Atlas One · Host Biology · Scale 07 — System

Desmoid Tumor

Desmoid tumor (aggressive fibromatosis) is a locally invasive spindle cell neoplasm; ~80% harbor CTNNB1 activating mutations; no metastatic potential; nirogacestat (gamma-secretase inhibitor, DeFi trial) FDA-approved 2023; sorafenib active; watch-and-wait for stable disease.

Also known as: desmoid tumor, aggressive fibromatosis, desmoid fibromatosis, deep fibromatosis, desmoid CTNNB1, FAP desmoid, mesenteric fibromatosis, sporadic desmoid, beta-catenin fibromatosis, APC desmoid

Scale 07 — SystemScale
draftStatus
2026-06-07Last Reviewed
This is an auto-generated stub page built from atlas entry frontmatter. A richer hand-crafted page is planned. See the atlas source for the full entry including detailed body sections.
desmoid-tumor

Desmoid Tumor

Desmoid tumor (aggressive fibromatosis) is a locally invasive spindle cell neoplasm; ~80% harbor CTNNB1 activating mutations; no metastatic potential; nirogacestat (gamma-secretase inhibitor, DeFi trial) FDA-approved 2023; sorafenib active; watch-and-wait for stable disease.

Entry Metadata

FieldValue
IDdesmoid-tumor
NameDesmoid Tumor
Statusdraft
Last reviewed2026-06-07
Atlas01-human
Scale07-system

Cross-Atlas Connections

connects-toCtnnb1 connects-toWnt Beta Catenin connects-toNotch connects-toVegf connects-toApc connects-toFap connects-toFibroblast connects-toGist connects-toSmall Intestine connects-toMusculoskeletal System connects-toColorectal Cancer connects-toEstrogen connects-toTgf Beta

Sources