Walton S J, Malietzis G, Clark S K, Havranek E
The Polyposis Registry, St Mark's Hospital, Watford Road, Harrow, HA1 3UJ, UK.
Department of Surgery and Cancer, Imperial College London, London, UK.
Fam Cancer. 2018 Oct;17(4):525-530. doi: 10.1007/s10689-017-0064-0.
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.
这项回顾性队列研究的目的是评估家族性腺瘤性息肉病相关硬纤维瘤引起的泌尿系统并发症发生率及其治疗情况。在35年期间，从一个前瞻性维护的息肉病登记数据库中识别出所有患有腹腔内硬纤维瘤的患者。将没有输尿管并发症的患者（n = 118，A组）与发生输尿管梗阻的患者（n = 40，B组）在人口统计学、治疗干预措施和生存结果方面进行比较。共识别出158例患者（56%为女性）。诊断时的中位年龄为31岁，硬纤维瘤通常在因家族性腺瘤性息肉病行结肠切除术后3.6年出现。25%的病例因肿瘤生长导致输尿管梗阻。两组在性别分布或硬纤维瘤诊断时的总体年龄方面无显著差异。在B组中，女性硬纤维瘤诊断时的中位年龄明显低于男性（分别为25岁和43岁）（p = 0.01）。38%的患者在硬纤维瘤诊断时已有输尿管梗阻，其余在48.6个月后出现，但有2例在20年后出现。73%（29/40）的患者进行了输尿管支架置入，大多数患者需要长期置入。6例发生永久性肾损伤，但两组之间的生存率无显著差异。输尿管梗阻在患有家族性腺瘤性息肉病和腹腔内硬纤维瘤的患者中很常见。风险最高的是结肠切除术后的年轻人。临床医生在诊断腹腔内硬纤维瘤后应定期积极检查泌尿系统，因为并发症可能在任何阶段隐匿出现。
