may have been previously treated with local therapies such as surgery,
             radiation, radiofrequency ablation, or cryosurgery provided this has been completed at
             least 4 weeks prior to registration and recovered from therapy related toxicity to
             less than CTCAE grade 2 and show no improvement in tumor size or symptom score.

          5. Patients may have been treated with systemic therapies such as tyrosine kinase
             inhibitors, hormone inhibitors or nonsteroidal anti-inflammatory drugs (NSAIDs)
             provided this has been completed at least 4 weeks prior to registration and recovered
             from any therapy related toxicity to less than CTCAE grade 2 and show no improvement
             in tumor size or symptom score.

          6. Patients may have been treated with systemic therapies such as cytotoxics, biologics
             or other unclassified experimental therapies provided this has been completed at least
             8 weeks prior to registration and recovered from any therapy related toxicity to less
             than CTCAE grade 2 and show no improvement in tumor size or symptom score.

          7. Patients who have been treated with immune therapies such as vaccines, dendritic or
             other whole cell therapies, oncolytic or other viral approaches within the preceding
             12 months should be discussed with the Medical Monitor prior to screening and
             enrollment into the study to determine eligibility.

          8. Age: 18 and over (no pre-pubertal patients)

          9. ECOG Performance status: 0-1

         10. Women of child-bearing potential (WOCBP) and men who are sexually active with WOCBP
             must agree to use one highly effective method of contraception, including hormonal
             contraceptives (e.g. combined oral contraceptives, patch, vaginal ring, injectables,
             and implants); intrauterine device (IUD) or intrauterine system (IUS); vasectomy or
             tubal ligation; and one effective method of contraception, including male condom,
             female condom, cervical cap, diaphragm or contraceptive sponge or abstain from sex for
             the duration of study participation and for 4 months following completion of BC2059
             administration. Should a woman become pregnant or suspect she is pregnant while she or
             her partner is participating in this study, she should inform her treating physician
             immediately. See section 8.7.3 for more information.

             Contraception includes:

               -  Total abstinence (when this is in line with the preferred and usual lifestyle of
                  the patient. Periodic abstinence (e.g., calendar, ovulation, symptothermal,
                  post-ovulation methods) and withdrawal are not acceptable methods of
                  contraception

               -  Female sterilization (have had surgical bilateral oophorectomy with or without
                  hysterectomy), total hysterectomy or tubal ligation at least 6 weeks before
                  taking study treatment. In case of oophorectomy alone, only when the reproductive
                  status of the woman has been confirmed by follow up hormone level assessment

               -  Male sterilization (at least 6 months prior to screening). For female patients on
                  the study the vasectomized male partner should be the sole partner for that
                  patient.

               -  Use of oral (estrogen and progesterone), injected or implanted combined hormonal
                  methods of contraception or placement of an intrauterine device (IUD) or
                  intrauterine system (IUS) or other forms of hormonal contraception that have
                  comparable efficacy (failure rate <1%), for example hormone vaginal ring or
                  transdermal hormone contraception.

               -  Sexually active males must use a condom during intercourse while taking drug and
                  for 4 months after stopping study treatment and should not father a child in this
                  period. A condom is required to be used also by vasectomized men in order to
                  prevent delivery of the drug via seminal fluid.

             In case of use of oral contraception women should have been stable on the same pill
             for a minimum of 3 months before taking study treatment.

             Women are considered post-menopausal and not of child bearing potential if they have
             had 12 months of natural (spontaneous) amenorrhea with an appropriate clinical profile
             (e.g. age appropriate, history of vasomotor symptoms) or have had surgical bilateral
             oophorectomy (with or without hysterectomy) or tubal ligation at least 6 weeks ago. In
             the case of oophorectomy alone, only when the reproductive status of the woman has
             been confirmed by follow up hormone level assessment is she considered not of child
             bearing potential.

         11. Hematopoietic:

               -  Absolute granulocyte count ≥ 1,500/mm3

               -  Platelet count ≥ 100,000/mm3

               -  Hemoglobin at least 10.0 g/dL (transfusion allowed, subjects that require
                  transfusion or growth factor need to demonstrate stable hemoglobin for at least 7
                  consecutive days of hemoglobin ≥ 10 g/dL)

         12. Hepatic:

               -  Bilirubin no greater than 1.5 times institutional upper limit of normal, in the
                  absence of documented Gilbert's syndrome

               -  Transaminases no greater than 3 times upper limit of normal (ULN)

               -  Alkaline phosphatase no greater than 3 times ULN

         13. Renal: Creatinine clearance ≥75 mL/min by Cockcroft-Gault

         14. Pulmonary:

               -  Diffusing capacity of the lung for carbon monoxide (DLCO) greater than 75%
                  predicted by single breath test

               -  Capillary oxygen saturation (O2 sat) > 95% by pulse oximetry

        Exclusion Criteria

          1. Patients who have not recovered to grade 1 from adverse events related to prior
             therapy excluding those considered not clinically significant (ex. Lymphopenia).

          2. History of allergic reactions attributed to compounds of similar chemical or biologic
             composition to BC2059 or other agents used in study

          3. Patients with metabolic bone disease (ex. Hyperparathyroidism, Paget's disease, or
             osteomalacia)

          4. Clinically significant, uncontrolled heart disease and/or cardiac repolarization
             abnormality or QTc > 480 msec

          5. Uncontrolled concurrent illness including, but not limited to: ongoing or active
             infection (Viral, bacterial, fungal or other)

          6. Psychiatric illness/social situations that would limit compliance with study
             requirements

          7. Pregnant and breastfeeding women are excluded from this study. The effects of BC2059
             on the developing human fetus have the potential for teratogenic or abortifacient
             effects. There is an unknown but potential risk for adverse events in nursing infants
             secondary to treatment of the mother with BC2059.

          8. HIV-positive patients on combination antiretroviral therapy are ineligible because of
             the potential for pharmacokinetic interactions with BC2059

          9. Patients with abnormal serum chemistry values other than the specific limits detailed
             above, that in the opinion of the Investigator is considered to be clinically
             significant, should be discussed with the Medical Monitor before being enrolled in the
             study.

         10. Lack of peripheral venous or central venous access or any condition that would
             interfere with drug administration or collection of study samples

         11. Personal history of malignancy except:

               -  Cervical intraepithelial neoplasia;

               -  Skin basal cell carcinoma;

               -  Treated localized prostate carcinoma with PSA <1 ng/mL;

               -  Neoplasia treated with curative intent, in remission for at least five years and
                  considered at low risk of relapse.

         12. Patients with familial adenomatous polyposis.