Treatment of Desmoid Tumor in Familial Adenomatous Polyposis When Active Surveillance Is No Longer Sufficient
This protocol applies to patients with mesenteric desmoid tumor in the context of familial adenomatous polyposis (FAP), as well as those with retroperitoneal or pelvic desmoid tumor, whose disease has not remained stable under active surveillance.
The prior approach — active surveillance with regular clinical and imaging follow-up — aims to confirm stable tumor size and absence of growth at consecutive appointments. When documented tumor growth occurs and that stability goal is not met, the next management step is required.
Treatment targets include absence of disease progression on imaging at 6 months, resolution of any complications, and tumor size stability or reduction.
References
DOI: 10.1001/jamaoncol.2024.1805
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.
In sporadic mesenteric DT cases with complications, such as bleeding, obstruction, or perforation, surgery plays a critical role.
The consensus of the Desmoid Tumor Working Group was that for most of the available treatments, a course of at least 6 months is required (provided there is absence of frank progression) prior to assessing effectiveness.
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