2012
DOI: 10.1155/2012/726537
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Extra-Abdominal Desmoid Tumors Associated with Familial Adenomatous Polyposis

Abstract: Extra-abdominal desmoid tumors are a significant cause of morbidity in patients with familial adenomatous polyposis syndrome. Understanding of the basic biology and natural history of these tumors has increased substantially over the past decade. Accordingly, medical and surgical management of desmoid tumors has also evolved. This paper analyzes recent evidence pertaining to the epidemiology, molecular biology, histopathology, screening, and treatment of extra-abdominal desmoid tumors associated with familial … Show more

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Cited by 12 publications
(10 citation statements)
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“…They can be diagnosed at any age, but are more common in young people (10–40 years), and are slightly more frequent in women than in men. Most DFs arise sporadically; some are related to pregnancy and trauma, and others appear as part of a syndrome with colonic polyposis, osteomas, and soft tissue tumours …”
Section: Introductionmentioning
confidence: 99%
“…They can be diagnosed at any age, but are more common in young people (10–40 years), and are slightly more frequent in women than in men. Most DFs arise sporadically; some are related to pregnancy and trauma, and others appear as part of a syndrome with colonic polyposis, osteomas, and soft tissue tumours …”
Section: Introductionmentioning
confidence: 99%
“…Recurrence is often problematic, as there are no clear treatments indicated for DF recurrence [17]. We conducted an investigation into factors involved in DF recurrence using immunohistochemistry and discovered that COX-2 expression was significantly associated with recurrence.…”
Section: Discussionmentioning
confidence: 99%
“…Due to the unpredictable natural history of the disease, it is difficult to establish a treatment regimen for DF [17]. Historically, surgery was the main treatment with or without radiation therapy [2,17].…”
Section: Introductionmentioning
confidence: 99%
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“…Two different types of DTs are described: sporadic ones and those associated with familial adenomatous polyposis (FAP). This manifestation accounts for 5–10% of DF [ 4 ]. There is no sex predilection and the average age at disease development is 30–40 years [ 5 ].…”
Section: Introductionmentioning
confidence: 99%