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Feasibility Of Pre-Operative Mtor Inhibitor Sirolimus In Children And Young Adults With Desmoid Tumor, Stephanie Verwys, Clara Magyar, Kathleen Glick, Douglas Hawkins, Archana Sharma, Brenda Weigel, Katherine Chastain, Joseph Khoury, Michele Manalang, Sarah Dry, Noah Federman, Aaron Weiss

Maine Medical Center

Background:

• Desmoid tumor represents an intermediate grade neoplasm with a striking predilection for locally invasive growth and recurrence following resection

• More effective, well-tolerated non-surgical treatment options are needed

• Current approaches

• If feasible, watchful waiting is the preferred approach

• 20-30% spontaneous regression

• In situations where treatment is indicated, the following approaches are utilized

• Surgery is the primary approach if minimal morbidity is anticipated

• Medical therapies

• Cytotoxic drugs

• Tyrosine kinase inhibitors

• Hydroxyurea

• Gamma secretase inhibitors

• mTOR Inhibitor Rationale

• Desmoid tumor is well-known to be associated with deregulation of …