Urological sequelae of desmoids associated with familial adenomatous polyposis

被引:0
|
作者
Walton, S. J. [1 ,2 ]
Malietzis, G. [3 ]
Clark, S. K. [1 ,2 ]
Havranek, E. [4 ]
机构
[1] St Marks Hosp, Polyposis Registry, Watford Rd, Harrow HA1 3UJ, Middx, England
[2] Imperial Coll London, Dept Surg & Canc, London, England
[3] St Marks Hosp, Dept Surg, Harrow, Middx, England
[4] Northwick Pk Hosp & Clin Res Ctr, Dept Urol, Harrow, Middx, England
关键词
Mesenchymal tumour; Familial adenomatous polyposis; Small bowel mesentery; Ureteric obstruction; RISK-FACTORS; TUMORS; GUIDELINES; MUTATIONS;
D O I
10.1007/s10689-017-0064-0
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
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 31years and desmoids typically occurred 3.6years 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 20years 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.
引用
收藏
页码:525 / 530
页数:6
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