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    Comparison of sporadic and FAP-associated desmoid-type fibromatoses.
    J Surg Oncol 2017 Nov 1;116(6):716-721. Epub 2017 Jun 1.
    Department of Gastrointestinal Surgery, Helsinki University Hospital and University of Helsinki, Helsinki, Finland.
    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.

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    A nation-wide study comparing sporadic and familial adenomatous polyposis-related desmoid-type fibromatoses.
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    The Netherlands Foundation for the Detection of Hereditary Tumours, Leiden, The Netherlands.
    Desmoid-type fibromatoses are neoplasms of fibroblastic origin, occurring sporadically or associated with familial adenomatous polyposis (FAP) coli. By comparing sporadic and FAP-associated desmoid-type fibromatoses, we tried to identify clinical characteristics, which may indicate FAP. Histopathology data of all Dutch patients with desmoid-type fibromatoses diagnosed between 1999 and 2009 were retrieved from PALGA, the nation-wide network and registry of histopathology in the Netherlands. Read More
    Desmoid tumor patients carry an elevated risk of familial adenomatous polyposis.
    J Surg Oncol 2016 Feb 10;113(2):209-12. Epub 2015 Dec 10.
    Department of Colorectal Surgery, Abdominal Center, Helsinki University Hospital, Helsinki, Finland.
    Background: The prevalence of desmoid tumors among patients with familial adenomatous polyposis (FAP) is at least 10%, and the prevalence of FAP among desmoid patients varies between 7.5-16%.

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    Clin Gastroenterol Hepatol 2007 Oct;5(10):1190-4
    Familial Gastrointestinal Cancer Registry and Department of Surgery, Mount Sinai Hospital, University of Toronto, Toronto, Ontario, Canada.
    Background & Aims: Desmoid tumors are non-metastasizing fibromatoses that occur in 10%-20% of subjects with familial adenomatous polyposis (FAP). Intra-abdominal desmoid tumors are a major cause of mortality in FAP. FAP-associated desmoid tumors are linked to trauma, particularly abdominal surgery, family history of desmoids, hormonal factors, and the location of the APC mutation. Read More
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    Department of Biology and Genetics, Medical University, Lódź.
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