Progress of fundamental research in Wilms'' tumor

The sectional anatomy of the pelvic floor was studied in plastinated sections of adult pelves by computed tomography and by magnetic resonance imaging. In sectional anatomy, the levator ani is composed of three portions that can be clearly distinguished by their planes of cleavage and by the course of their fiber bundles. No muscular connections are found between the levator ani portions and the pelvic organs. The fascia of the levator ani in always interposed between the muscle and the pelvic organs. The sectional anatomy of the sphincter ani externus reveals a subdivision into a subcutaneous and a deep portion. Although the puborectalis portion of the levator ani and the deep portion of the sphincter ani externus are more or less continuous, in sectional anatomy they can be distinguished due to their different origins and attachments.     

Renal function after nephrectomy for Wilms'' tumor]

In times of reduced monetary resources of the current German health system, it is more and more difficult for the German physicians to comply with the high medicinal care standard and to practice economically. Nevertheless, the economical reasons cannot deny the high medical quality standards. Regarding the principle of the unity of jurisdiction, the validity of the social welfare law, that a performance has to be "just sufficient and suitable", must concur with the demand of liability law of "indication of the medical service". The economical duties reach their limit when they increase the risk for the patient. On the other hand, the economy interests have to be regarded by the "principle of the allowed risk". Therefore, it should be considered that in every single case the severity and probability of the risk has to be weighed against the cost aspect.     

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.     

Metachronous bilateral Wilms'' tumor: the importance of time interval to the development of a second tumor

Radiosurgery, a bladeless brain surgery without opening skull, requires higher imaging accuracy as compared to microsurgery. Accordingly, we must refine the ways we use the MR scanner and interpret the obtained images. A well tuned and regularly calibrated MR scanner provides excellent images, which allow us to define detailed intracranial structures without distortion. This enables us to obtain a reliable imaging diagnosis despite the fact that pathologic diagnosis is not available in many radiosurgical patients. Due to its three-dimensional imaging and excellence in tissue and spatial contrast, MR is important in exploring new radiosurgical indications. Large arteriovenous malformations (AVM), dural AVM of cavernous sinus and trigeminal neuralgia are some of the successful examples. By using MR, longitudinal investigation of radiosurgical effects becomes feasible. For tumors, the longitudinal studies are important in optimization of dose selection. For AVM, MR is reliable in verification of radiosurgical result. The reliability is comparable to conventional x-ray angiography. Application of MR enhances the essence of noninvasiveness of radiosurgery.     

Clear cell sarcoma of kidney with tumor extension into the right atrium

A 28-year-old man with a Wilms tumor extending into his right atrium was successfully treated using cardiopulmonary bypass. Histologic examination revealed clear cell sarcoma with invasion of the inferior vena cava. This case represents the oldest patient with clear cell sarcoma of the kidney ever published in the English literature.     

Familial Wilms'' tumor associated with a WT1 zinc finger mutation

Cardiac metastasis from gynecological malignancies is rare. Only six cases of carcinoma of the uterine cervix have been reported where the diagnosis of malignant pericardial effusion was made antemortem. The treatment of neoplastic pericardial effusion is controversial; both surgical and nonsurgical treatments are advocated. We present a patient with pericardial effusion secondary to carcinoma of the cervix and recommend subxiphoid pericardial fenestration for reliable long-term control of malignant effusion.     

Noninvasive detection of bladder cancer with the BTA stat test

PURPOSE: We assess the sensitivity and specificity of the noninvasive BTA stat urine test for detection of primary and recurrent bladder cancer with special reference to the size, grade and stage of the tumors, and examine the effect of intravesical bacillus Calmette-Guerin treatment on the results. MATERIALS AND METHODS: A total of 250 patients recruited from 3 medical centers provided voided urine samples for the BTA stat test and cytopathological study. Of these patients 162 were monitored following resection of bladder tumors and 88 were evaluated for the first time for hematuria or irritative voiding symptoms. Each patient underwent cystoscopy. Biopsies were obtained when a bladder tumor was seen or if carcinoma in situ was suspected. The sensitivity, specificity and accuracy of the BTA stat test were compared to standard voided urine cytology. RESULTS: No tumor was found in 122 patients, primary transitional cell carcinoma was found in 71 and cystoscopy revealed recurrent tumors in 57. Overall sensitivity of the BTA stat test was 82.8% and specificity was 68.9%. Sensitivity of urine cytology was 39.8% and specificity was 95.1%. The BTA stat test detected 90.1% of the primary and 73.7% of the recurrent tumors. All patients with carcinoma in situ, high grade tumors, muscle invasive cancer and tumors larger than 2 cm. were diagnosed by the BTA stat test. CONCLUSIONS: The BTA stat test can be used as a screening test for bladder cancer in patients with hematuria or irritative voiding symptoms and for surveillance of those who have not been treated with intravesical bacillus Calmette-Guerin.     

Histopathological variations among cases of Wilms'' tumor in Bangladesh and its relationship with prognosis

Both glucagon and glucagon-like peptide-1 (GLP-1) play an important role in the regulation of nutrient homeostasis. In this study, the tissue distributions of the expression of receptor genes for glucagon and GLP-1 were examined. Expression of glucagon receptor gene was detected in liver, kidney, ileum and pancreatic islets but not in brain. In contrast, expression of GLP-1 receptor gene was detected in brain, pancreas and pancreatic islets but not in liver, kidney, or ileum. To investigate the existence and characteristics of glucagon and GLP-1 receptors on pancreatic beta cells, expression of the receptor genes and translational regulation of the expression of the receptor genes by glucose were analyzed in a mouse pancreatic beta cell line, MIN6 cells. In the cDNA pool of MIN6 cells, both glucagon and GLP-1 receptor genes were identified and showed higher expression level in MIN6 cells cultured under high glucose condition than in those cultured under low glucose condition. These results suggest that glucagon and GLP-1 receptor genes are expressed in pancreatic beta cells and their expression is upregulated by glucose.     

Yolk sac tumor of the bladder

A 1-year-old white boy presented with hematuria and was found to have a large polypoid bladder mass. Light and electron microscopic, and immunohistochemical diagnosis was a yolk sac (endodermal sinus) tumor. This is the first case of a primary yolk sac tumor of the bladder to be described in the English literature.     

Molecular genetics of Wilms'' tumour

The cardiopulmonary response elicited by intravenous bacteria or endotoxin is well characterized in swine and has two major components. The first represents the acute pulmonary and broncho-constrictive phase (0-2 h) and the second phase (3-8 h) represents increased microvascular permeability, hypotension, and enhanced leukocyte-endothelial adhesion. The pulmonary vasoconstriction and bronchoconstriction of phase 1 results in acute pulmonary hypertension and airway dysfunction, which may result in rapid mortality. Because this acute pulmonary response may not mimic the development of human septic shock, we sought to block this early phase and examine the role of tumor necrosis factor in the latter septic phase (3-8 h). Employing a thromboxane A2 (TXA2) receptor antagonist (BAY U 3405) in the presence of LD100 Escherichia coli challenge, we blocked the acute pulmonary hypertensive phase and prevented early mortality, however, TXA2 blockade did not affect the latter development of septic shock and death. This latter lethal phase, characterized by prolonged leukopenia, was blocked in a dose-dependent manner by tumor necrosis factor monoclonal antibody. We conclude that the TXA2-blocked E. coli-challenged swine may provide a novel animal model in which to investigate the pathophysiology of acute septic shock.     

Urine cytology in the detection of bladder tumor recurrence

Of 118 patients with primary bladder tumors seen since 1966, 73 have been followed with urine cytology since 1969. Of the 406 tests there have been 85 positive, 296 negative and 25 ambiguous reports. The incidence of falsely positive results is estimated at 4% but the incidence of falsely negative results cannot be assessed in this study. Currently, 51 patients are living, 2 of whom had been seen in 1966. Of the 51 patients 43 are being followed with urine cytology. Bimonthly urine cytology has been found to be a relaible, convenient, safe, less hazardous and less costly method for the detection of bladder tumor recurrence.     

Constitutive expression of the Wilms'' tumor gene (WT1) in the leukemic cell line U937 blocks parts of the differentiation program

OBJECTIVE: To characterize relationships among blood pressure, pulse rate, vitamin C status and other protective and risk factors for older British people, from a national survey. DESIGN: A cross-sectional analysis of survey data. SETTING: A population study, representative of mainland Britain. SUBJECTS: Among 914 people of both sexes living in the community, 373 were taking blood-pressure-lowering drugs and were therefore excluded from the analyses. INTERVENTIONS: Completion of an interview on health, lifestyle and dietary habits, recording of a 4-day dietary record, anthropometry and taking of a blood sample to determine haematological and biochemical status. MAIN OUTCOME MEASURES: Systolic and diastolic blood pressures, pulse rate, indices of micronutrient status including plasma ascorbate concentration, nutrient intake and haematology. RESULTS: Plasma ascorbate concentration was inversely correlated to systolic and diastolic blood pressures and pulse rate. Other covariates of blood pressure included age, sex, domicile, plasma retinol, fibrinogen and gamma-tocopherol concentrations, erythrocyte count, prothrombin time and urine sodium: creatinine ratio. Covariates of pulse rate included sex, domicile, plasma fibrinogen and platelet count. Blood pressure was also correlated to intake of vitamin C. CONCLUSIONS: Plasma ascorbate concentration and intake of vitamin C are covariates of blood pressure in older people living in Britain. New intervention studies are now needed, to test for possible causalities.     

Recombinant tumor necrosis factor for superficial bladder tumors

Twenty patients with histologically documented superficial bladder cancer (Ta, T1, Tis) were treated with intravesical administration of TNF 400-1800 micrograms. Of 18 patients with a marker lesion, 2 obtained a complete response for 8+ and 18 months. Two had a partial response and were given other intravesical therapies after 5 and 7 months. No or minimal systemic absorption of TNF was observed and documented in 4 of 20 patients by pharmacokinetic studies, and no patients developed antibodies to intravesically administered TNF. TNF was well tolerated in doses up to 1800 micrograms. No systemic or local side effects were observed. Modest activity was attained with intravesical TNF, even in pretreated patients.     

Hepatobiliary tuberculosis presenting as a gall bladder tumor

In this study 52 canine fecal samples were examined for the presence of canine parvovirus (CPV). The two different test systems used to confirm infection were electron microscopy as standard method and an enzyme-linked immunosorbent assay (ELISA). In comparison, the results conferred in 92.3%. The CPV ELISA had a sensitivity of 89.7% and a specificity of 100%. Therefore, it can be used as a quick, reliable method for diagnosis of canine parvovirus infection in veterinary practice.     

Loss of heterozygosity on chromosome 16 in sporadic Wilms'' tumour

Activated cell-mediated immunity, associated for example with HIV infection, is accompanied by elevated concentrations of neopterin and 7,8-dihydroneopterin. Recent data have indicated a role of neopterin derivatives in virus activation and apoptotic cell death, processes likely to involve the action of oxygen free radicals. Because T cell death in AIDS is likely to involve the Fas/Fas ligand system and the action of oxygen free radicals and 7,8-dihydroneopterin, we compared the kinetics and sensitivity of apoptotic cell death of human leukemic Jurkat T cells to that of treatments with 7,8-dihydroneopterin, anti-Fas, and H2O2. Upon incubation with 5 mM 7,8-dihydroneopterin and 50 microM hydrogen peroxide over a period of 24 hr, bimodal kinetics were observed with peaks at 5.5 hr (7,8-dihydroneopterin, 13.1%; H2O2, 11.4%) and at 24 hr (7,8-dihydroneopterin, 11.2%; H2O2, 13.2%). In contrast, anti-Fas (20 ng/mL)-induced apoptosis increased steadily over time, peaking at 11 hr (43.2%). Interestingly, anti-Fas-induced apoptosis was suppressed upon co-incubation with 7,8-dihydroneopterin and H2O2 by 62% and 68%, respectively. We also compared the sensitivity to drug treatments of apoptosis induced by 7,8-dihydroneopterin, anti-Fas antibodies, and H2O2. 7,8-Dihydroneopterin-mediated, and similarly anti-Fas- and H2O2-mediated, apoptosis was not inhibited by a broad range of pharmacological inhibitors, such as actinomycin D, cycloheximide, cyclosporin A, and various protein kinase inhibitors. On the contrary, inhibitors with antioxidant abilities, such as pyrrolidinedithiocarbamate, significantly blocked 7,8-dihydroneopterin-, H2O2- as well as anti-Fas-mediated apoptosis. These results imply that 7,8-dihydroneopterin-, H2O2-, and anti-Fas-mediated cell death might involve related redox sensitive signal transduction pathways.     

The causes of mortality following cystectomy for bladder tumor

The analysis of the treatment results is given for 183 patients with bladder cancer following cystectomy under various methods of urine derivation. Ureterocutaneostomy, Brickers operation, ureteral implantation into an isolated segment of the sigmoid, ureteral implantation into the colonic reservoir with controlled evacuation, radical cystectomy with ureteral implantation into an isolated rectal bladder with formation of sigmostoma, ureterosigmoanastomosis were performed in 94, 15, 10, 35, 12 and 17 patients, respectively. As shown by the follow-up available for 151 patients, 55 patients died of the tumor progression, 22 of renal complications, 11 of other causes. Within 2 postoperative years the disease progression and renal failure caused death in 65 (73.9%) out of 88 patients. The least lethality due to renal failure (3.2%) was recorded in patients with colonic reservoir.     

Bilateral ureteral inverted papilloma with synchronous transitional cell tumor of the bladder

A procedure for measuring the power of an ambient and goal-oriented equivalent scattered radiation dose in the radiation control of X-ray rooms. The procedure is based on the concept of the effective solid angle of a dosimeter directivity diagram. The plots of the angular response of a S2010 selective X-ray dosimeter for studying the radionuclides 119Sn, 241Am, 57Co are exemplified. The scaling factors for dosimetric reading for isotropic and nonuniform fields of radiation were calculated from the obtained values of effective angular solid angles (0.7, 0.8, 1.2 pi).