Literature DB >> 29488047

Urological sequelae of desmoids associated with familial adenomatous polyposis.

S J Walton1,2, G Malietzis3, S K Clark4,5, E Havranek6.   

Abstract

The aim of this retrospective cohort study was to review urological complication rates arising from familial adenomatous polyposis associated desmoid tumours and their management. All patients over a 35-year period were identified from a prospectively maintained polyposis registry database and had an intra-abdominal desmoid tumour. Those without ureteric complications (n = 118, group A) were compared to those that developed ureteric obstruction (n = 40, group B) for demographics, treatment interventions and survival outcomes. 158 (56% female) patients were identified. Median age at diagnosis was 31 years and desmoids typically occurred 3.6 years after colectomy for familial adenomatous polyposis. Ureteric obstruction secondary to tumour growth occurred in 25% of cases. There was no significant difference in gender distribution or overall age at desmoid diagnosis between the two groups. In group B, the median age at desmoid diagnosis was significantly younger in women compared to men (25 and 43 years, respectively) (p = 0.01). Thirty-eight percent of patients already had ureteric obstruction at desmoid diagnosis, the remainder occurred after 48.6 months, but 20 years in two cases. Seventy-three percent (29/40) had ureteric stenting, a long-term requirement for most. Permanent renal injury occurred in six cases but survival between the two groups was not significantly different. Ureteric obstruction occurs frequently in patients with familial adenomatous polyposis and an intra-abdominal desmoid tumour. Those most at risk are the young following colectomy. Clinicians should actively survey the renal tract at regular intervals after a diagnosis of an intra-abdominal desmoid tumour as complications can arise insidiously, at any stage.

Entities:  

Keywords:  Familial adenomatous polyposis; Mesenchymal tumour; Small bowel mesentery; Ureteric obstruction

Mesh:

Substances:

Year:  2018        PMID: 29488047     DOI: 10.1007/s10689-017-0064-0

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


  12 in total

1.  Ureteric obstruction in familial adenomatous polyposis-associated desmoid disease.

Authors:  Myles Joyce; Emilio Mignanelli; James Church
Journal:  Dis Colon Rectum       Date:  2010-03       Impact factor: 4.585

2.  Risk factors predicting desmoid occurrence in patients with familial adenomatous polyposis: a meta-analysis.

Authors:  A Sinha; P P Tekkis; D C Gibbons; R K Phillips; S K Clark
Journal:  Colorectal Dis       Date:  2010-06-02       Impact factor: 3.788

3.  Desmoids and genotype in familial adenomatous polyposis.

Authors:  James Church; Xhileta Xhaja; Lisa LaGuardia; Margaret O'Malley; Carol Burke; Matthew Kalady
Journal:  Dis Colon Rectum       Date:  2015-04       Impact factor: 4.585

4.  β-catenin (CTNNB1) mutations and clinicopathological features of mesenteric desmoid-type fibromatosis.

Authors:  Sebastian Huss; Julia Nehles; Elke Binot; Eva Wardelmann; Jens Mittler; Michaela Angelika Kleine; Helen Künstlinger; Wolfgang Hartmann; Peter Hohenberger; Sabine Merkelbach-Bruse; Reinhard Buettner; Hans-Ulrich Schildhaus
Journal:  Histopathology       Date:  2012-09-28       Impact factor: 5.087

5.  Screening guidelines and premorbid diagnosis of familial adenomatous polyposis using linkage.

Authors:  G M Petersen; J Slack; Y Nakamura
Journal:  Gastroenterology       Date:  1991-06       Impact factor: 22.682

6.  Frequent mutations in the beta-catenin gene in desmoid tumors from patients without familial adenomatous polyposis.

Authors:  Y Miyoshi; K Iwao; G Nawa; H Yoshikawa; T Ochi; Y Nakamura
Journal:  Oncol Res       Date:  1998       Impact factor: 5.574

7.  Family history, surgery, and APC mutation are risk factors for desmoid tumors in familial adenomatous polyposis: an international cohort study.

Authors:  Marry H Nieuwenhuis; Jérémie H Lefevre; Steffen Bülow; Heikki Järvinen; Lucio Bertario; Solen Kernéis; Yann Parc; Hans F A Vasen
Journal:  Dis Colon Rectum       Date:  2011-10       Impact factor: 4.585

8.  The desmoid tumor. I. Incidence, sex-, age- and anatomical distribution in the Finnish population.

Authors:  J J Reitamo; P Häyry; E Nykyri; E Saxén
Journal:  Am J Clin Pathol       Date:  1982-06       Impact factor: 2.493

9.  Familial adenomatous polyposis: desmoid tumours and lack of ophthalmic lesions (CHRPE) associated with APC mutations beyond codon 1444.

Authors:  R Caspari; S Olschwang; W Friedl; M Mandl; C Boisson; T Böker; A Augustin; M Kadmon; G Möslein; G Thomas
Journal:  Hum Mol Genet       Date:  1995-03       Impact factor: 6.150

10.  Guidelines for the clinical management of familial adenomatous polyposis (FAP).

Authors:  H F A Vasen; G Möslein; A Alonso; S Aretz; I Bernstein; L Bertario; I Blanco; S Bülow; J Burn; G Capella; C Colas; C Engel; I Frayling; W Friedl; F J Hes; S Hodgson; H Järvinen; J-P Mecklin; P Møller; T Myrhøi; F M Nagengast; Y Parc; R Phillips; S K Clark; M Ponz de Leon; L Renkonen-Sinisalo; J R Sampson; A Stormorken; S Tejpar; H J W Thomas; J Wijnen
Journal:  Gut       Date:  2008-01-14       Impact factor: 23.059

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