Desmoid tumors (aggressive fibromatosis) arise from connective tissue cells or fibroblasts. In general, they are slow growing and do not metastasize; however, locally aggressive desmoid tumors can cause severe morbidity and loss of function. In Mar 28 2017, Shivaani Kummar and others published an article in << Journal of Clinical Oncology>> which title is “Clinical Activity of the γ-Secretase Inhibitor PF-03084014 in Adults With Desmoid Tumors (Aggressive Fibromatosis)”, evaluate objective response rate after therapy with the γ-secretase inhibitor PF-03084014 in patients with recurrent, refractory, progressive desmoid tumors. Seventeen patients with desmoid tumors received PF-03084014 150 mg orally twice a day in 3-week cycles. Archival tumor and blood samples were genotyped for somatic and germline mutations in APC and CTNNB1. Finally, PF-03084014 was well tolerated and demonstrated promising clinical benefit in patients with refractory, progressive desmoid tumors who receive long-term treatment.
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