Extra-Abdominal Desmoid Tumors Associated with Familial Adenomatous Polyposis
نویسندگان
چکیده
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 adenomatous polyposis syndrome.
منابع مشابه
Multiple desmoid tumors in a patient with familial adenomatous polyposis caused by the novel W421X mutation.
Familial adenomatous polyposis (FAP) is a rare syndrome characterized by the presence of hundreds to thousands of colorectal adenomas and is responsible for less than 1% of all colorectal cancers. The syndrome is also characterized by extra-colorectal features including amongst others upper gastrointestinal tract polyps and desmoid tumors. The syndrome is inherited by an autosomal dominant gene...
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ورودعنوان ژورنال:
دوره 2012 شماره
صفحات -
تاریخ انتشار 2012