巨大後腹腔惡性腫瘤造成雙側腎水腫之案例分享
董世祥1、謝德生1、李君豪2
1國泰綜合醫院外科部 泌尿科； 2一般外科
A huge retroperitoneal tumor causing bilateral obstructive uropathy-- case report
Shih-Shiang Tung1 Teh-Sheng Hsieh1 Chun-Hao Lee2
1Division of Urology, Department of Surgery, Cathay General Hospital, Taipei, Taiwan
2 Division of General Surgery, Department of Surgery, Cathay General Hospital, Taipei, Taiwan
Retroperitoneal tumor causing unilateral hydronephrosis is not unusual. However, a huge retroperitoneal tumor more than 30cm in diameter, leads to bilateral hydronephrosis is rare to see.
A 69-year-old female, with many medical history including diabetes, hypertension, hyperuricemia and deep vein thrombosis came to our emergency department due to dyspnea and general weakness for a few days. PE found a distension abdomen, especially on the right side. Blood tests revealed acute kidney injury (Creatinine: 3.82mg/dL) and chest X-ray showed increased heart size. Kidney echo showed bilateral moderate hydronephrosis, especially of right side. Under the impression of hypervolemic heart failure, the patient was admitted to our CV ward. To survey the cause of bilateral obstructive uropathy, abdominal CT was arranged and showed a 30cm retroperitoneal mass. MRI was then performed and a huge, lobulated mass up to 30cm causing encasement of right ureter and bilateral hydronephrosis, with compression of adjacent IVC causing marked flattening. Other structures including aorta, pancreas head, right kidney and small bowel were also compressed. Retroperitoneal sarcoma was considered first. Other differential diagnosis including lymphoma or desmoid tumor. CT-guided biopsy was performed and found only fibrotic tissue.
Combined surgery by urologist and general surgeon was arranged after her general condition improved by medical treatment for one month (Creatinine: 1.36mg/dL). Right DBJ was indwelled smoothly and laparotomy for en bloc resection of the tumor was performed. During the operation, we found the tumor was soft, movable and elastic. Frozen section reported also only fibrotic tissue. Right DBJ and barely touchable and right ureter was hard to identified and right renal pedicle was hard to manage. With meticulous dissection, we removed the en bloct tumor without injury her right kidney and ureter. The post-operative course was uneventful. The permanent pathology showed well-differentiated liposarcoma with greatest diameter of 31.5cm in size.
Liposarcoma is a rare mesenchymal neoplasm arise from lipocytes. The average age of diagnosis is about 50 year of age. It is the most common subtype and counting about 20% of all soft tissue sarcomas. Well-differentiated liposarcomas (WDLs) is a relatively benign and metastasis was rarely found. However, local invasion or transformation in to more aggressive cell type is reported. Also, local recurrence was about 30-50% of cases, especially when the tumor was located at less accessible sites such as those in the retroperitoneum. A large randomized controlled trial comparing radiotherapy followed by surgery to surgery alone in WDL tumors found little difference between the two regimens. These recurrent patients received surgical resection repeatedly and ultimately may cause death due to their injurious effects on vital organs.
Regardless of the high recurrence rate of the disease, the multiple comorbidities of this patient should also be taken into consideration seriously. We overcame the difficulty of the operation and preserved her right kidney and ureter. As a result, she may have a better chance to confront her medical problems in the future.