This protocol covers sporadic (non-FAP) mesenteric desmoid tumor in patients whose disease has progressed past what active surveillance could manage — where tumor growth was confirmed at multiple consecutive follow-up evaluations.
Sporadic mesenteric desmoid tumor arising without familial adenomatous polyposis. These tumors may present with, or develop, gastrointestinal complications including bleeding, bowel obstruction, or tumor abscess — at initial diagnosis, during surveillance, or in the course of medical therapy.
The previous management approach — active surveillance with regular clinical and imaging follow-up — was no longer adequate. The goals of that line were stable tumor size on imaging and absence of tumor growth at multiple consecutive follow-up appointments. Those targets were not achieved. This protocol represents the structured next step.
First, patients with sporadic mesenteric DT may present with gastrointestinal complications of bleeding, bowel obstruction, or tumor abscess either at diagnosis, during active surveillance, or while undergoing medical therapy.
In sporadic mesenteric DT cases with complications, such as bleeding, obstruction, or perforation, surgery plays a critical role.
The goal of surgery in this scenario is not to pursue or achieve an R0 resection because this may be associated with considerable and unnecessary morbidity.
Resection of the tumor should be performed synchronously with the treatment of complications when feasible and without resulting in excessive bowel resection.
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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