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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Diseases of the colon & rectum 43 (2000), S. 1535-1539 
    ISSN: 1530-0358
    Keywords: Telomere ; Desmoid ; Fibromatosis ; Familial adenomatous polyposis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract PURPOSE: The aim of this study was to establish the length of telomeres in familial adenomatous polyposis-associated desmoids. METHODS: DNA from 21 desmoids and five desmoid precursor lesions was digested withHinfI andRsaI restriction enzymes. Southern blotting of the resolved fragments was performed, and the membranes were hybridized with a specific probe attached to a chemiluminescent substrate. Terminal restriction fragment lengths were measured. RESULTS: The median terminal restriction fragment length for the desmoids was 8 (range, 6–9.6)vs. 7.7 (range, 6.4–9.9) kb for their controls. Median terminal restriction fragment length for the desmoid precursor lesions was 9 (range, 7.8–10.4)vs. 8.8 (range, 6.8–10.9) kb for their controls. There was no statistically significant difference between samples and their controls. CONCLUSION: Immortality may not be necessary for desmoid development, or there may be other mechanisms maintaining telomere length. Novel treatments involving telomerase inhibition will be inappropriate in the management of desmoids.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Diseases of the colon & rectum 43 (2000), S. 1012-1014 
    ISSN: 1530-0358
    Keywords: Familial adenomatous polyposis ; Desmoid ; Intrahepatic
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Desmoids are uncommon proliferations of fibroblasts that occur with disproportionate frequency in patients with familial adenomatous polyposis. They do not metastasize and are histologically benign. Despite this, the unpredictable and often aggressive nature of familial adenomatous polyposis-associated desmoids and their tendency to occur in intra-abdominal sites means that they present a difficult management problem, and they are a leading cause of death in patients with familial adenomatous polyposis who have undergone colectomy. We report a case of a patient with familial adenomatous polyposis who had extensive and aggressive desmoid disease and whose management was further complicated by a large intrahepatic desmoid. There are no previous reports of desmoids occurring in the liver.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Diseases of the colon & rectum 43 (2000), S. 1759-1762 
    ISSN: 1530-0358
    Keywords: Desmoid ; Familial adenomatous polyposis ; Surgery
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract PURPOSE: Desmoids are rare, locally aggressive but nonmetastasizing fibrous masses that occur sporadically and in association with familial adenomatous polyposis. Therapeutic options are limited, and there is reluctance to operate on mesenteric desmoids because of the risk of complications or recurrence. Consequently, there is an increasing reliance on alternative therapeutic modalities. Nonsurgical options are of variable efficacy, however, and surgery is still required for the complications of desmoids. Each of the last four patients with desmoids presenting to this unit has required life-saving surgery as a result of failure of nonsurgical treatments or the development of complications. METHODS: We present case reports of four consecutive patients with large mesenteric desmoid tumors requiring surgical management. RESULTS: All four patients had massive mesenteric desmoids. Three cases associated with familial adenomatous polyposis had developed their desmoids after colectomy while the sporadic desmoid had continued to grow rapidly after diagnosis at laparotomy. Sulindac and toremifene were unsuccessful in all cases and one patient with familial adenomatous polyposis suffered dramatic erosion of her desmoid through the abdominal wall during antisarcoma chemotherapy. Two others required emergency laparotomy for complications, and the sporadic case underwent elective resection for symptomatic relief. Three had complete excision of their desmoid, and all remained well with no recurrence at a median follow-up of 12 (range, 7–14) months. CONCLUSION: Despite the risks, there remains a role for surgery in the management of large mesenteric desmoids.
    Type of Medium: Electronic Resource
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