A desmoid tumor-staging system separates patients with intra-abdominal, familial adenomatous polyposis-associated desmoid disease by behavior and prognosis

PURPOSE: Intra-abdominal desmoid tumors associated with familial adenomatous polyposis are heterogeneous. A recent staging system categorizes desmoids according to size, symptoms, and complications. We applied the staging system to determine whether it separates patients into clinically significant groups. METHODS: The staging system was applied to 101 patients with familial adenomatous polyposis. RESULTS: There were 21 patients with Stage I tumors, 36 with Stage II, 26 with Stage III, and 18 with Stage IV. Twelve patients with Stage I and five with Stage II tumors needed no treatment. Eight patients with Stage I disease received medical treatment, as did 26 with Stage II, 16 with Stage III, and 15 with Stage IV. Six Stage I tumors had surgery, as did 20 Stage II, 12 Stage III, and 13 Stage IV. Chemotherapy was given to 1 Stage II tumor, 7 Stage III, and 5 Stage IV. No patient with Stages I or II disease died. Four Stage III patients (15 percent) and 8 Stage IV patients (44 percent) died from desmoids. Finally 89 percent with Stage I, 65 percent with Stage II, 59 percent with Stage III, and 50 percent with Stage IV disease were asymptomatic; 81 percent of Stage I desmoids, 78 percent of Stage II, 42 percent of Stage III, and 28 percent of Stage IV were stable or disappeared. CONCLUSION: Desmoid staging identifies tumors by prognosis and its use for designing prospective treatment studies is reasonable.