A case of bilateral renal cell carcinoma showing a rapid course of death in a long-term hemodialysis patient due to polycystic kidney

A case of bilateral renal cell carcinoma in a 42-year-old polycystic kidney male is reported. He had been treated with hemodialysis for 22 years. An abnormal small mass was found in one of the left renal cystic lesions by screening ultrasonography and CT scan at the 19th year of the hemodialysis. Left radical nephrectomy was performed and the histological diagnosis was a renal cell carcinoma (RCC). There was no evidence of recurrence and metastasis, however, he presented with asymptomatic macrohematuria two years after the operation. CT scan demonstrated the rapidly progressing right renal tumor and multiple para-aortic lymph node swelling. Right nephrectomy and lymphadenectomy were performed and pathological examination showed the advanced RCC with multiple lymph node metastasis. Eleven months after the second operation followed by interferon therapy. he died of multiorgan metastasis of the RCC. This is the first bilateral RCC case in polycystic kidney patient treated with hemodialysis in Japan.     

Extracorporeal shockwave lithotripsy of ureteral stone in a patient with en bloc kidney transplantation: a case report

We report a case of ureterolithiasis in a patient with an en bloc kidney transplantation, using extracorporeal shockwave lithotripsy (ESWL). The patient presented with asymptomatic macrohematuria. Computed tomography revealed a ureteral calculus just below the pyeloureteral junction with hydronephrosis of the medially positioned kidney. Took two sessions of ESWL were required for complete disintegration of the stone. At 3 years after successful treatment, the patient has an excellent functioning and stone-free graft.     

Perinephric liposarcoma mimicking cystic renal tumor

Liposarcoma is one of the most common primary retroperitoneal neoplasms, and the perinephric region is a frequent location for them. Liposarcomas show a variety of radiographic features in terms of histological types and tumor sizes, so the specific diagnosis of liposarcoma is often difficult. We present a unique case of perinephric dedifferentiated liposarcoma mimicking cystic renal tumor. A 71-year-old man presented himself at our hospital with a palpable mass in his upper right abdomen. Abdominal computerized tomography (CT) revealed a well-defined cystic mass at the lower pole of the right kidney that contained heterogeneous solid components and small foci of fat. There were no signs of lymphadenopathy or tumor thrombus in the renal vein. Metastatic evaluation by chest x-ray and bone scan was negative. The probable diagnosis was cystic renal cell carcinoma or atypical angiomyolipoma. Because we could not exclude the possibility of cystic malignancy, a right radical nephrectomy was performed. Grossly, the tumor was predominantly encapsulated by a unilocular fibrous capsule and was filled with bloody fluid and debris. The anterior portion of the tumor was composed of various-sized soft and rubbery masses covered with necrotic tissue. The histological diagnosis was dedifferentiated liposarcoma arising in the perinephric retroperitoneum with extensive necrosis, and the cyst wall was composed of a necrotic tumor with a well differentiated liposarcoma and a fibrous capsule. Although the tumor widely covered the right kidney, there was no microscopic invasion of the kidney. No signs of tumor recurrence were noted six months after the operation.     

Adult Wilms'' tumor mimicking hemorrhagic renal cyst

A 36-year-old man presented with macroscopic hematuria associated with right flank pain. Examination of the patient revealed a cystic mass in the right kidney. Because the mass had increased in size, enucleation of the mass was performed. Histopathological findings revealed nephroblastoma, therefore, radical nephrectomy was performed. We believe the pathogenesis of the cystic formation to be a process in which a tumor that had developed in the pericalyceal region spontaneously ruptured, exuding urine into the perinephric space, forming a cystic mass. The patient is alive with no evidence of disease 24 months after the operation.     

Long ureteral polyp: a case report

A case of long ureteral polyp is presented. The patient was a 62-year-old woman complaining of asymptomatic macrohematuria. Radiological examinations revealed ureteral tumor. A tumor was found with cystoendoscopy, and by transurethral biopsy in bladder the tumor was not malignant. Polypectomy was performed. The tumor removed was fibrous polyp measuring about 8 cm in length.     

Diagnostic Difficulties in Diffuse Xanthogranulomatous Pyelonephritis: A Case Report and Review of the Literature

Xanthogranulomatous pyelonephritis is a rare, severe, and chronic infection of the kidney characterized by the replacement of renal parenchyma by the xanthoma cells which is difficult to diagnose radiologically. We report a 40-year-old male who had vague abdominal mass and fever for more than 2 months. Weight loss and fatigue were the associated symptoms. Ultrasonography revealed a grossly enlarged right kidney with replacement of renal parenchyma by multiple hypoechoic areas with perinephric thickening and a large staghorn calculus. An abdominal computed tomogram demonstrated an irregular, enlarged right kidney with multiple low-density round areas and calculi. Diminished excretion of contrast media and a severe perinephric inflammatory reaction were present. A presurgical diagnosis of xanthogranulomatous pyelonephritis was made. First, drainage of the extrarenal abscess under proper antibiotic coverage was done and later, a two stage nephrectomy was done considering the debilitating condition of the patient.     

Renal inflammatory pseudotumor after embolization for arteriovenous malformation: a case report

A 61-year-old man was admitted to our hospital with the chief complaint of asymptomatic macrohematuria. Because he was diagnosed with arteriovenous malformation of the right kidney by angiography, embolization was performed. However, 2 years and 3 months later, bleeding from the right kidney was detected. Computed tomography (CT) revealed a low density area (1.8 cm) in the right kidney protruding to the renal pelvic adjacent to the old embolized lesion. Since we could not make a conclusive diagnosis as malignant disease by ureteroscopy and angiography, we have decided to follow the case carefully. Nine months later, macroscopic hematuria worsened, and the low density area by CT in the right kidney expanded. Therefore, we finally decided to perform right total nephroureterectomy. The tumor was histologically diagnosed as renal inflammatory pseudotumor. This is the 14th case reported in Japan. We here report the renal inflammatory pseudotumor with a review of the Japanese literature.     

A case of inverted papilloma of the ureter with malignant findings

A 70-year-old female with inverted papilloma of the ureter is presented. She was hospitalized because of asymptomatic macrohematuria. Excretory urography and retrograde pyelography demonstrated a filling defect with smooth contour at the mid-portion of the right ureter. Abdominal computer tomographic (CT) scan showed mass lesion (CT number 41) at the same portion as the filling defect. She was diagnosed as having an ureteral tumor and right nephroureterectomy was performed. The gross specimen contained a 24 X 12 mm, polypoid, pedunculated and smooth-surfaced tumor. Pathological diagnosis was inverted papilloma. However, microscopic examination revealed a malignant finding corresponding to transitional cell carcinoma grade 1. From the Japanese and foreign literature, 25 cases of ureteral inverted papilloma were collected and are reviewed. Clinical and pathological features are discussed.     

肾淋巴管扩张症临床分析

目的 总结肾淋巴管扩张症的临床特点及治疗方法。 方法 分析2008年12月、2010年6月收治的2例肾淋巴管扩张症患者临床资料。例1,女,37岁。右腰腹部痛8d。B超检查示双肾周混合性回声,与肾脏分界欠清。CT检查示双肾被膜下低密度影,内混有散在点状高密度影。保守治疗3周后疼痛缓解。3个月后右侧腰痛加重,CT检查示右肾周被膜下大量积液,B超引导下穿刺置管引流后积液消失;2个月后复查B超示左侧肾周少量积液,穿刺抽液后行积液常规及瘤细胞检查。例2,女,32岁。左腰部酸痛不适3年。B超检查示左肾周围不规则囊性病变,CT检查示左肾后外侧分叶状囊性病变,与肾实质分界不清,左肾受压前移。诊断为左肾淋巴管瘤,硬膜外麻醉下行淋巴管瘤切除术。 结果 例1囊液涂片见大量淋巴细胞和少量中性粒细胞,考虑为淋巴液,临床诊断为肾淋巴管扩张症,随访2个月未见复发。例2术后病理检查囊壁衬以扁平上皮细胞伴淋巴细胞浸润,淋巴管呈囊状扩张,诊断为肾囊性淋巴管瘤。术后随访9个月未见复发。 结论 B超、CT检查有助于肾淋巴管扩张症的诊断,穿刺细胞学和组织病理学检查可确诊。无症状者可密切随访,有症状者可穿刺引流,但复发率较高;也可手术切除扩张淋巴管+无水乙醇破坏内皮细胞治疗,复发率低,但可能发生淋巴漏。     

Emphysematous pyelonephritis in a xanthogranulomatous kidney. An unusual cause of pneumoperitoneum

Emphysematous pyelonephritis is a rare, life-threatening suppurative infection of the renal parenchyma and perinephric tissues. The disease is encountered primarily in patients with diabetes mellitus or ureteral obstruction associated with perinephric and intrarenal gas. Causative organisms are those normally found in the urinary and gastrointestinal tracts; however, anaerobic bacteria have been demonstrated in only 1% of cases. We describe a case of emphysematous pyelonephritis, which presented as an acute abdomen with pneumoperitoneum in a nondiabetic patient. No visceral injury was found at laparotomy. Multiple gas-producing organisms, including Clostridium ramosum (not previously reported, to our knowledge), were the cause of the free intraperitoneal and perinephric air. Subsequent radical nephrectomy revealed a xanthogranulomatous kidney. An aggressive surgical approach combined with intensive antibiotic therapy, after aerobic and anaerobic culture of excised tissue, is lifesaving.     

Bilateral renal cell carcinoma: report of a case

A case of asynchronous bilateral renal cell carcinoma is reported. The patient was a 71-year-old man who visited our clinic with complaints of asymptomatic macrohematuria and fever on November 20, 1960. Clinical diagnosis was left renal tumor and left nephrectomy was performed on December 4, 1960. Histological diagnosis was renal cell carcinoma (common type, clear cell subtype, alveolar type and G1). The postoperative course was uneventful until complaints of diarrhea and weight loss in November, 1983. He visited our clinic again with a right abdominal mass on January, 1984. Right renal selective angiography revealed an enlargement and abnormal vascularity with tumor stain, hypervascularity and pooling in the whole kidney except for the upper pole lesion. CT scan revealed a space occupying lesion. Right radical nephrectomy was performed on March 6, 1984. Histological diagnosis was renal cell carcinoma (common type, mixed subtype, alveolar and tubular type, G2). He was treated by hemodialysis and steroid therapy after right nephrectomy but he died of massive gastro-intestinal bleeding on April 22, 1984. The paper is the 15th report of a bilateral renal cell carcinoma in Japan.     

Diffuse xanthogranulomatous pyelonephritis in a child with severe complications

Xanthogranulomatous pyelonephritis is a rare disease in childhood. Because the symptoms and signs are chronic and non-specific, preoperative diagnosis is usually difficult. We report an 8-year-old boy who had an abdominal mass and anemia for more than 6 months. Fever and dyspnea occurred 4 days prior to admission. Ultrasonography revealed an enlarged right kidney with multiple parenchymal hypoechogenic areas, absence of normal parenchymal structures, and perinephric thickening with multiple calcifications. An abdominal computed tomogram demonstrated an irregular, enlarged right kidney with multiple low-density round areas consistent with hydronephrosis and calculi. Diminished excretion of contrast media and a severe perinephric inflammatory reaction were present. Poor right kidney function was demonstrated by Tc^99m -diethylenetriamine penta-acetic acid split renal function examination. We diagnosed xanthogranulomatous pyelonephritis preoperatively based on the clinical and radiological features. The child first had drainage of an extrarenal abscess and antibiotic therapy, followed by definitive nephrectomy. The hospital course was complicated with pleural effusion, peritonitis, pelvic abscess, and sepsis. A two-stage nephrectomy requiring less radical resection and decreasing the surgical complications would have been preferable.     

Erythropoietin-Producing Renal Cell Carcinoma Arising from Acquired Cystic Disease of the Kidney

A 49-year-old woman had been on hemodialysis for 18 years. She presented with left back pain and macrohematuria. Radiologic studies demonstrated a left renal tumor with acquired cystic disease of the kidney. Her serum erythropoietin (EPO) level was 78.4 U/L despite no history of EPO supplementation. Left radical nephrectomy was performed. Pathologic examination revealed EPO-producing renal cell carcinoma. After surgery, the patient's serum EPO level decreased markedly to 15.1 U/L. The measurement of serum EPO levels may be useful for detecting and monitoring a recurrence of renal cell carcinoma with acquired cystic disease of the kidney in patients on long-term hemodialysis.     

A case of xanthogranulomatous pyelonephritis presenting with the flank subcutaneous mass

A 51-year-old female exhibited fever, left flank pain and left flank mass in March, 1993. Drip infusion pyelography (DIP) revealed a non-functioning left kidney with shadows of calculi, and abdominal computerized tomography (CT) showed renal calculi and multilocular cystic lesions in the left kidney extending through the perinephric space into the mass on the left flank. Percutaneous nephrostomy and percutaneous drainage were performed, followed by left nephrectomy. Histopathological findings revealed xanthogranulomatous pyelonephritis. There have been a few case reports of xanthogranulomatous pyelonephritis forming nephrocutaneous fistula in the back.     

Primary osteosarcoma of the bladder: a case report]

A case report of primary osteosarcoma of the bladder is herein presented. A 75-year-old man presented with asymptomatic macrohematuria. Cystoscopic examination revealed a large tumor with a broad base arisen on the anterior wall. Pelvic computed tomographic scan showed multiple calcifications in the tumor itself. Total cystectomy and ileal conduit were performed. Histopathological diagnosis was osteosarcoma. The patient died 10 weeks after surgery. Metastasis to lung, heart, lymphnode, ureter and peritoneum were identified by autopsy. Primary osteosarcoma of the bladder is a rare tumor, only 25 cases have been reported previously. The prognosis of this tumor is very poor. Nineteen of these patients died within 6 months.     

Danazol therapy of endometriosis of the bladder and ureter: a case report

Endometriosis of the urinary tract is uncommon. A thirty-year-old woman was referred to our hospital, complaining of macrohematuria. Intravenous pyelography (IVP) showed bilateral hydro nephrosis. Cystoscopic examination revealed a mass around the bilateral ureteral orifice. Danazol therapy was commenced. After 6 months of therapy, IVP showed the improvement of hydronephrosis of left kidney and cystoscopy revealed the resolution of most of the mass in the bladder. For definite diagnosis and treatment, danazol therapy is considered to be effective for endometriosis, but if secondary fibrosis has occurred, surgical treatment may be necessary.     

Traumatic rupture of a renal cell carcinoma

After sustaining very minor trauma, a man presented with flank pain and fever. A complete clinical and radiological evaluation led to exploration for suspected ruptured renal carbuncle with perinephric abscess. A ruptured renal cell carcinoma was found. Differential diagnostic considerations include renal tumor, abscess, cyst, and hydronephrotic kidney. Radical nephrectomy is indicated if the contralateral kidney can sustain life. The patient shows no sign of recurrence or metastases 4 months following antibiotic treatment and nephrectomy followed by chemotherapy and radiation of the renal bed.     

Carcinoma of the urachus--report of 4 cases

Four cases of urachal carcinoma are presented. The first case was a 36-year-old man who was admitted with the chief complaint of macrohematuria with mucinous debris. Cystoscopic examination revealed a single tumor at the dome of the bladder. Partial cystectomy and postoperative radiation therapy were performed. Histological findings showed a well differentiated adenocarcinoma. He has been well for twelve years postoperatively. The second case was a 52-year-old man who was admitted with the chief complaint of macrohematuria with mucinous debris. Cystoscopic examination revealed a single tumor at the dome of the bladder. Partial cystectomy and postoperative radiochemotherapy were performed. Histological findings showed a moderately differentiated adenocarcinoma. He died of a recurrent tumor 30 months after operation. The third case was 70-year-old man who was admitted with the chief complaint of macrohematuria with mucinous debris. Cystoscopic examination revealed multiple tumors at the dome of the bladder, internal urethral orifice and right lateral wall. Total cystectomy, ureterocutaneostomy and postoperative chemotherapy were performed. Histological findings showed a poorly differentiated adenocarcinoma. He died of a recurrent tumor 30 months after operation. The fourth case was a 68-year-old woman who was admitted with the chief complaint of macrohematuria with mucinous debris. Cystoscopic examination revealed a single tumor at the dome of the bladder. CT scan demonstrated a mass with calcification extending from the bladder dome superiorly. Partial cystectomy and postoperative chemotherapy were performed. Histological findings showed a well differentiated adenocarcinoma. She has been well for three months postoperatively.(ABSTRACT TRUNCATED AT 250 WORDS)     

A case of acquired hemophilia A discovered by right renal bleeding

An 81-year-old man was referred to our hospital with a chief complaint of asymptomatic macrohematuria. Cystoscopy revealed bleeding from the right ureteral orifice. Various examinations, including ureteroscopy, failed to find any abnormalities. As gingival bleeding followed the macrochematuria, further examinations of blood coagulatory function were undertaken. Decreased factor VIII coagulant activity accompanied by the presence of factor VIII inhibitor was revealed, leading to a final diagnosis of acquired hemophilia A. Macrohematuria and gingival bleeding immediately disappeared with oral administration of prednisolone at 30 mg per day, and the titer of factor VIII inhibitor decreased to an undetectable level by the 45th day of treatment. We emphasized the importance of blood coagulation testing in the examination of patients with macrohematuria.     

Localized amyloidosis of the ureter: report of a case

A case of primary amyloidosis of the ureter is presented. The patient was a 48-year-old woman with a 5-year history of asymptomatic macrohematuria. Roentgenographic examination showed left hydronephrosis and stenosis of the left distal ureter. Histological examination of the biopsied specimen during the operation revealed no malignancy; and, left partial ureterectomy and uretero- vesiconeostomy was performed. Congo red stain proved amyloid deposition after surgery. Rectal biopsy was negative for amyloid; and, other laboratory findings were within normal range. Thus it was diagnosed as a primary localized amyloidosis of the ureter. Importance of intraoperative histologic examination was discussed. This is the 22nd case in the literature.