High Cyclin E Staining Index in Blastemal, Stromal or Epithelial Cells Is Correlated with Tumor Aggressiveness in Patients with Nephroblastoma

PURPOSE: Identifying among nephroblastoma those with a high propensity for distant metastases using cell cycle markers: cyclin E as a regulator of progression through the cell cycle and Ki-67 as a tumor proliferation marker, since both are often deregulated in many human malignancies. METHODOLOGY/PRINCIPAL FINDINGS: A staining index (SI) was obtained by immunohistochemistry using anti-cyclin E and anti-Ki-67 antibodies in paraffin sections of 54 postchemotherapy nephroblastoma including 42 nephroblastoma without metastasis and 12 with metastases. Median cyclin E and Ki-67 SI were 46% and 33% in blastemal cells, 30% and 10% in stromal cells, 37% and 29.5% in epithelial cells. The highest values were found for anaplastic nephroblastoma. A correlation between cyclin E and Ki-67 SI was found for the blastemal component and for the epithelial component. Univariate analysis showed prognostic significance for metastases with cyclin E SI in stromal cells, epithelial cells and blastemal cells (p = 0.03, p = 0.01 and p = 0.002, respectively) as well as with Ki-67 SI in blastema (p<10(-4)). The most striking data were that both cyclin E SI and blastemal Ki-67 SI discriminated between patients with metastases and patients without metastasis among intermediate-risk nephroblastoma. CONCLUSIONS: Our findings show that a high cyclin E SI in all components of nephroblastoma is correlated with tumor aggressiveness and metastases, and that assessment of its expression may have prognostic value in the categorization of nephroblastoma

Congenital anomalies of the male urethra

The spectrum of congenital anomalies of the male urethra is presented. The embryologic basis of each anomaly, when known, is discussed. Clinical and imaging features of each entity are presented

The influence of perioperative factors on primary severe hypospadias repair

Hypospadias is one of the most common congenital malformations of the male genitalia. Severe cases present with associated curvature greater than 30 degrees and the meatus opening proximally to the penoscrotal junction. The perioperative management of patients with primary severe hypospadias is variable. Systematic evaluation of the upper urinary tract and the search for enlarged prostatic utricles seem unnecessary in patients with isolated primary severe hypospadias, and should be limited to severe cases with associated extraurinary malformations. Detection of a disorder of sex development is key for gender assignment and prognosis, but the identification of cases warranting a full work-up and the influence of such a diagnosis on the success of hypospadias repair is controversial. Preoperative hormonal stimulation allows for penile growth irrespective of the administration route. Associated morbidity is minimal, but its influence on the success of surgery is still unknown. An age of 6-18 months is generally recommended for surgery, but no trial data support this policy. Second-layer coverage of the urethroplasty and postoperative urinary drainage seem to reduce the complications of surgery, whereas postoperative antibiotic prophylaxis and type of dressing have minimal impact on surgical success. Overall, most interventions are based on weak evidence, and their influence on the outcomes of repair is ill-defined. Clinicians should be made aware of the evidence supporting any single intervention in order to standardize their management policies. We hope the issues outlined here will prompt researchers to design new studies to address the clinically relevant questions

Surgical Management of Primary Severe Hypospadias in Children: Systematic 20-Year Review

Purpose: We systematically reviewed the literature published during the last 20 years on the treatment of primary proximal hypospadias associated with severe ventral curvature. Materials and Methods: We reviewed studies published between 1990 and December 2009, searching for "hypospadias" in MEDLINE (R)/PubMed (R), EMBASE (R), Web of Science (R) and the Cochrane Library. Results: The search yielded 69 pertinent studies. These studies were generally of low quality (69.5% surgical series). Based on the literature, curvature should be addressed stepwise starting with ventral dissection that extends underneath the urethral plate (urethral plate mobilization). Contrary to former practice, urethral plate division seems to have more of a role if significant curvature persists. Dorsal plication seems sufficient to correct only minor degrees of curvature, while a minority of cases require ventral lengthening. No urethroplasty techniques appear to be definitively superior. After urethral plate division a staged urethroplasty has lower complication rates but a second operation is required, which might otherwise be avoided in approximately 70% of cases. Conclusions: The present systematic review shows the weak evidence backing current management of primary severe hypospadias. We even lack a clear-cut definition of severe hypospadias and associated curvature. Hence, while we developed general recommendations for treatment based on our review of available evidence, we emphasize the need to establish shared criteria for accurate preoperative or introperative patient stratification, and to define objective outcome measures and followup intervals for data reporting to make comparison of surgical approaches reliable

Abstracts presented at the European Society for Pediatric Urology (ESPU) meetings (2003-2010): Characteristics and outcome

Objective: To determine the characteristics and outcome of abstracts presented to the meetings of the European Society for Pediatric Urology (ESPU). Material and methods: Abstract books from 2003 to 2010 were reviewed and subsequent publication of presented abstracts determined by MEDLINE/PubMed search. Results: Of 1194 abstracts, 50-78% per year originated from 15 to 20 European countries and 50-22% from 8 to 13 non-European countries; 233 (19%) were basic science and 961 (81%) clinical. Clinical abstracts included 135 (14%) multicenter/prospective/randomized trials. These figures did not change significantly over time. A total of 564 (47%) abstracts were subsequently published, 65% within 1 year of the meeting, mostly in the Journal of Urology (33%) and the Journal of Pediatric Urology (21%). Multicenter/prospective/randomized trials studies (OR 2.03; 95% CI 1.37-2.96) and abstracts originating from outside Europe (OR 1.61; 95% CI 1.26 -2.05) were significantly more likely to be subsequently published in full. Conclusion: The ESPU meetings are a true occasion for international exchange of scientific endeavors. Almost half of the abstracts are subsequently published. The Journal of Urology and the Journal of Pediatric Urology are consistently the two major target journals for publication. Non-European countries, irrespective of whether English-speaking or not, seem significantly more likely to publish their abstracts. (C) 2013 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved

Management of boys with nonpalpable undescended testis

Cryptorchidism is one of the most common genitourinary disorders in young boys. Although the management of boys with palpable testis is standardized, there are no formal guidelines for the management of boys with nonpalpable testis. In this Review we look at the current trends in the diagnosis and treatment of this disorder, as well as the indications for therapy and surgical procedures. On the basis of current evidence, we find that there is no optimum orchidopexy technique for the treatment of intra-abdominal testis, although it is preferable to adopt techniques that preserve the spermatic vessels. We also briefly examine the follow-up of patients with this disorder and its common complications. As yet, there are no data that assess the potential of laparoscopic orchidopexy being a risk factor for impaired fertility later in life

E-consultation for DSD: a global platform for access to expert advice

Electronic consultation (e-consultation) has been used for some years to facilitate communication between patients and their doctors, but it is also emerging as a valuable tool aiding communication between clinicians, both primary care and specialists, about a patient's care. Telehealth systems are being developed to provide video consultations to support diagnosis and management, as well as supporting clinical networks and health professional education. In some world regions, it seems increasingly likely that most rare diseases will be managed through a network of centres of expertise, and e-consultation systems may become a vital component of the service provided by these networks. Long-distance consultation across geographical and national boundaries has been used between colleagues in DSD for many years. However, the development of a robust and secure e-consultation service within the international DSD community appears to be timely. It will extend the current database and e-learning facilities, and should be achieved with the objective of providing expert opinion on a worldwide basis. It is proposed to install a steering committee to oversee the various practical, legal and cultural issues setting standards on data collection and exchange. The opportunity to broaden access to healthcare for all DSD patients and to widen discussion across the DSD community is valuable, and it is the view of the authors that this should be pursued and developed