Unresectable Desmoid Tumours Causing Obstructive Uropathy in Familial Adenomatous Polyposis

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Desmoid Tumours in Familial Adenomatous Polyposis

The term desmoid was first used by Muller [1] to describe the tendon-like aspect and the hard consistence of this type of proliferation (desmos in Greek means band). Desmoid tumours (DTs) are classified as extraor intraabdominal. The extra-abdominal DTs arise from fascial or muscoloaponeurotic structures predominantly of the abdominal wall (Fig 1a, 1b, 1c) and occasionally of the shoulder girdl...

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Desmoid tumours in familial adenomatous polyposis

Desmoids are rare, benign fibromatous lesions, which can arise in patients with familial adenomatous polyposis (FAP), a disorder caused by germline adenomatous polyposis coli (APC) gene mutation. This study investigated the risk of desmoids in FAP, the relation between specific APC gene mutations and desmoid formation, and the clinical characteristics ofFAP patients with desmoids. Eighty three ...

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Familial Adenomatous Polyposis: Screening, Surgery and Desmoid Tumours

.......................................................................................... 6 TIIVISTELMÄ ....................................................................................... 8 LIST OF ORIGINAL PUBLICATIONS ..................................................... 10 ABBREVIATIONS ............................................................................... 11

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[Desmoid tumor in familial adenomatous polyposis].

INTRODUCTION Desmoid tumors (DT) are rare benign tumors that do not metastasize, but tend to invade locally. DT are frequently seen in patients with familial adenomatous polyposis (FAP), and diagnosis and treatment are often difficult. METHOD The article presents the clinical picture, diagnosis and treatment of DT in patients registered in the Danish Polyposis Register by the end of 1999. R...

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Familial adenomatous polyposis and desmoid tumors

Familial adenomatous polyposis (FAP) is a hereditary condition transmitted in an autosomal dominant fashion, which is characterized by the appearance of numerous adenomatous polyps in the colon. This condition results from mutation of the APC gene and accounts for 1% of cases of colorectal cancer. FAP is frequently associated with extracolonic manifestations: desmoid tumors, osteomas, pigmented...

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ژورنال

عنوان ژورنال: Asian Journal of Surgery

سال: 2005

ISSN: 1015-9584

DOI: 10.1016/s1015-9584(09)60351-1