Treatment of Desmoid Tumor in Familial Adenomatous Polyposis — Mesenteric, Retroperitoneal, and Pelvic Presentations

Patients with familial adenomatous polyposis (FAP) carry a heightened risk of mesenteric desmoid tumor. This protocol covers the specific clinical scenario of mesenteric desmoid tumor arising in the setting of FAP, as well as retroperitoneal and pelvic desmoid tumor.

Clinical scenario

Mesenteric desmoid tumor in a patient with familial adenomatous polyposis, or retroperitoneal desmoid tumor, or pelvic desmoid tumor. Anatomical proximity to bowel and vital structures is central to ongoing clinical assessment in these presentations.

Initial management approach

The initial strategy involves active surveillance — structured monitoring with regular clinical and imaging follow-up — before any decision toward active treatment. The complete regimen and decision criteria are in the full protocol.

Treatment goals

Stable tumor size on imaging
Absence of tumor growth at multiple consecutive follow-up appointments

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1001/jamaoncol.2024.1805

However, a period of active surveillance should be proposed as the initial strategy for all patients with DT, before making any decision in favor of active treatment.

Patients with mesenteric DT, whose further progression or residual disease after medical therapy completion would result in bowel complications, should also be considered for surgical resection.

For nonmesenteric DT, in cases of progression on medical treatment or active surveillance, surgery may be discussed in the multidisciplinary team when morbidity of resection is limited or when further progression or residual disease after therapy completion would result in bowel complications in cases of sporadic mesenteric DTs.

Persistent tumor growth was ascertained by observing tumor size increase at multiple (>2) consecutive follow-up appointments, threatening quality of life (QOL), life, or function or posing a potential risk to the feasibility or effectiveness of first-line treatment.

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