Soon Jia Lin, Lau Weber K O, Seow-Choen Francis, Cheng Christopher W S
Department of Urology, Singapore General Hospital, Singapore.
Asian J Surg. 2005 Jul;28(3):233-7. doi: 10.1016/S1015-9584(09)60351-1.
Desmoid tumour-related ureteral obstruction in familial adenomatous polyposis presents difficult management problems. Its role in altering prognosis for the worse in young patients has been neglected. Two patients are reported and 14 cases in the literature reviewed. Both our patients developed intra-abdominal desmoid tumours following proctocolectomies with pouch reconstruction (performed 0.7 and 2.5 years earlier). Despite therapy with sulindac and tamoxifen, both suffered progressive ureteral obstruction that required initial percutaneous drainage. Ureteral stenting was effective and safe in both patients (10 and 18 months' follow-up, respectively), but the long-term outcome awaits assessment. The optimal regimen of adjuvant medical therapy is uncertain. Successful renal autotransplantation and combination chemotherapy have been tried after failure of conventional therapy.
家族性腺瘤性息肉病中硬纤维瘤相关的输尿管梗阻带来了棘手的处理问题。其在使年轻患者预后恶化方面的作用一直被忽视。本文报告了2例患者，并复习了文献中的14例病例。我们的2例患者在接受直肠结肠切除并回肠储袋肛管吻合术（分别于0.7年和2.5年前进行）后均发生了腹腔内硬纤维瘤。尽管使用舒林酸和他莫昔芬进行了治疗，但2例患者均出现了进行性输尿管梗阻，最初均需要经皮引流。输尿管支架置入术对2例患者均有效且安全（分别随访10个月和18个月），但长期结果有待评估。辅助药物治疗的最佳方案尚不确定。在传统治疗失败后，已尝试成功的自体肾移植和联合化疗。
