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The Desmoid Tumor Research Foundation

June 2017

Comparison of sporadic and FAP-associated desmoid-type fibromatoses.

Author Information

Anna Lepistö,Ari Ristimäki,Laura Koskenvuo

Department of Gastrointestinal Surgery, Helsinki University Hospital and University of Helsinki, Helsinki, Finland.,Research Programs Unit and HUSLAB, Department of Pathology, Helsinki University Hospital and University of Helsinki, Helsinki, Finland.

Publication

Summary

Desmoid-type fibromatosis is a rare disease of which 7.5-16% have been reported to be related to familial adenomatous polyposis (FAP). We sought to compare the characteristics and treatment of sporadic and FAP-related desmoid-type fibromatoses. The predictors for FAP occurrence among desmoid tumor patients are large tumor size, intra-abdominal location, multiple tumors, and patient's young age. Desmoid-type fibromatosis patients suffer a high recurrence rate, also among those experiencing sporadic tumors, but the risk of death due to the tumor is low. Conversely, desmoid disease represents a substantial cause of death among FAP patients.

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