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Desmoid Tumor (also called aggressive Fibromatosis) is a rare, benign but locally infiltrative fibroblastic neoplasm that arises from musculoaponeurotic structures.

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It is non-metastasizing, but has a high recurrence rate after excision and can cause significant morbidity due to local invasion.

It is often associated with Familial Adenomatous Polyposis (FAP), particularly in the Gardner syndrome variant.

Desmoid Tumor (NORD)

Etiology

Genetic associations:

https://doi.org/10.1038/s41698-022-00308-1

Type	Association
Sporadic	Most common; linked to β-catenin mutation (CTNNB1)
‣-associated	Occurs in ~15% of FAP patients; often post-colectomy
Gardner syndrome	FAP + desmoid tumors + epidermoid cysts + osteomas

Common Locations:

Location	Clinical Relevance
Abdominal wall	Common in postpartum women; better prognosis
Intra-abdominal	Mesentery or retroperitoneum (FAP patients); can encase bowel or vessels
Extra-abdominal	Shoulder, thigh, chest wall

Clinical features

Typically presents in young adults, with female predominance
Symptoms depend on location:
- Abdominal wall: Palpable mass
- Mesenteric/retroperitoneal: Pain, bowel obstruction, ureteral obstruction
Slow-growing, but locally aggressive
Recurrence rates: up to 40%–60% after surgery

Radiology

https://youtu.be/GNaBdhZ2XNU