Adult wilms tumor in the renal pelvis: case report with review of the literature

An 84-year-old man was referred to our hospital with gross hematuria. Abdominal computed tomography demonstrated a well-circumscribed enhanced mass in the right pelviureteral junction. Retroperitoneoscopic nephroureterectomy was performed because of a clinical diagnosis of renal pelvic carcinoma. Pathologic examination led to a final diagnosis of an adult Wilms tumor arising from the renal pelvis. This is the first report of a Wilms tumor in the renal pelvis of an adult patient.     

成人肾母细胞瘤的诊疗及预后分析（附10例报告）

目的：探讨成人肾母细胞瘤的诊疗方法和预后。方法：回顾性分析10例成人肾母细胞瘤患者的临床资料。男8例,女2例,年龄16～62岁,平均29岁。就诊症状为单纯肉眼血尿3例、腰腹部肿块或腰腹痛4例（伴血尿2例）、无症状查体发现3例。患者均行B超、IVU、尿脱落细胞学检查,8例行CT平扫＋强化检查。影像学检查提示肾占位6例、肾盂占位3例、输尿管占位1例。CT提示肾门或腹腔淋巴结肿大4例。10例均行手术治疗,根治性肾切除6例,患肾、输尿管全段切除＋膀胱输尿管口袖口状切除4例,2例根治性肾切除患者因肿物与周围粘连严重切除部分腹膜。行腹膜后淋巴结清扫5例。结果：所有病例均于术后病理确诊为肾母细胞瘤,预后良好组织型（FH）4例,预后不良组织型（UH）6例,病理证实淋巴结转移3例。按美国国家肾母细胞瘤研究组（NWTS）分期标准,分别为Ⅰ期4例、Ⅱ期3例、Ⅲ期3例。7例患者接受术后辅助放化疗。随访9例患者1～13年,5例无瘤生存,生存时间为1．5～13年,平均4．4年。1例术后1年死于肿瘤肺转移,1例术后1年死于肿瘤脑转移,2例术后2年内局部复发,再次接受手术。结论：成人肾母细胞瘤为罕见的恶性肿瘤,预后较差,确诊主要依靠病理诊断,目前尚无公认的最佳治疗手段。手术治疗并辅以合理、及时的放疗、化疗是改善其预后的有效措施。     

Giant idiopathic renal arteriovenous fistula requiring urgent nephrectomy

Idiopathic renal arteriovenous fistulas (AVFs) with acute hemorrhage are exceedingly rare. However, a rare entity such as a renal AVF may be overlooked and have disastrous consequences, requiring an urgent management decision between endovascular or surgical treatment. We report the case of a 61-year-old man, who presented with painless gross hematuria and clot retention. Computed tomography revealed a giant renal arteriovenous malformation, in the absence of any clinical stigmata such as hypertension or congestive heart failure. Gross hematuria resulted from AVF erosion into the collecting system. Given the size of the AVF, endovascular coil embolization was attempted but deemed too risky, necessitating urgent nephrectomy.     

Childhood leiomyoma of kidney

Renal leiomyoma is a rare cause of hematuria and a rare renal tumor of childhood. It has minimal malignant potential and is difficult to distinguish clinically from Wilms tumor. All cases reported in the literature were treated by nephrectomy.     

Distal nephron carcinoma: a rare kidney tumor

Bellini's duct carcinoma is a very rare tumor originating from the collecting tubules of the kidney. It should be part of differential diagnosis in cases with gross hematuria, failed diagnostic proof of a tumor within the collecting system and a centrally located hypovascular mass. Analysis of the cytoskeleton by means of cytokeratin patterns enables classification as a tumor of urothelial origin.     

Granulocyte-colony stimulating factor producing squamous cell carcinoma of the renal pelvis: a case report

We report a case of granulocyte-colony stimulating factor (G-CSF)-producing squamous cell carcinoma of the renal pelvis. A 71?year-old woman presented with gross hematuria and leucocytosis of 21,300/mm3 (neutrophil : 86%) in the peripheral blood, but with no focus of infection. Right renal pelvic mass was found at a nearby hospital and she was referred to our hospital for examination and treatment. We performed right nephroureterectomy for a right renal pelvic tumor. Hematoxylin-eosin staining revealed squamous cell carcinoma of the renal pelvis and tumor cells stained strongly positive for G-CSF. According to these histopathological findings, we diagnosed this case as G-CSF-producing squamous cell carcinoma of the renal pelvis. She is presently alive without any new recurrent lesions for 12 months.     

Inflammatory myofibroblastic tumor of the renal pelvis

Inflammatory myofibroblastic tumor (IMT) of the genitourinary tract is rare and has been classified into separate groups based on their anatomical site and postulated aetiology. Herein, we present a case of IMT of the renal pelvis. A 44-year-old man presented with gross hematuria. Abdominal computed tomography (CT) and magnetic resonance imaging (MRI) revealed a tumor of the left renal pelvis. Under the diagnosis of left renal pelvic tumor, he underwent left nephrectomy and ureterectomy. Microscopic examination revealed fascicular spindle cell proliferation in an oedematous myxoid background with an infiltrate of plasma cells. The spindle cells were strongly positive for smooth muscle actin (SMA) and vimentin, and negative for desmin and anaplastic lymphoma kinase (ALK). Diagnosis of the lesion was IMT of the renal pelvis.     

成人肾母细胞瘤的诊断与治疗（附12例报告）

目的探讨成人肾母细胞瘤的诊断和治疗方法。方法回顾性分析12例成人肾母细胞瘤患者的临床资料。结果本组患者年龄16-63岁,表现为肉眼血尿2例,腰腹痛6例,4例为体检发现;术前诊断为肾肿瘤9例,肾盂肿瘤3例。12例均行手术治疗,术后病理均诊断为肾母细胞瘤。按照美国国家肾母细胞瘤研究组（NWTS）分期标准,本组分别为Ⅰ期5例、Ⅱ期3例、Ⅲ期3例、Ⅳ期1例。术后辅以放疗和化疗,平均随访41个月,3年生存率为48%。结论成人肾母细胞瘤是一种比较少见的恶性肿瘤,术前确诊困难,手术治疗并辅以放疗、化疗是其主要的治疗方法。     

A case of mesoblastic nephroma in an adult patient

We treated a rare case of adult mesoblastic nephroma. The patient was a 52-year-old Japanese man with the chief complaint of intermittent gross hematuria and left lumbar pain. Abdominal ultrasonography, computed tomography, excretory urography, retrograde pyelography and angiography revealed a left renal tumor suspected to be a left pelvic tumor. A left nephroureterectomy was performed. The histologic examination showed a mesoblastic nephroma. A total of 38 adult mesoblastic nephroma cases were reviewed.     

Renal pelvic tumor in chronic renal failure: report of a case

A 44-year-old female was admitted because of gross hematuria six years after beginning hemodialysis for chronic renal failure. There was a past history of hematuria one year before admission. Retrograde pyelography, computed tomographic scan and angiography had been performed, and a probable malignant tumor of left renal pelvis had been found in 1986. We recommended surgery at the time, but she refused treatment and was followed up. On June 22, 1987, the patient had a second attack of gross hematuria resulting in bladder tamponade, and hematuria from the left ureteric orifice was confirmed at cystoscopy. Ultrasound showed left obstructive nephropathy. Left nephrectomy was performed because of the presumptive diagnosis of malignant tumor. The histological diagnosis was transitional cell carcinoma of the left renal pelvis. Two courses of M-VAC (methotiexate, vinblastine, adriamycin and cisplatin) chemotherapy were accordingly given postoperatively. The incidence of renal pelvic tumor and its chemotherapy in patients with chronic renal failure are discussed.     

Primary amyloidosis of renal pelvis with duplicate collecting system

A case of primary amyloidosis localized to the inferior renal pelvis of a kidney with a duplex collecting system is described. The onset of gross hematuria and mild flank pain necessitated investigations which yielded a clinical impression of tumor within the lower pelvis of a duplex collecting system. At laparotomy a grossly hemorrhagic-appearing mass in the lower pelvis was noted and a left ureteronephrectomy was undertaken. Histologic examination of the surgical specimen revealed amyloid deposition within the inferior pelvis, the corresponding distal portions of the papillae, and the most proximal ureter. Amyloid was not present elsewhere within either the renal parenchyma or the superior collecting system.     

A case of Churg-Strauss syndrome which cheaf complain was gross hematuria

We report a case of Churg-Strauss syndrome complaining of gross hematuria. A 74-year-old man was admitted to our hospital for further examination of gross hematuria. Abdominal CT and retrograde pyelography revealed left renal pelvic tumor, and left nephrectomy was performed. There was no tumor, but submucous hemorrhage was seen in the renal pelvis. The histopathologic diagnosis was allergic granulomatous angitis. The administration of prednisolone was done, but he suddenly died of acute heart failure after 1 month postoperatively.     

Flexible ureteropyeloscopy in the diagnosis of benign essential hematuria

Benign essential hematuria is an uncommon syndrome that constitutes a dilemma in diagnosis for the urologist. We studied 32 patients with flexible ureteropyeloscopy. Previous studies included renal arteriography, computerized tomography, ultrasound and urinary cytology. The entire intrarenal collecting system was inspected in 28 of the 32 patients and discrete lesions were found in 16. The most common finding was a hemangioma on a renal papilla in 11 patients. A discrete lesion was treated in 12 patients with successful results in 11. Nonspecific abnormalities were found in 9 patients and attempts at treatment of these lesions in 4 were unsuccessful. No lesion was found in 5 patients. Flexible ureteropyeloscopy offers a minimally invasive approach for the diagnosis of unilateral gross hematuria. Treatment of solitary small discrete lesions was highly successful.     

Synchronous ipsilateral conventional renal cell and transitional cell carcinoma

Simultaneous occurrence of renal cell carcinoma (RCC) and transitional cell carcinoma (TCC) in the same kidney is unusual. We report a 61-year-old man with ipsilateral synchronous renal adenocarcinoma and renal pelvic TCC. He was referred to our department for gross hematuria and right flank pain. CT and MRI studies revealed a 57 × 50 mm irregular and infiltrative upper right kidney mass with necrotic components. A right radical nephrectomy was done. Pathological diagnosis was a high grade tumor originating from just beneath the intact urothelium of renal pelvis and infiltrating through the parenchyma showing solid and occasional tubular growth patterns. A second tumor in close proximity to the first was reported as well differentiated RCC. This is a rare case of combined renal malignancies.     

A case of amyloidosis of the renal pelvis

We report a case of localized amyloidosis of the renal pelvis and upper ureter arising in a 74-year-old man who presented at our hospital with gross hematuria. The intravenous pyelogram showed right hydronephrosis and stenosis of right renal pelvis and upper ureter. The cystoscopy view was normal, but the right retrograde-ureteropyelogram showed a long irregular stricture of the renal pelvis. Ureteroscopy was performed and rubbing biopsy of edematous, bleeding lesion suggested class IIIb, transitional cell carcinoma. With the diagnosis of a right renal pelvic tumor, right nephroureterectomy was performed. The histology report stated "massive deposits of amyloids are seen in the segment of macroscopically abnormal renal pelvis". Amyloidosis of the renal pelvis is a rare entity and 12 cases were reported in the Japanese and English literature.     

Renal hemangioma and renal artery aneurysm in the Klippel-Trenaunay syndrome

We describe a young woman with the Klippel-Trenaunay-Weber syndrome in whom gross unilateral hematuria developed. A renal arteriogram showed a left renal artery aneurysm. Artery reconstruction was impossible and left nephrectomy was performed. Pathological examination showed multiple renal pelvic hemangiomas as the cause of the gross hematuria.     

Simultaneous bilateral renal pelvic tumors: a case report]

A case of simultaneous bilateral renal pelvic tumors is reported. A 64-year-old man with the chief complaint of gross hematuria and left flank pain was admitted. Clinical investigations revealed a tumor in the right pelvis and ureter, and another tumor in the left renal pelvis. The right ureteral tumor had invaded the bladder. Right nephroureterectomy, total cystectomy, left partial pyelectomy and ureterocutaneostomy were performed. By pathological examination, right renal pelvic and ureteral tumors were non-papillary transitional cell carcinoma, grade 3, pT4, and the left renal pelvic tumor was papillary transitional cell carcinoma, grade 2, pT1. To our knowledge, this is the 16th case of simultaneous bilateral urothelial tumors of the upper urinary tract in Japan.     

Botryoid Wilms tumor: case report and review of literature

A rare case of botryoid Wilms tumor is presented. The main clinical manifestations were persistent low-grade fever, malaise, and proteinuria associated with microhematuria. Ultrasonography revealed an echogenic mass in the right kidney, and a contrast-enhanced mass was found in the dilated collecting system by contrast-enhanced computed tomography. The surgically resected tumor was a polypoid, light-yellow, glistening mass that occupied a large part of the renal pelvis and originated from the pelvicaliceal wall. Part of the tumor extended to the proximal ureter, resulting in hydronephrosis in the involved kidney. No parenchymal lesion was observed. Microscopic examination revealed epithelial, stromal, and blastemal components, which indicated Wilms tumor. Infection had occurred in the hydronephrotic kidney, which presumably had caused the major presenting symptoms. The prognosis of our patient and previously reported cases of botryoid Wilms tumor was good compared with that of typical Wilms tumor, since the botryoid type can be detected at an early stage. Received: 28 August 1998 / Revised: 13 January 1999 / Accepted: 13 January 1999     

Renal cell carcinoma invading the urinary collecting system: implications for staging

PURPOSE: Current TNM staging of renal cell carcinoma is based on the tumor propensity for local extension (T), nodal involvement (N) and metastatic spread (M). Locally advanced renal cell carcinoma may involve the perirenal fat, adrenal glands, renal vein, vena cava and/or urinary collecting system. The existing TNM classification does not reflect the ability of renal cell carcinoma to invade the urothelium. We evaluated the incidence and characteristics as well as overall and cancer specific survival of renal cell carcinoma invading the urinary collecting system. METHODS AND MATERIALS: We reviewed pathological findings in 504 kidneys from 475 patients with renal cell carcinoma who presented to our institution in a 3-year period. Urothelial involvement required evidence of gross or histological invasion of the renal calices, infundibulum, pelvis or ureter. Demographic and survival data were obtained from medical records and an institutional cancer registry for tumors invading the urothelium. Stage specific survival data were then compared with tumors not involving the urinary collecting system. RESULTS: Definitive urothelial involvement by the primary tumor was interpretable in 426 of 504 kidneys. Invasion of the collecting system was identified in 61 of 426 cases (14%). Mean diameter of the invading lesions was 10.2 cm. (range 3 to 26). The majority of cases showed clear cell and sarcomatoid histology. Invasion by a papillary lesion was rare. Involvement of the collecting system was most common at the renal poles. Of 61 lesions invading the collecting system 48 (79%) were stage pT3 or greater, while only 13 (21%) were pathologically localized stage pT2 or less. Vascular invasion was identified in 38 renal cell carcinoma cases (62%) with urothelial involvement. A total of 16 cases (26%) were associated with vena caval thrombus. Invading tumors were high Fuhrman grade III or IV in 43 cases (70%). Overall disease specific survival was poor with a median of 19 months. In patients with localized stage pT1 or pT2N0M0 disease and urothelial invasion median disease specific survival was 46 months. CONCLUSIONS: Renal cell carcinoma lesions involving the renal collecting system are characteristically large, high grade and high stage. Clear cell carcinoma most commonly invades, while invasion by papillary tumors is rare. Overall the prognosis for high stage lesions with urothelial involvement is poor and does not appear significantly different from the reported disease specific survival of patients with high stage lesions without urothelial invasion. Localized tumors 4 cm. or less, which are amenable to elective nephron sparing surgery, rarely invade the urothelium. However, when a low stage pT2 or less renal lesion involves the urinary space, survival appears worse than equivalently staged renal cell carcinoma without invasion. Including urothelial invasion into current TNM staging systems for renal cell carcinoma is unlikely to provide significant additional prognostic or therapeutic information.     

Myxoma of the kidney

The fifth reported case of renal myxoma is described. This tumor was removed from a sixty-two-year-old man with a history of hematuria. Diagnostic evaluation had revealed a mass in the lower pole which produced only splaying of the intrarenal vasculature on arteriography. Right nephrectomy was performed. The myxoma was gelatinous in gross appearance and arose from the collecting system. Although these tumors rarely metastasize, a tendency to local recurrence demands their complete removal.