Desmoid tumors

Desmoid tumors (aggressive fibromatosis) are rare, locally aggressive soft-tissue tumors that infiltrate surrounding structures but do not metastasize. Many are driven by aberrant Wnt/β-catenin signaling, and gamma-secretase inhibition of the Notch pathway has emerged as a targeted strategy. Progression-free survival and tumor-volume response are the key efficacy readouts.

Also known as: desmoid tumor, aggressive fibromatosis, desmoid fibromatosis