Effects of nimodipine and isradipine on endothelin-1-induced contraction of pregnant rat myometrium

Bladder malignancy in the renal transplant recipient is an infrequent occurrence. The 11 previously reported cases reflect an aggressive tumor growth with invasion, requiring partial or complete cystectomy with or without conduit diversion. We report an additional case in a 40-yr-old woman with a living related renal transplant, who experienced rapid progression of her tumor over 3 wk from initial hematuria to a pelvic mass involving the anterior bladder. Her allograft ureter and native ureters, as well as her left iliac vein, became obstructed with tumor in another 2 wk. Biopsy showed poorly differentiated, invasive transitional carcinoma. Attempted resection was abandoned because of finding tumor involvement in most of the pelvis. Chemotherapy was not attempted. She died 2 wk after her attempted resection from tumor burden. Our report presents a collective review of these previously reported 11 cases plus our case. These bladder tumors demonstrate a rapid progression of invasive disease and respond poorly to chemotherapy. There is a possible association of bladder tumors with cyclophosphamide immunosuppression. An aggressive surgical approach should be followed, especially since these tumors present in a younger age group.     

Rhabdomyosarcoma of the urinary bladder with intraepithelial spread in an adult

Rhabdomyosarcoma of the urinary bladder in adults is exceedingly rare. Reported cases have been of the embryonal botryoid type, as seen in children. We will describe a case of pleomorphic rhabdomyosarcoma occurring in the urinary bladder of an adult. In this case, we noted striking intraepidermal migration of tumor cells, resembling the epidermotropic growth characteristic of tumor cells in Paget disease of the breast. The pathological features of this apparently unique case form the subject of this report.     

UV irradiation of polystyrene pipets releases PCR inhibitors

Wilms' tumor is a renal tumor that develops in childhood and is one of the most common malignant tumors in children under 5 years old. Bone metastases are extremely rare in Wilms' tumor, with only two reported cases in adults. We report the case of a patient who underwent nephrectomy for Wilms' tumor at the age of 10 years and presented with a bone mass in his left femur 13 years later.     

Extrarenal Wilms' tumor: diagnosis and treatment review regarding a case report

OBJECTIVES: To report a case of extrarenal Wilms' tumor, an uncommon site of presentation of this tumor type. The diagnostic and prognostic aspects of this condition are also discussed. METHODS/RESULTS: A case of retroperitoneal extrarenal Wilms' tumor in a 2-year-old child is presented. The patient underwent surgery and received postoperative chemotherapy. At 3-years' follow-up, no evidence of metastasis has been observed. CONCLUSIONS: Extrarenal Wilms' tumor is rare and has been reported in the literature principally as case reports. Its clinical presentation varies according to the extrarenal localization. The procedures utilized to determine the size of the primary tumor, regional node involvement and the presence of distant metastasis are similar to those utilized in Wilms' tumor of the kidney. Our results demonstrate the utility of chemotherapy; the cytostatic agents utilized appear to be as effective as in Wilms' tumor of the kidney. In our view, radiotherapy should be reserved for the large unresectable residual tumor mass and for distant metastasis.     

Renal pathology in WAGR syndrome

The Wilms' tumor-aniridia-genital anomalies-mental retardation (WAGR) syndrome is associated with an increased risk for developing Wilms' tumor. A right nephrectomy was performed following the diagnosis of Wilms' tumor in a 2-year-old girl with WAGR syndrome and chromosome 11, del 11p13. Pathologic examination revealed intralobar nephrogenic rests and a peripelvic multicystic mass, sharply delineated from the adjacent typical intralobar nephrogenic rests and renal parenchyma, which may represent a cystic Wilms' tumor (cystic partially differentiated nephroblastoma). We studied the expression of the H19 gene by in-situ hybridization performed on paraffin sections of the kidney. H19 is an imprinted maternally-expressed gene that is not translated to protein and functions as a regulatory RNA molecule. It is tightly linked with the paternally-imprinted gene of insulin-like growth factor 2. While IGF2 presumably plays a role in tumorigenesis of Wilms' tumor, H19 is not expressed in the majority of Wilms' tumors. The expression of H19 in the intralobar nephrogenic rests was found to be prominent in the component of the blastema and markedly reduced with differentiation to tubular structures similar to the fetal kidney. The differential diagnosis of hyperplastic intralobar nephrogenic rests from a small Wilms' tumor arising in intralobar nephrogenic rests is difficult. Complete understanding of the chain of molecular events occurring in the evolution of Wilms' tumors may lead to the development of tumor markers to be used on paraffin sections and so help in the differential diagnosis of hyperplasia versus malignant transformation.     

Lysine 194 is functional in isocitrate lyase from Escherichia coli

BACKGROUND: Peritoneal involvement by Wilms' tumor indicates stage III disease. CT is the single preferred modality in determining the extent and staging of Wilms' tumor; however, the CT appearances of Wilms' tumor involvement of the peritoneum have not been specifically addressed in the literature. OBJECTIVE: The objective of this study was to demonstrate the CT manifestations when there is involvement of the peritoneum, mesentery and/or omentum in Wilms' tumor. MATERIALS AND METHODS: Four cases of Wilms' tumor form the basis of this report. They were examined on Elscint CT scanners. RESULTS: Masses ("dropped metastases") in the pelvis were present in all four patients. Three patients had masses in the mesentery of the small bowel and sigmoid colon. Infiltration of the greater omentum was identified in two patients as a mantle of tumor separating bowel from the anterior abdominal wall. Ascites was present in two patients. In one patient broad-based solid masses of varying sizes were noted on the parietal and on the visceral surfaces of the peritoneum, and in a different patient a discrete mass was noted in the lesser omentum. CONCLUSION: The peritoneal spaces, recesses, ligaments and folds are invisible unless invaded by disease which is well demonstrated on CT.     

A novel technique for management of the en bloc bladder cuff and distal ureter during laparoscopic nephroureterectomy

PURPOSE: The optimal technique of excising the juxtavesical ureter and bladder cuff during laparoscopic nephroureterectomy is still evolving. We report on a novel transvesical needlescopic (2 mm. instrumentation) assisted technique of en bloc retrieval of the juxtavesical ureter and bladder cuff during laparoscopic radical nephroureterectomy for upper tract transitional cell carcinoma. MATERIALS AND METHODS: Retroperitoneal laparoscopic nephroureterectomy was performed in 8 patients using this technique. Two needlescopic ports (2 mm.) inserted suprapubically into the bladder were used in combination with a cystoscopically positioned Collins knife. RESULTS: Satisfactory circumferential detachment of the bladder cuff and en bloc mobilization of 3 to 4 cm. of the intact pelvic extravesical ureter were achieved transvesically in each case in a manner comparable to open surgery. CONCLUSIONS: This technique simulates established open surgical principles of treating the distal ureter during laparoscopic nephroureterectomy.     

Diagnostic and prognostic role of basic fibroblast growth factor in Wilms' tumor patients

Basic fibroblast growth factor (bFGF) is a potent angiogenic peptide implicated in the growth and metastasis of solid tumors. Elevated concentrations of bFGF have been found in the urine of patients with bladder, prostate, and renal tumors. Furthermore, urinary bFGF levels have been shown to correlate with extent of disease. In order to test the utility of urinary bFGF as a Wilms' tumor marker, we measured bFGF levels in preoperative and postoperative urine samples from 97 patients with Wilms' tumor. Preoperative urine samples (n = 97), early postoperative samples obtained from 1 to 3 weeks after surgery (n = 43), and late postoperative samples obtained from 1 to 6 months after surgery (n = 66) were collected from Wilms' tumor patients at 30 institutions between 1989 and 1993. Urine samples from age-matched controls (n = 17) were also obtained. The bFGF levels were determined in duplicate by a competitive sandwich ELISA capable of measuring bFGF at the pg/ml level. Samples were normalized for creatinine content. Urinary bFGF was elevated in 42% of preoperative samples when compared to controls (>90th percentile of normal). Patients with stage III, IV, and V disease had significantly higher preoperative levels of urinary bFGF when compared to patients with stage I and II disease (P < 0.01). Patients with relapse or persistent disease had significantly elevated late postoperative bFGF levels when compared to disease-free patients and controls (P < 0.05). Thus, in patients with Wilms' tumor, elevated preoperative urinary bFGF levels raise the suspicion of aggressive disease while elevated postoperative levels may indicate recurrence or persistence of disease. These data suggest that bFGF is a biological marker for Wilms' tumor and may have a role in the evaluation of patients with this disease.     

A case of asynchronous renal cell carcinoma and urothelial cancer of the urinary bladder and left ureter

A 49-year-old male with left renal cell carcinoma and urothelial cancer (bladder and residual left ureter), which asynchronously occurred, was reported. He had received radical nephrectomy due to renal cell carcinoma 12 years earlier. He was followed up by his local physician for 7 years postoperatively, during which time no metastatic lesion was detected. However, he presented with macroscopic hematuria on January 7, 1992, and a diagnosis of urinary bladder cancer was made at our hospital. Computerized tomography demonstrated a non-papillary, broad-based tumor on the left wall of the urinary bladder, which histologically was transitional cell carcinoma (grade 3). Radical cystectomy, ureterectomy of the left residual ureter and ileal conduit were performed. Histological examinations showed that the urinary bladder tumor was transitional cell carcinoma, grade 3, pT-3b, and CIS (transitional cell carcinoma, grade 3) was found in the residual left ureter. Chemotherapy containing cis-platinum was performed as an adjuvant therapy, but multiple lung metastatic lesions appeared 2 months postoperatively, the histology of which was transitional cell carcinoma, suggesting metastasis from the urothelial cancer. Chemotherapy was ineffective, and he died of the disease 9 months after the operation. If this patient had been under long-term follow-up, the urothelial cancer may have been resected completely by transurethral resection. Our report indicated the importance of examination of the urinary tract in patients with such cancers, as well as the necessity of long-term follow-up.     

Development of transgenic yellow poplar for mercury phytoremediation

We report a case of a teratoid Wilms tumor arising within a supernumerary ectopic ureteropelvic structure in a 7-year-old boy. The tumor was near the right kidney but was completely separate from it. On the contralateral side, the child had a duplication of the ureteral system. Pathologic examination revealed the mass to be completely enveloped by a fibromuscular sac lined by urothelium. The tumor had a botryoid, polypoid architecture and, in addition to areas of classic Wilms tumor, had extensive squamous, mucinous, and columnar elements. Teratoid Wilms tumors are rare and, to the best of our knowledge, have not previously been described as arising either in anomalous urinary structures or at 7 years of age.     

Ureteral triplication

A case of ureteral triplication is presented. There was reflux of the middle ureter, which did not end in the kidney, but rather ended blindly. There was atrophic pyelonephritis of the upper pole because of an obstructed ureter, and a normal lower caleceal system and ureter. There were three ureters but only two originated in the kidney and two entered the bladder, with only one ureter originating from the kidney entering the bladder normally. The refluxing ureter and the obstructed upper pole and ureter were removed.     

Unilateral implantation of the ureter into the posterior urethra in boys. 5 cases (author's transl)

The authors report five new cases of unilateral implantation of the ureter into the posterior urethra in boys. Study of these cases confirms the classical data, i.e.: --The chief presenting symptom is infection. There is no urinary incontinence. --The principle examinations useful in diagnosis are the I.V.P. and urethroscopy. --The ectopic termination is in most cases associated with a double ureter, arising from the upper pelvis. However, the ureter may be single (1/5). --In three cases out of five the upper kidney was dysplasic, necessitating partial nephrectomy. In two cases out of five the kidney corresponding to the ectopic ureter was functional, making possible conservatrice surgery with reimplantation of the pathological ureter into the bladder.     

Infrequent mutation of the WT1 gene in 77 Wilms' Tumors

Homozygous deletions in Wilms' tumor DNA have been a key step in the identification and isolation of the WT1 gene. Several additional loci are also postulated to contribute to Wilms' tumor formation. To assess the frequency of WT1 alterations we have analyzed the WT1 locus in a panel of 77 Wilms' tumors. Eight tumors showed evidence for large deletions of several hundred or thousand kilobasepairs of DNA, some of which were also cytogenetically detected. Additional intragenic mutations were detected using more sensitive SSCP analyses to scan all 10 WT1 exons. Most of these result in premature stop codons or missense mutations that inactivate the remaining WT1 allele. The overall frequency of WT1 alterations detected with these methods is less than 15%. While some mutations may not be detectable with the methods employed, our results suggest that direct alterations of the WT1 gene are present in only a small fraction of Wilms' tumors. Thus, mutations at other Wilms' tumor loci or disturbance of interactions between these genes likely play an important role in Wilms' tumor development.     

The G401 cell line, utilized for studies of chromosomal changes in Wilms' tumor, is derived from a rhabdoid tumor of the kidney

The histology, ultrastructure, and messenger RNA expression of heterotransplants derived from the G401 cell line (American Type Culture Collection) have been characterized by comparison with Wilms' and rhabdoid tumors of the kidney. This analysis illustrates that the properties of G401 heterotransplant were consistent with a rhabdoid phenotype rather than that of a Wilms' tumor. The G401 cell line has been utilized in recent experiments to demonstrate the central role of chromosome 11 in Wilms' tumor. However, the present results suggest that these experiments may be more relevant to define the involvement of chromosome 11 in rhabdoid tumor of the kidney, a malignancy distinct from Wilms' tumor. This is clinically relevant since the rhabdoid tumor of the kidney is very aggressive and associated with an extremely poor prognosis.     

Mutations of the p53 tumor suppressor gene occur infrequently in Wilms' tumor

Mutations of the p53 tumor suppressor gene occur frequently in a variety of adult-onset tumors, including colon, breast, lung, and brain, yet are infrequently identified in pediatric malignancies. Wilms' tumor, a common solid tumor of childhood, can be associated with mutations of the WT1 gene. Alterations of the p53 gene have been shown to modulate the ability of WT1 to transactivate its targets. Although positive p53 immunostaining has been demonstrated in Wilms' tumors, the correlation to p53 gene mutations is not clear. We examined Wilms' tumor samples for p53 mutations utilizing polymerase chain reaction-single-strand conformation polymorphism analysis and single-strand DNA sequencing. Mutations in the coding region of the p53 gene were demonstrated in 2 of 21 (9.5%) Wilms' tumors. Each mutation yielded a substitution of amino acid residues. One mutation was located in exon 6 and the other in exon 7. Both mutations were found in tumors from patients with advanced stage disease. Focal anaplasia was demonstrated in one of these tumors. Our data suggest that although p53 mutations occur infrequently in Wilms' tumor, they may be associated with advanced disease.     

Fine needle aspiration cytology of clear cell sarcoma of the kidney. A case report

BACKGROUND: Fine needle aspiration cytology is an established method for cytodiagnosis. Its application is particularly suited to the pediatric age group. A correct cytodiagnosis of especially large tumors can result in timely cytoreductive chemotherapy, thereby facilitating surgery. While the cytomorphology of Wilms' tumor is well documented, there is a paucity of literature concerning other uncommon pediatric renal tumors. CASE: A 2-year-old female underwent surgery for a renal mass following aspiration cytology. The histologic diagnosis was clear cell sarcoma of the kidney (CCSK). The cytologic smears were cellular and pleomorphic. The main feature of cytologic interest was the presence of deep nuclear indentations and grooves in many of the tumor cells. CONCLUSION: The cytologic features of CCSK are distinct and different from those of other renal tumors in children. Its recognition in cytology is important because its behavior is more aggressive than that of Wilms' tumor. A correct diagnosis can result in the institution of appropriate treatment.     

Reconstruction of the ureter: a review of operative and functional aspects

The importance of the urodynamic characteristics of the ureter are considered after plastic and reconstructive operations. Choice of the most suitable method is as important as good operative technique. The partial replacement of the lower end of the ureter by a bladder flap shows good results. Different types of bladder flap procedures do not influence the dynamics of the ureter as its continuity is preserved. Irrespective of the material used, failures of autoplastic or alloplastic segmental replacement are due to the lack of functional adaptation of the prostheses. By partial replacement good results may be obtained if vesico-ureteral reflux is avoided.     

TNM classification for urological cancer

The 5th edition of the new TNM classification for urological cancer has been published by UICC in 1997. Herein, the classification of 4 urological carcinomas (kidney, urinary bladder, renal pelvis and ureter, and urethra) is presented and discussed in comparison with the latest revisions in 1987 and 1992. In the 5th edition, the main revised points are as follows: As for kidney, the primary tumor cut off between T1 and T2 was changed from 2.5 cm to 7.0 cm, and the N classification was simplified as for urinary bladder, all muscle invasive tumors (T2 or T3a in the 1992 classification) are included in the T2 category, which is then subdivided into T2a and T2b; in the urethra, new T categories on transitional cell carcinoma of the prostate and prostatic urethra have been added, and the N classification is simplified; there is no change in the classification for the renal pelvis and ureter. According to these changes, a new system of stage grouping is proposed. There may still be widespread disagreement over the appropriateness of some of the changes introduced in the 5th edition of 1997. It is essential to continue efforts to improve the accuracy of determining the clinical extent of malignant tumors, and to work together in order to achieve our objectives for a unified system of TNM classification.     

Association of extrarenal Wilms'' tumor with a horseshoe kidney

Nephroblastoma (Wilms' tumor) is the most common renal malignancy in childhood. Extrarenal Wilms' tumor is uncommon, and the diagnosis is almost always postsurgical. The authors report two cases, located in the sigmoid mesocolon, one of these having an associated horseshoe kidney. Both were treated with excision followed by chemotherapy. They are now 36 months and 7 months postchemotherapy, respectively, and are symptom and recurrence free. Both had favorable histological findings. Association with a horseshoe kidney raises an important issue regarding the histogenesis of these tumors. The authors believe that there is a nexus between the fusion of metanephric blastema during the sixth to seventh week of intrauterine life and the "ectopic" metanephric blastema cells that may give rise to extrarenal Wilms' tumor. Association with a horseshoe kidney with an extrarenal Wilms' tumor has been reported on five previous occasions. The authors closely examine the link between the two.