Comparison of sporadic and FAP‐associated desmoid‐type fibromatoses. Issue 6 (1st June 2017)

• Record Type: Journal Article
• Authors: Koskenvuo, Laura; Ristimäki, Ari; Lepistö, Anna
• Is Part Of: Journal of surgical oncology. Volume 116:Issue 6(2017)
• Page Start: 716
• Page End: 721
• Publication Date: 2017-06-01
• Subjects: aggressive fibromatosis -- desmoid tumor -- desmoid‐type fibromatosis -- familial adenomatous polyposis (FAP); Cancer -- Surgery -- Periodicals; Neoplasms -- Periodicals; 616
• DOI: 10.1002/jso.24699
• Languages: English
• ISSNs: 0022-4790

Abstract

Abstract : Background and Objectives: 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. Methods: Altogether 220 patients were included in the study after receiving a diagnosis of desmoid‐type fibromatosis by the Pathology Department of Helsinki University Hospital, with adequate follow‐up. Patients were included from January 1, 1980 until April 30, 2015. Results: FAP‐related tumors were found in 22 (10%) patients. FAP‐related desmoid‐type fibromatoses were larger, more commonly multiple, and more often intra‐abdominally situated. Surgery was the treatment of choice for 179 (90%) of the sporadic patients and for 18 (82%) of FAP‐related patients. Resections with non‐involved margins (R0) were more common in sporadic desmoid‐type fibromatoses (55% vs. 23%, P = 0.048). The risk of recurrence was 25% in sporadic‐ and 44% in the FAP‐related group. Three (14%) patients with FAP‐related desmoid‐type fibromatoses died from the disease. Conclusions: 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.