Dejaco C, Ferenci P, Schober E, Kaserer K, Függer R, Novacek G, Gangl A
Department of Gastroenterology and Hepatology, Internal Medicine IV, University of Vienna, Austria.
J Hepatol. 1999 Jul;31(1):156-9. doi: 10.1016/s0168-8278(99)80176-7.
In a 46-year-old man endoscopic retrograde cholangiopancreatography and computed tomography scan showed a stenosis of the common bile duct by a hypodense mass highly suggestive of a Klatskin tumor. Histologic examination of the resected tumor revealed only non-specific inflammatory, fibrotic tissue without any evidence of malignancy. Three months later, the patient presented with hydronephrosis of the left kidney. Computed tomography scan showed a retroperitoneal mass with encasement of the left ureter. A percutaneous nephrostomy was performed and immunosuppressive therapy with prednisolone and azathioprine was initiated. Under this medication, almost complete regression of the pelvic mass and reopening of the ureter were observed within 3 weeks. Eight months later, azathioprine was withdrawn and prednisolone was tapered continuously to a dose less than 10 mg/day. After a follow-up of 2 years, the patient is still well. Although the histologic findings were non-specific, further evaluation of this case suggests that Ormond's disease was responsible for the tumor that had to be resected.
一名46岁男性患者，内镜逆行胰胆管造影术和计算机断层扫描显示，胆总管因低密度肿块而狭窄，高度提示为肝门部胆管癌。对切除肿瘤的组织学检查仅发现非特异性炎症、纤维化组织，未发现任何恶性证据。三个月后，患者出现左肾积水。计算机断层扫描显示腹膜后肿块包绕左输尿管。进行了经皮肾造瘘术，并开始使用泼尼松龙和硫唑嘌呤进行免疫抑制治疗。在这种药物治疗下，3周内观察到盆腔肿块几乎完全消退，输尿管重新通畅。8个月后，停用硫唑嘌呤，泼尼松龙持续减量至每日剂量小于10毫克。经过2年的随访，患者情况仍然良好。尽管组织学检查结果是非特异性的，但对该病例的进一步评估表明，奥蒙德病是导致必须切除的肿瘤的原因。
