輸尿管副神經瘤：罕見病例報告

鄭琮翰、盧則宏

國立成功大學醫學院附設醫院 泌尿部

Ureteral Paraganglioma: A Rare Case Report

Tsung-Han Cheng, Ze-Hong Lu

Department of Urology, National Cheng Kung University Hospital, Tainan, Taiwan

Background: Paraganglioma of urinary tract is rare, and was once reported in bladder, kidney and prostate⁽¹⁾. The ureteric paraganglioma is rare, and only 6 cases have been reported^(1-6). Herein, we presented a case of ureteric paraganglioma presenting severe gross hematuria and bladder tamponade.

Case presentation: A 48-year-old female, who denied any systemic diseases, presented with severe gross hematuria and voiding difficulty for one week and came to our hospital emergent department for help.

Tracing back her history, she went to other hospital several weeks ago. Left hydronephrosis and left retroperitoneal mass with massive blood clots in renal pelvis and bladder was detected by Computed tomography (CT) scan. Diagnostic semi-rigid ureteroscopy was performed there, but ureteroscopy cannot pass the compression site. Elevated blood pressure was found during ureteroscopic manipulation according to medical record. Double J stent was placed for hydronephrosis and she came to our hospital for further management. At our emergency department, fever up to 39.2’c was recorded, and blood pressure showed 112/71 mmHg, accompanied with dysuria and urinary frequency and urgency. Basic hemogram revealed leukocytosis and elevated CRP level, but there was no deterioration of renal function. Meanwhile, pyuria was also recorded. CT scan was taken at our hospital, which showed left hydronephrosis with a left retroperitoneal tumor about 4.3cm compressing left ureter with massive blood clots in bladder (Fig 1). Fever gradually subsided under adequate antibiotics treatment. However, gross hematuria persisted with intermittent Foley catheter obstructed with blood clots. Manual Foley irrigation was performed several times but in vain. 24 hours urine collection for catecholamines examination also failed due to bladder tamponade. After discussing with the patient, emergent operation of left retroperitoneal mass excision including possible nephroureterectomy was arranged.

During the operation, a round tumor was found adhesive onto left upper ureter, and severe adhesion between the tumor and gonadal vein was also identified. Extreme high blood pressure(>200mmHg) was detected during tumor manipulation even under multiple antihypertensive agents by anesthesiologists. After tumor blood supply ligation, blood pressure came back to normal range rapidly. The post-operative recovery period was not remarkable. The specimen was 4X3.5X3 cm elastic mass attached to left ureter, compatible with CT findings (Fig 2). The pathologic report revealed paraganglioma attached to the muscular wall of ureter. The neoplastic cells are round to polygonal with abundant eosinophilic granular to clear cytoplasm. The tumor cells are positive for Chromogranin A and Synaptophysin. 

Six months after the surgery, the level of 24 hours urine catecholamines were within normal limit. Computed tomography also revealed no recurrence of retroperitoneal tumor.

Discussion: Paraganglioma which is also described as pheochromocytoma by previous articles of bladder has been first described in 1953 by Zimmerman⁽⁷⁾, and it stands for a very rare subtype of bladder tumor, representing only 0.06% of bladder tumor and 1% of all pheochromocytoma⁽⁸⁾. The onset age was around 20-40 years old, and is more prevalent in women than in men⁽⁹⁾. On top of that, paraganglioma of ureteric origin is rarer, and only six cases around the world have been reported^(1-6)_. The previous reports showed the ureteral paraganglioma could be measured from 3.5 to 6cm and could be located from renal hilum to the site of insertion to bladder. The postoperative follow-up time of all five cases was around 1 to 2 years, and no tumor recurrence was found. For paraganglioma arising from genitourinary tract, bladder is the most common site of growth. The malignancy rate of these tumor has been described as 15%-20%. The ureteric paraganglioma identified so far was probably benign as reported. Also, they could be managed by surgical excision⁽¹⁾. The majority of initial presentation is hypertension or related symptoms, and followed by hydronephrosis. Gross hematuria as initial symptoms are relatively rare. Different diagnosis is a challenge in clinical practice since hypertension is the most common symptoms in previous reports. Furthermore, in our case, blood pressure within normal range is detected at most time, but hypertension was recorded during tumor manipulation. Preoperative catecholamine evaluation such as urine vanillylmandelic acid (VMA) may be helpful for diagnosis. In our case, we failed to collect 24hour urine sample due to repeated bladder tamponade. It needs further study in the future.

Conclusion: We described a mid-age woman with ureteric paraganglioma presenting with gross hematuria. Paraganglioma should be kept in mind if blood pressure surged during operative mobilization of main tumor. The follow-up image study, renal function and urine test of 24-hour period should also be monitored.

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