Literature DB >> 23397065

Familial adenomatous polyposis: not all masses are desmoids.

J Sloane1, P Ranchod, G Williams, S K Clark.   

Abstract

Familial adenomatous polyposis (FAP) is a multi-system disease characterised by the development of hundreds to thousands of colorectal adenomas which inevitably progress to carcinoma without treatment. It is commonly associated with extra colonic lesions including osteomas, epidermoid cysts and desmoid tumours. Desmoid tumours are troublesome due to their size and bulk and are a significant cause of morbidity and mortality in FAP. This series highlights three cases in which patients with FAP developed masses thought initially to be manifestations of the condition. Further investigation in fact demonstrated neurofibromatosis type 1 in all three patients. All masses in FAP patients cannot be assumed to be disease related (desmoid, osteoma, epidermoid cyst or carcinoma).

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Year:  2013        PMID: 23397065     DOI: 10.1007/s10689-012-9580-0

Source DB:  PubMed          Journal:  Fam Cancer        ISSN: 1389-9600            Impact factor:   2.375


  5 in total

Review 1.  Current ideas in desmoid tumours.

Authors:  N Julian H Sturt; Susan K Clark
Journal:  Fam Cancer       Date:  2006       Impact factor: 2.375

2.  Staging intra-abdominal desmoid tumors in familial adenomatous polyposis: a search for a uniform approach to a troubling disease.

Authors:  James Church; Terri Berk; Bruce M Boman; Jose Guillem; Craig Lynch; Patrick Lynch; Miguel Rodriguez-Bigas; Larry Rusin; Tom Weber
Journal:  Dis Colon Rectum       Date:  2005-08       Impact factor: 4.585

3.  Progesterone receptor expression in neurofibromas.

Authors:  Margaret E McLaughlin; Tyler Jacks
Journal:  Cancer Res       Date:  2003-02-15       Impact factor: 12.701

4.  Identification of FAP locus genes from chromosome 5q21.

Authors:  K W Kinzler; M C Nilbert; L K Su; B Vogelstein; T M Bryan; D B Levy; K J Smith; A C Preisinger; P Hedge; D McKechnie
Journal:  Science       Date:  1991-08-09       Impact factor: 47.728

Review 5.  Extra-intestinal manifestations of familial adenomatous polyposis.

Authors:  Emma J Groen; Annemieke Roos; Friso L Muntinghe; Roelien H Enting; Jakob de Vries; Jan H Kleibeuker; Max J H Witjes; Thera P Links; André P van Beek
Journal:  Ann Surg Oncol       Date:  2008-07-09       Impact factor: 5.344

  5 in total

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