#20-21 Staging of Desmoid Tumours

Studies #20 and #21

were both important FAP studies in proposing and testing a staging system for abdominal desmoid tumors. Desmoid disease is a real challenge for patients with FAP and our large experience at the Cleveland Clinic allowed some significant research.

Study #20

Church J. Berk T. Boman BM. Guillem J. Lynch C. Lynch P. Rodriguez-Bigas M. Rusin L. Weber T. Collaborative Group of the Americas on Inherited Colorectal Cancer. Staging intra-abdominal desmoid tumors in familial adenomatous polyposis: a search for a uniform approach to a troubling disease. Dis Colon Rectum. 2005; 48: 1528-34.

Introduction: Desmoid tumors are a clinical problem in 12 to 15 percent of patients with familial adenomatous polyposis. There is no predictably effective treatment for intra-abdominal desmoid tumors, which sometimes cause significant complications by their effects on the ureters or bowel. The relative rarity and the clinical heterogeneity of intra-abdominal desmoid tumors make randomized studies difficult to do. In this article a staging system is proposed to make multi-institutional studies easier.

Methods: Intra-abdominal desmoid tumors can be staged according to their size, clinical presentation and growth pattern.

Conclusion: A way of staging intra-abdominal desmoid tumors is proposed to facilitate stratification by disease severity during collaborative studies of various treatments.

Study #21

Church J, Lynch C, Neary P, LaGuardia L, Elayi E. A desmoid tumor-staging system separates patients with intra-abdominal, familial adenomatous polyposis-associated desmoid disease by behavior and prognosis. Dis Colon Rectum 2008; 51: 897-901.

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.