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Context Urethral stricture management guidelines are an important tool for guiding evidence-based clinical practice. Objective To present a summary of the 2021 version of the European Association of Urology (EAU) guidelines on management of urethral strictures in females and transgender patients. Evidence acquisition The panel performed a literature review on these topics covering a time frame between 2008 and 2018 and used predefined inclusion and exclusion criteria for study selection. Key papers beyond this time period could be included as per panel consensus. A strength rating for each recommendation was added based on the review of the available literature and after panel discussion. Evidence synthesis Management of urethral strictures in females and transgender patients has been described in a few case series in the literature. Endoluminal treatments can be used for short, nonobliterative strictures in the first line. Repetitive endoluminal treatments are not curative. Urethroplasty encompasses a multitude of techniques and adaptation of the technique to the local conditions of the stricture is crucial to obtain durable patency rates. Conclusions Management of urethral strictures in females and transgender patients is complex and a multitude of techniques are available. Selection of the appropriate technique is crucial and these guidelines provide relevant recommendations. Patient summary Although different techniques are available to manage narrowing of the urethra (called a stricture), not every technique is appropriate for every type of stricture. These guidelines, developed on the basis of an extensive literature review, aim to guide physicians in selecting the appropriate technique(s) to treat a specific type of urethral stricture in females and transgender patients. Take Home Message Although different techniques are available to manage urethral strictures, not every technique is appropriate for every type of stricture. Management of urethral strictures in females and transgender patients is complex and a multitude of techniques are available. Selection of the appropriate technique is crucial and these guidelines provide relevant recommendations.

Introduction: Transgender health care interventions (e.g., gender-affirming surgery) support transgender and gender-diverse people to transition to their gender and are delivered in both centralized (by one interdisciplinary institution) and decentralized settings (by different institutions spread over several locations). In this exploratory study, we investigated the relationship between centralized and decentralized delivery of transgender health care, client-centeredness, and psychosocial outcomes. Methods: A retrospective analysis of 45 clients undergoing vaginoplasty at one medical center was conducted. Mann–Whitney U tests assessed differences regarding five dimensions of client-centeredness and psychosocial outcomes between the health care delivery groups. To address shortcomings regarding the small sample size, we applied a rigorous statistical approach (e.g., Bonferroni correction) to ensure that we only identified predictors that were actually related to the outcomes. Results: All aspects of client-centered care were scored average or high. Decentralized delivery of care was more client-centered in terms of involvement in care/shared decision-making and empowerment. However, participants from decentralized health care delivery settings scored lower on psychosocial health (p = 0.038–0.005). Conclusions: The factor of (de-)centralized health care delivery appears to have a significant impact on the provision of transgender health care and should be investigated by future research.

Purpose Previous research on genital gender-affirming surgery lacked to build a framework that took various surrounding factors into account. E.g., transgender health care services are delivered in both centralized (by one interdisciplinary institution) and decentralized settings (by different medical institutions spread over several locations). The present study investigated the effects of different structural and clinical aspects of gender-affirming genital surgery on psychosocial outcomes. Methods We surveyed former transgender and gender-diverse people who completed a vaginoplasty between 2014 and 2018. 45 participants were included in the study. We calculated hierarchical linear regression analyses to assess the relationship between psychosocial outcome measures (gender congruence, mental health, quality of life) and different aspects of gender-affirming genital surgery (e.g., setting of service delivery). To address shortcomings regarding the small sample size, we applied a rigorous statistical approach (e.g., Bonferroni correction) to ensure that we only identify predictors that are actually related to the outcomes. Results A non-responder analysis revealed no systematic bias in the recruitment procedure. Treatment satisfaction was a significant predictor for gender congruence. Moreover, we found the setting of service delivery (centralized, decentralized) to predict psychological health and the physical health dimension of quality of life. The effect sizes of our models were moderate to high, and models explained up to 26% of the total variance with a power up to 0.83. Conclusion The present study is an exploratory attempt into the manifold relationships between treatment-related factors (e.g., aesthetic outcome), the setting of service delivery, and their effects on gender-affirming genital surgery.

Zusammenfassung Einleitung Die Induratio penis plastica (IPP) ist eine erworbene chronische Erkrankung der Tunica albuginea und/oder des Septums der Corpora cavernosa, die zu Deformitäten und Verkrümmungen des Penis führen und die Sexualität der Betroffenen stark beeinträchtigen kann. Darüber hinaus kann die IPP bei den betroffenen Patienten zu emotionalen Problemen und zu Beziehungsproblemen führen. Forschungsziele Die vorliegende Übersichtsarbeit gibt einen Überblick über die konservativen und chirurgischen Therapieoptionen der IPP, geht detailliert auf die psychischen Besonderheiten und Probleme der Betroffenen ein und stellt mögliche psycho- und sexualtherapeutische Interventionen dar. Methoden Es handelt sich um eine nicht-systematische narrative Übersichtsarbeit. Mithilfe einer Pubmed-Datenbankanalyse wurden Original- und Übersichtsarbeiten zur IPP identifiziert und im Hinblick auf Methodik und Ergebnisse ausgewertet. Überlegungen zur interdisziplinären Integration einer unterstützenden Psychotherapie werden angestellt. Ergebnisse Die konservative und die chirurgische Therapie stellen keine kausale, sondern eine symptomatische Behandlung dar. Die Effektivität der konservativen Therapie ist eingeschränkt. Die chirurgische Therapie stellt den Goldstandard zur Behandlung der schweren IPP dar, kann aber Nebenwirkungen wie erektile Dysfunktion und Längenverlust des Penis zur Folge haben. Die IPP führt häufig zu psychischen Belastungen, die im Rahmen einer interdisziplinär integrierten Psychotherapie adressiert werden sollten. Ein entsprechend kombiniertes Vorgehen wird vorgeschlagen. Schlussfolgerung Um die Erwartungen an die konservative und chirurgische Therapie nicht zu überhöhen, müssen die Patienten über die Möglichkeiten und Limitationen realistisch und ausführlich aufgeklärt werden. Die psychischen Spezifika und Belastungen sollten bei der Therapie der IPP interdisziplinär integriert berücksichtigt werden.

The benefits and harms of the available types of surgical management for lichen sclerosus-related (LS) strictures remain unclear and, thus, clear and robust clinical practice recommendations cannot be given. To assess the role of single-stage OMGU in the management of LS strictures and explore how its benefits and harms compare with the alternative management options. Medline, Embase and Cochrane controlled trial databases (CENTRAL, CDSR) were systematically searched. Randomized (RCTs) and nonrandomized studies (NRCSs) comparing single-stage OMGU with other surgical management options for LS strictures and single-arm studies on single-stage OMGU were included. Risk of bias (RoB) was assessed. Of the 1912 abstracts identified, 15 studies (1 NRCS and 14 single-arm studies) were included, recruiting in total 649 patients. All studies were at high RoB. In the only NRCS available, stricture-free rate (SFR) for single-stage and staged OMGU was 88% vs 60%, respectively (p = 0.05), at a mean follow-up of 66.5 months. SFR range for single-stage OMGU in single-arm studies was 65–100% (mean/median follow-up, 12–59 months). Single-stage OMGU had low complication rates and beneficial impact on LUTS and QoL. The present SR highlights the methodological limitations of the available literature. In the absence of adverse local tissue conditions, and taking into consideration benefit–harm balance and surgeon’s skills and expertise, single-stage OMGU can be justified in patients with LS strictures.

Objective To present a summary of the 2021 version of the European Association of Urology (EAU) guidelines on management of male urethral stricture disease. Evidence acquisition The panel performed a literature review on these topics covering a time frame between 2008 and 2018, and used predefined inclusion and exclusion criteria for the literature to be selected. Key papers beyond this time period could be included as per panel consensus. A strength rating for each recommendation was added based on a review of the available literature and after panel discussion. Evidence synthesis Management of male urethral strictures has extensively been described in literature. Nevertheless, few well-designed studies providing high level of evidence are available. In well-resourced countries, iatrogenic injury to the urethra is one of the most common causes of strictures. Asymptomatic strictures do not always need active treatment. Endoluminal treatments can be used for short, nonobliterative strictures at the bulbar and posterior urethra as first-line treatment. Repetitive endoluminal treatments are not curative. Urethroplasty encompasses a multitude of techniques, and adaptation of the technique to the local conditions of the stricture is crucial to obtain durable patency rates. Conclusions Management of male urethral strictures is complex, and a multitude of techniques are available. Selection of the appropriate technique is crucial, and these guidelines provide relevant recommendations. Patient summary Injury to the urethra by medical interventions is one of the most common reasons of male urethral stricture disease in well-resourced countries. Although different techniques are available to manage urethral strictures, not every technique is appropriate for every type of stricture. These guidelines, developed based on an extensive literature review, aim to guide physicians in the selection of the appropriate technique(s) to treat a specific type of urethral stricture.

Urethral stricture management guidelines are an important tool for guiding evidence-based clinical practice.To present a summary of the 2021 European Association of Urology (EAU) guidelines on diagnosis, classification, perioperative management, and follow-up of male urethral stricture disease.The panel performed a literature review on the topics covering a time frame between 2008 and 2018, and using predefined inclusion and exclusion criteria for the literature. Key papers beyond this time period could be included if panel consensus was reached. A strength rating for each recommendation was added based on a review of the available literature after panel discussion.Routine diagnostic evaluation encompasses history, patient-reported outcome measures, examination, uroflowmetry, postvoid residual measurement, endoscopy, and urethrography. Ancillary techniques that provide a three-dimensional assessment and may demonstrate associated abnormalities include sonourethrography and magnetic resonance urethrogram, although these are not utilised routinely. The classification of strictures should include stricture location and calibre. Urethral rest after urethral manipulations is advised prior to offering urethroplasty. An assessment for urinary extravasation after urethroplasty is beneficial before catheter removal. The optimal time of catheterisation after urethrotomy is72 h, but is unclear following urethroplasty and depends on various factors. Patients undergoing urethroplasty should be followed up for at least 1 yr. Objective and subjective outcomes should be assessed after urethral surgeries, including patient satisfaction and sexual function.Accurate diagnosis and categorisation is important in determining management. Adequate perioperative care and follow-up is essential for achieving successful outcomes. The EAU guidelines provide relevant evidence-based recommendations to optimise patient work-up and follow-up.Urethral strictures have to be assessed adequately before planning treatment. Before surgery, urethral rest and infection prevention are advised. After urethral surgery, x-ray dye tests are advised before removing catheters to ensure that healing has occurred. Routine follow-up is required, including patient-reported outcomes. These guidelines aim to guide doctors in the diagnosis, care, and follow-up of patients with urethral stricture.

Context Four techniques for graft placement in one-stage bulbar urethroplasty have been reported: dorsal onlay (DO), ventral onlay (VO), dorsolateral onlay (DLO), and dorsal inlay (DI). There is currently no systematic review in the literature comparing these techniques. Objective To assess if stricture recurrence and secondary outcomes vary between the four techniques and to assess if one technique is superior to any other. Evidence acquisition The EMBASE, MEDLINE, and Cochrane Systematic Reviews—Cochrane Central Register of Controlled Trials (CENTRAL; Cochrane HTA, DARE, HEED) databases and ClinicalTrials.gov were searched for publications in English from 1996 onwards. Randomised controlled trials (RCTs), nonrandomised comparative studies (NRCSs), observational studies (cohort, case-control/comparative, single-arm), and case series with ≥20 adult male participants were included. Evidence synthesis A total of 41 studies were included involving 3683 patients from one RCT, four NRCSs, and 36 case series. Owing to the overall low quality of the evidence, a narrative synthesis was performed. Conclusions No single technique appears to be superior to another for bulbar free graft urethroplasty. Both DO and VO are suitable for bulbar augmentation urethroplasty, with a ≤20% recurrence rate over medium-term follow-up. No recommendations can be made regarding DI or DLO techniques owing to the paucity of evidence. Secondary outcomes including sexual function, and complications are infrequently reported. Recurrence rates deteriorate in the long term for both DO and VO procedures. Patient summary We reviewed the evidence for four different skin-graft techniques used to repair narrowing of a section of the urethra (bulbar urethra, under the scrotum and perineum) in men. Two of the techniques seem to give consistent results, with recurrence rates lower than 20%. Recurrence rates increase over time, so patients should continue to monitor their symptoms. There is poorer reporting of other outcomes such as sexual function, urinary symptoms, and complications, and it is possible that these occur more frequently than the current data suggest.

Posterior urethral injuries in men usually result from polytrauma with pelvic fractures and severe associated injuries. If left untreated, posterior urethral injury can lead to severe complications and even death, so precise diagnosis should be made when urethral injury is suspected. The cardinal symptom of urethral injury is blood leakage from the meatus. Retrograde urethrography is the diagnostic tool of choice. In the case of complete urethral avulsion, the formation of a urethral stricture due to pronounced scarring fibrosis is inevitable. In principle, various therapeutic approaches are available. Often, urinary diversion by means of a transurethral or suprapubic catheter is performed first. However, there is also the possibility of direct endoscopic or open urethral realignment with subsequent temporary catheterization. Immediate surgical reconstruction is useful only in exceptional cases of simultaneous bladder neck or rectal injury. In cases of complete urethral avulsion, secondary transperineal bulboprostatic reanastomosis at the interval after three months of suprapubic continuous drainage with excellent success rate is the gold standard and can be supplemented with ancillary maneuvers if necessary to ensure a tension-free anastomosis.Posteriore Harnröhrenverletzungen beim Mann gehen meist auf Polytraumata mit Beckenfrakturen und schweren Begleitverletzungen zurück. Unbehandelt kann eine posteriore Harnröhrenverletzung zu schweren Komplikationen bis hin zum Tod führen, so dass bei Verdacht auf Harnröhrenverletzung eine präzise Diagnostik erfolgen sollte. Das Kardinalsymptom einer Harnröhrenverletzung stellt Blutaustritt aus dem Meatus dar. Diagnostisches Mittel der Wahl ist die retrograde Urethrographie. Bei komplettem Harnröhrenabriss ist die Ausbildung einer Harnröhrenstriktur durch ausgeprägte, narbige Fibrosierung unvermeidbar. Grundsätzlich stehen verschiedene therapeutische Ansätze zur Verfügung. Häufig erfolgt zunächst eine Harnableitung mittels transurethralem oder suprapubischem Katheter. Es gibt jedoch auch die Möglichkeit einer direkten endoskopischen oder offenen Kontinuitätswiederherstellung mit anschließender passagerer Katheterableitung. Eine sofortige operative Rekonstruktion ist nur in Ausnahmefällen bei simultaner Blasenhals- oder Rektumverletzung sinnvoll. Bei vollständigen Harnröhrenabriss stellt die sekundäre transperineale, bulboprostatische Reanastomose im Intervall nach dreimonatiger suprapubischer Dauerableitung mit exzellenter Erfolgsrate den Goldstandard dar und kann bei Bedarf um Zusatzmanöver ergänzt werden, um eine spannungsfreie Anastomose zu gewährleisten.

Purpose To evaluate the interplay of stricture recurrence, sexual function, and treatment satisfaction after substitution urethroplasty. Methods Observational study of men undergoing 1-stage buccal mucosal graft urethroplasty for anterior urethral stricture between 2009 and 2016. Patients were dichotomized by self-reported treatment satisfaction. Sexual function was assessed by validated and non-validated patient-reported outcome measures. Functional recurrence was defined as symptomatic need of re-intervention. Bivariate analyses, Kaplan–Meier estimates, qualitative and quantitative analyses by uni- and multivariable regression were employed to evaluate the interplay of sexual function, functional recurrence, and treatment satisfaction. Results Of 534 men with bulbar (82%), penobulbar (11%), and penile strictures (7.3%), 451 (84%) were satisfied with the surgery. There were no differences in stricture location, previous treatment, graft length, or surgical technique between satisfied and unsatisfied patients (all p ≥ 0.2). Recurrence-free survival was 85% at a median follow-up of 33 mo and decreased significantly with each Likert item towards increasing dissatisfaction (p < 0.001). Dissatisfied patients more often reported postoperative loss of rigidity, tumescence, reduced ejaculatory volume, ejaculatory pain, and reduced penile length (all p ≤ 0.042). In 83 dissatisfied men, functional recurrence (28%) and oral morbidity (20%) were the main drivers of dissatisfaction in qualitative analysis. Multivariable analyses revealed functional recurrence and impaired postoperative ejaculatory function as independent predictors of treatment dissatisfaction (all p ≤ 0.029) after adjusting for confounders. Conclusion We found an association of both functional success and sexual function with patient-reported treatment satisfaction after substitution urethroplasty. Such findings validate the clinical significance of defining the symptomatic need for re-intervention as an endpoint and underline the importance of further research evaluating sexual function before and after open urethral reconstruction.

Nephrometry scores aid in clinical decision-making, yet evidence is scarce regarding their impact on cumulative morbidity following partial nephrectomy (PN).Retrospective, monocentric study of 122 patients with suspicious renal masses undergoing open or robot-assisted PN between January 2019 and August 2020. Morbidity assessment followed European Association of Urology guidelines on complication reporting. 30-d complications were extracted using a PN-specific catalog, were graded by the Clavien-Dindo classification (CDC), and Comprehensive Complication Index (CCI®) values were calculated. The impact of nephrometry on cumulative morbidity was evaluated by (1) comparing morbidity estimates between Preoperative Aspects and Dimensions Used for an Anatomical (PADUA)/R.E.N.A.L. complexity groups, (2) by Pearson's correlation between nephrometry scores and CCI®, and (3) by multivariable regression models using any 30-d complication and 30-d CCI® as endpoints.Of 122 patients, 101 (83%) underwent open and 21 (17%) robot-assisted PN. Median PADUA and R.E.N.A.L. scores were 9 (interquartile range, IQR 8-10) and 8 (IQR 6-9), respectively. Of 218 complications in 92 patients (75%), the majority was classified as minor (CDC grade ≤IIIa). Median 30-d CCI® was 8.7 (IQR 0.50-15). There was a small positive correlation between PADUA or R.E.N.A.L. score with CCI® (all P ≤ 0.026), explaining 4.7% and 4.1% of the variation in CCI®, respectively. After adjustment, nephrometry scores were associated with any 30-d complication and the CCI® (all P ≤ 0.011). PADUA and R.E.N.A.L. high complexity tumors were positively associated with both morbidity endpoints compared to low complexity tumors (all P ≤ 0.041).At a referral center, PN may be safely performed, even if morbidity assessment follows a strict protocol. Nephrometry risk classification does only marginally translate into clinical relevant differences regarding short-term complications. Thus, nephron-sparing surgery should not be withheld from patients with high complexity renal masses.

Zusammenfassung Hintergrund Geschlechtsdysphorie (GD, ehemals Transsexualität) bezeichnet einen Leidensdruck, der sich aus der Inkongruenz zwischen den körperlichen Geschlechtsmerkmalen und dem Erleben des eigenen Geschlechts ergeben kann (Geschlechtsinkongruenz: GIK). Die Gesundheitsversorgung von Personen mit GIK/GD bezieht sich v. a. auf geschlechtsangleichende Maßnahmen (z. B. Hormonbehandlung) und Unterstützung bei assoziierten Bedürfnissen (z. B. durch Psychotherapie). Zudem gelten genitalchirurgische Eingriffe als effektive Verfahren zur Reduktion von GIK/GD und zur Verbesserung der psychischen Gesundheit und Lebensqualität. Als evidenzbasiert gilt eine integrative Gesundheitsversorgung, bei der die beteiligten Bereiche interdisziplinär kooperieren. Ziel der Studie Bei der medizinischen Versorgung von Patient_innen mit GIK/GD lassen sich grundsätzlich zwei Settings unterscheiden: interdisziplinär integriert an einem Standort vs. dezentral verteilt über mehrere Standorte. Die Trans-Gesundheitsversorgung in Deutschland ist bisher weitgehend dezentral organisiert. Wie sich das Versorgungssetting auf die Wirksamkeit und Qualität genitalangleichender Operationen auswirkt, wurde bisher nicht untersucht. Ziel der TransCare-Studie ist es daher, prospektiv katamnestisch das psychosoziale und klinische Outcome von Patient_innen mit GIK/GD zu untersuchen, die eine feminisierende Genitaloperation in Anspruch nehmen, und auf die möglichen Auswirkungen des Versorgungssettings (interdisziplinär integriert vs. dezentral verteilt) hin zu vergleichen. Methodik Im Zuge eines naturalistischen Längsschnittdesigns soll eine möglichst praxisnahe Stichprobe rekrutiert und die teilnehmenden Patient_innen prospektiv befragt werden. Ergebnisse Die Studienergebnisse sollen Erkenntnisse über die Rolle des Versorgungssettings im Rahmen der genitalchirurgischen Behandlung von Patient_innen mit GIK/GD hervorbringen. Schlussfolgerung Auf Basis der TransCare-Studie soll die Versorgungsqualität empirisch fundiert verbessert werden.

Cadherin-16 (CDH16) is a member of the cadherin superfamily which is also termed kidney specific cadherin (ksp-cadherin) because of its preferred expression in the normal kidney where it plays a pivotal role in tubulus formation during embryonal development. However, little is known about the prognostic role of CDH16 in renal cell carcinomas.CDH16 protein was analyzed by immunohistochemistry in more than 1,300 renal cell carcinomas (RCCs) in a tissue microarray format.A CDH16 positivity was found in 81% of 1,115 interpretable tumors. Staining was weak in 34%, moderate in 32% and strong in 15% of tumors. CDH16 positivity was more common in oncocytomas (99% positive) and chromophobe carcinomas (94%) than in papillary (73%) and clear cell RCCs (82%; P0.0001). In clear cell RCC, reduced CDH16 staining was significantly linked to high-grade (P0.0001), advanced pT stage (P = 0.0452), distant metastasis (P = 0.0003) as well as to shortened recurrence free (P0.0001), overall (P = 0.0002), and tumor specific survival (P = 0.0092). In a multivariate analysis including pT, pN, ISUP grade, and M status, CDH16 expression loss was an independent prognostic parameter for overall (P = 0.0016) and tumor specific survival (P = 0.0218) in clear cell RCC. Unequivocal associations with tumor phenotype and prognosis were not seen in papillary RCC.Our data demonstrate that reduction of CDH16 expression is a strong and potentially clinically useful independent predictor of poor prognosis in clear cell RCC.

Eine auserhalb der normalen Position befindliche Niere wird als Nierendystopie oder Nierenektopie bezeichnet (Beckenniere, thorakale Niere). Eine gekreuzte renale Ektopie liegt vor, wenn der Ureter zum Ostium der Gegenseite kreuzt. Hierbei wird die Mittellinie uberquert. In 90 % der Falle besteht dabei eine zusatzliche Fusionsanomalie mit der ipsilateralen Niere (S-Niere, L-Niere); cave: abnormale Gefasversorgung. Eine Fusion ohne Ektopie stellt z. B. die Hufeisenniere oder Kuchenniere dar. In der Diagnostik steht die Sonographie an vorderster Stelle, das i.v. Urogramm hat nur beim Erwachsenen Bedeutung. Zusatzliche Untersuchungen wie Nierenfunktionsprufung oder MRT konnen je nach Symptomatik erforderlich sein.

Background Deletions of 17p13 recurrently occur in renal cell carcinoma (RCC) but their prognostic role seems to be uncertain. Methods To determine prevalence, relationship with tumor phenotype, and patient prognosis, a tissue microarray containing samples from 1809 RCCs was evaluated using dual labeling fluorescence in situ hybridization (FISH) with 17p13 and chromosome 17 centromere probes. Results A 17p13 deletion was found in 72 of 1429 interpretable tumors. The frequency of 17p13 deletions varied greatly between RCC subtypes and was highest in chromophobe RCC (24/72; 33.3%). 17p13 deletions were also found in 35 (3.7%) of 946 clear cell RCC, 9 (4.3%) of 208 papillary RCC, 1 of 121 oncocytomas (0.8%), as well as in several rare cases of comprising 1 of 7 Xp11.2 translocation cancers, 1 of 3 collecting duct carcinomas, and 1 of 20 not otherwise specified (NOS) carcinomas. In clear cell carcinomas, 17p13 deletions revealed a strong and consistent association with higher Fuhrman, ISUP, and Thoenes grade (p < 0.0001 each), and linked to advanced tumor stage (p = 0.0168), large tumor diameter (p = 0.0004), distant metastases (p = 0.0077), cancer-specific survival (p = 0.0391), and recurrence-free survival (p = 0.0072). In multivariate analysis, 17p13 deletions showed in clear cell RCC a dependent prognostic role for established clinical-pathological parameters. Conclusion 17p13 deletions have a dual role in RCC. They are associated with disease progression in clear cell RCC and possibly other subtypes and they are linked to the development of chromophobe RCC—a subtype with a particularly favorable prognosis.

Objectives To evaluate objective treatment success and subjective patient-reported outcomes in patients with radiation-induced urethral strictures undergoing single-stage urethroplasty. Patients and methods Monocentric study of patients who underwent single-stage ventral onlay buccal mucosal graft urethroplasty for a radiation-induced stricture between January 2009 and December 2016. Patients were characterized by descriptive analyses. Kaplan–Meier estimates were employed to plot recurrence-free survival. Recurrence was defined as any subsequent urethral instrumentation (dilation, urethrotomy, urethroplasty). Patient-reported functional outcomes were evaluated using the validated German extension of the Urethral Stricture Surgery Patient-Reported Outcome Measure (USS PROM). Results Overall, 47 patients were available for final analyses. Median age was 70 (IQR 65–74). Except for two, all patients had undergone pelvic radiation therapy for prostate cancer. Predominant modality was external beam radiation therapy in 70% of patients. Stricture recurrence rate was 33% at a median follow-up of 44 months (IQR 28–68). In 37 patients with available USS PROM data, mean six-item LUTS score was 7.2 (SD 4.3). Mean ICIQ sum score was 9.8 (SD 5.4). Overall, 53% of patients reported daily leaking and of all, 26% patients underwent subsequent artificial urinary sphincter implantation. Mean IIEF-EF score was 4.4 (SD 7.1), indicating severe erectile dysfunction. In 38 patients with data regarding the generic health status and treatment satisfaction, mean EQ-5D index score and EQ VAS score was 0.91 (SD 0.15) and 65 (SD 21), respectively. Overall, 71% of patients were satisfied with the outcome. Conclusion The success rate and functional outcome after BMGU for radiation-induced strictures were reasonable. However, compared to existing long-term data on non-irradiated patients, the outcome is impaired and patients should be counseled accordingly.

ZusammenfassungDer Hodenhochstand stellt eine der häufigsten Erkrankungen im Kindesalter dar und wird bei 3 – 8 % der männlichen Patienten diagnostiziert. Das Ziel der Therapie des Hodenhochstandes ist der Deszensus in den Hodensack innerhalb des ersten Lebensjahres, um anhaltende degenerative Veränderungen des Hodens zu vermeiden. Oft ist dazu eine operative Behandlung notwendig.Die chirurgische Therapie des tastbaren Hodens im Bereich der Leiste ist die Orchidopexie und bei nicht tastbaren Hoden ein laparoskopischer Ansatz mit ggf. inguinaler Freilegung. Die Orchidopexie für einen tastbaren Hoden kann durch einen traditionellen Leistenschnitt (inguinal) oder transskrotal behandelt werden. Der inguinale Zugangsweg gilt für die Orchidopexie als operative Methode der Wahl.

To describe the operative technique and report outcomes from the largest series of patients who underwent single-stage dorsal inlay buccal mucosal graft urethroplasty (BMGU) for isolated meatal stenoses and fossa navicularis strictures. First, we evaluated patients who underwent single-stage BMGU for distal urethral strictures (meatus and fossa navicularis) between 2009 and 2016 at our department. Clinical and surgical characteristics were prospectively collected in an institutional database. Recurrence was defined as symptomatic need of any instrumentation during follow-up, was retrospectively assessed by patient interview, and recurrence-free survival was plotted using Kaplan–Meier curves. Second, a systematic literature review was performed through Medline to summarize the available evidence on distal urethroplasty using flaps or grafts. Of 32 patients, 16 (50%) presented with a hypospadias-associated stricture, followed by seven (22%), five (16%), and four (13%) patients with iatrogenic, inflammatory, and congenital strictures, respectively. At a median follow-up of 42 months (IQR 23–65), single-stage dorsal inlay BMGU was successful in 22 patients (69%), and estimated recurrence-free survival rates were 79% and 74% at 12 and 24 months, respectively. Overall, 62 patients from five studies in the literature review underwent BMGU for isolated distal strictures and success rates ranged from 56 to 100%. Recurrent meatal stenoses and fossa navicularis strictures represent some of the most complex uro-reconstructive challenges. Inlay BMGU proves to be a valid and efficient last-resort single-stage technique. However, higher recurrence risk must be considered and staged urethroplasty should be discussed individually. Prospective randomized controlled trials are needed to prove the superiority of flaps, grafts or staged approaches over each other in this context.

Objective To analyze functional outcomes and complication rates of distal double cuffs (DC) or transcorporal cuffs (TC) as salvage approaches in high-risk patients, since there is an ongoing debate about optimal cuff-placement in a salvage setting (SV). Existing studies analyzing DC or TC are controversial with respect to functional outcomes and complication rates. Studies directly comparing both approaches in SV are scarce. Methods and Patients Prospective data collection was performed since 2009. DC/TC were applied according to a standardized protocol in SV. Salvage DC was chosen in case of a membranous single cuff explantation due to erosion or infection. TC were implanted after bulbar urethroplasty or DC explantation. Activation was performed 6 weeks postoperatively. Further follow-up was scheduled 6/24 months postoperatively and every 2 years thereafter. Primary/secondary endpoints were continence/complication rates. Results In total, 71 high-risk patients were available for analysis (58 DC, 13 TC). Median age was 70 years. Median follow-up was 24 months. Objective/social continence were 88%/94% in the DC and 72%/100% in the TC cohort, respectively (P = .37/P = 1). Overall, there were no significant differences with respect to infections, erosions, mechanical failure, and explantation rates. The times of explantation-free survival were similar in Kaplan-Meier analysis (Log-rank 0.399). Conclusion Complication and continence rates were not significantly different between both cohorts. Hence, a DC in SV can be considered as equally safe and effective. A sequential implantation (first DC, second TC) may be a viable approach to extend overall AUS incontinence therapy.

Urethral stricture management guidelines are an important tool for guiding evidence-based clinical practice.To present a summary of the 2021 version of the European Association of Urology (EAU) guidelines on management of urethral strictures in females and transgender patients.The panel performed a literature review on these topics covering a time frame between 2008 and 2018 and used predefined inclusion and exclusion criteria for study selection. Key papers beyond this time period could be included as per panel consensus. A strength rating for each recommendation was added based on the review of the available literature and after panel discussion.Management of urethral strictures in females and transgender patients has been described in a few case series in the literature. Endoluminal treatments can be used for short, nonobliterative strictures in the first line. Repetitive endoluminal treatments are not curative. Urethroplasty encompasses a multitude of techniques and adaptation of the technique to the local conditions of the stricture is crucial to obtain durable patency rates.Management of urethral strictures in females and transgender patients is complex and a multitude of techniques are available. Selection of the appropriate technique is crucial and these guidelines provide relevant recommendations.Although different techniques are available to manage narrowing of the urethra (called a stricture), not every technique is appropriate for every type of stricture. These guidelines, developed on the basis of an extensive literature review, aim to guide physicians in selecting the appropriate technique(s) to treat a specific type of urethral stricture in females and transgender patients. TAKE HOME MESSAGE: Although different techniques are available to manage urethral strictures, not every technique is appropriate for every type of stricture. Management of urethral strictures in females and transgender patients is complex and a multitude of techniques are available. Selection of the appropriate technique is crucial and these guidelines provide relevant recommendations.

Objectives To analyse functional outcomes and complication rates of artificial urinary sphincter (AUS) implantation with a distal bulbar double cuff (DC) for the treatment of stress urinary incontinence (SUI) in men with and without a history of external beam radiotherapy (RT). Patients and methods Data of all patients undergoing AUS implantation with a distal bulbar DC (DC-AUS) were collected prospectively from 2009 to 2015. Indications for DC implantation were based on urethral risk factors in terms of RT and previous proximal bulbar urethral interventions including, endoscopic or open surgery for urethral stricture or SUI. Implantation was carried out to a standardised protocol. Activation of the AUS was performed 6 weeks after implantation. Further follow-up (FU) included pad test, uroflowmetry, post-void residual urine measurements, radiography, and a standardised questionnaire. Continence and complication rates were compared between patients with a history of RT and non-RT patients. Explantation-free survival was estimated using Kaplan-Meier curves and the log-rank test. Firth's penalized Cox-regression analyses were performed to analyse proportional hazard ratios for explantation. Results In all, 150 men (median age 70 years, interquartile range [IQR] 66-74) after DC-AUS implantation were available for analysis. Overall, 73 men (48.7%) had a history of RT. The median (IQR) FU was 24 (7.25-36) months. Baseline clinical characteristics only differed regarding previous open SUI surgery (P = 0.016). Social and objective continence was achieved in 94.8% and 84.3% of all patients treated by implantation of a DC-AUS, respectively. Between the RT and non-RT patients there were no statistically significant differences in continence rates [social continence: 100% vs 90.2% (P = 0.194); objective continence: 87% vs 82% (P = 0.877)]. For complications rates there were no significant differences between RT and non-RT patients after DC-AUS implantation [infection (P = 0.09), erosion (P = 0.31), mechanical failures (P = 0.14)]. According to Kaplan-Meier analysis explantation rates in patients with a history of RT (26.0%) vs non-RT patients (20.8%), estimated explantation-free survival, and AUS durability, did not differ significantly (log-rank P = 0.219). Conclusion Our data from a large institutional series indicate DC-AUS implantation to be an effective and safe treatment strategy in men with SUI and a history of RT.

Background PD-L1 expression predicts response to immune checkpoint inhibitors in renal cell carcinomas (RCC), but has also been suggested to be linked to poor patient outcome. Methods We analyzed PD-L1 in > 1400 RCC in a tissue microarray format by immunohistochemistry. Results were compared with histological tumor type, parameters of cancer aggressiveness, and intratumoral CD8+ cytotoxic cells. Result At a cut-off level of 5% PD-L1 positive tumor cells, PD-L1 positivity was seen in 6.3% of 633 clear cell RCC (ccRCC), 18.2% of 165 papillary RCC, 18.8% of 64 chromophobe RCC, and 41.7% of 103 oncocytomas. In ccRCC, PD-L1 positivity was significantly linked to high ISUP (p p p p = 0.0042), short recurrence-free (p p = 0.0002). Intratumoral CD8+ lymphocytes were more frequent in PD-L1 positive (1055 ± 109) than in PD-L1 negative ccRCC (407 ± 28; p + cells (p p p = 0.0027), and Thoenes grade (p p = 0.0280). Conclusions These data suggest that PD-L1 expression in highly immunogenic RCCs facilitates immune evasion and contributes to cancer aggressiveness.

Gender Dysphoria (GD, formerly known as transsexualism) describes distress and impairment in an individual caused by the incongruence between the experienced gender and the sex assigned at birth (Gender Incongruence: GI). Transgender health care focusses on gender-affirmative treatments (e. g., hormone therapy) and associated needs (e. g., psychotherapy). Moreover, genital surgery is considered an effective treatment to reduce GI/GD and improve mental health and quality of life. Interdisciplinary cooperation between the associated medical facilities is regarded as evidence-based health care.To date, THC is delivered in zwei different health care settings: interdisciplinary and centralized from one medical institution vs. decentralized, spread over several medical institutions. In Germany, individuals with GI/GD access health care mostly in decentralized structures. The consequences of feminizing genital surgery carried out in centralized and decentralized health care structures in terms of quality and effectiveness have not been investigated so far. The goal of the TransCare study is to prospectively examine individuals with GI/GD seeking feminizing genital surgery regarding demographics and clinical characteristics as well as to analyze the influence of centralized and decentralized health care settings on the psychosocial and clinical outcome.To recruit a valid and comprehensive sample, participants were questioned prospectively.The results of the study should help gain new insights into the influence of centralized and decentralized health care settings carrying out feminizing genital surgery on psychosocial and clinical outcomes for the patients.Based on the TransCare study, we suggest that health care should be improved according to individual patient needs.Geschlechtsdysphorie (GD, ehemals Transsexualität) bezeichnet einen Leidensdruck, der sich aus der Inkongruenz zwischen den körperlichen Geschlechtsmerkmalen und dem Erleben des eigenen Geschlechts ergeben kann (Geschlechtsinkongruenz: GIK). Die Gesundheitsversorgung von Personen mit GIK/GD bezieht sich v. a. auf geschlechtsangleichende Maßnahmen (z. B. Hormonbehandlung) und Unterstützung bei assoziierten Bedürfnissen (z. B. durch Psychotherapie). Zudem gelten genitalchirurgische Eingriffe als effektive Verfahren zur Reduktion von GIK/GD und zur Verbesserung der psychischen Gesundheit und Lebensqualität. Als evidenzbasiert gilt eine integrative Gesundheitsversorgung, bei der die beteiligten Bereiche interdisziplinär kooperieren.Bei der medizinischen Versorgung von Patient_innen mit GIK/GD lassen sich grundsätzlich zwei Settings unterscheiden: interdisziplinär integriert an einem Standort vs. dezentral verteilt über mehrere Standorte. Die Trans-Gesundheitsversorgung in Deutschland ist bisher weitgehend dezentral organisiert. Wie sich das Versorgungssetting auf die Wirksamkeit und Qualität genitalangleichender Operationen auswirkt, wurde bisher nicht untersucht. Ziel der TransCare-Studie ist es daher, prospektiv katamnestisch das psychosoziale und klinische Outcome von Patient_innen mit GIK/GD zu untersuchen, die eine feminisierende Genitaloperation in Anspruch nehmen, und auf die möglichen Auswirkungen des Versorgungssettings (interdisziplinär integriert vs. dezentral verteilt) hin zu vergleichen.Im Zuge eines naturalistischen Längsschnittdesigns soll eine möglichst praxisnahe Stichprobe rekrutiert und die teilnehmenden Patient_innen prospektiv befragt werden.Die Studienergebnisse sollen Erkenntnisse über die Rolle des Versorgungssettings im Rahmen der genitalchirurgischen Behandlung von Patient_innen mit GIK/GD hervorbringen.Auf Basis der TransCare-Studie soll die Versorgungsqualität empirisch fundiert verbessert werden.

Purpose To analyze the perceived learning opportunities of participants of the International Meeting on Reconstructive Urology (IMORU) VIII for both live surgery demonstrations (LSD) and semi-live surgery demonstrations (SLSD). Safety and educational efficacy of LSD and SLSD at live surgery events (LSE) have been debated extensively, however, objective data comparing learning benefits are missing. Methods We conducted a detailed survey, which employed the Kirkpatrick model, a well-established assessment method of training models, to investigate participants preferences as well as the learning benefit of LSE. Furthermore, we employed an audience response system and the Objective Structured Assessment of Technical Skills (OSATS), a well-established assessment method of surgery skills, to let our participants rate the perceived learning opportunity of LSD and SLSD. Results Of 229 participants at the IMORU VIII, 39.7% returned our questionnaires. 90% stated that they prefer LSD. On all levels of Kirkpatrick’s training evaluation model, the IMORU received high ratings, suggesting a high learning benefit. For the assessment of OSATS, a total of 23 surgical cases were evaluable. For all six utilized items, LSD scored significantly better ratings than SLSD. Conclusion Our study suggests that there is still a rationale for LSD, as participants attributed a statistically significant higher learning benefit to LSD over SLDS. Evaluation of the survey showed that for LSE such as the IMORU VIII, a high learning benefit can be expected. Considering that most of our participants are active surgeons with high caseloads, their opinion on the educational value of LSE is of high relevance.

Objectives: The artificial urinary sphincter (AUS) is the gold standard treatment for severe stress urinary incontinence (SUI). According to the literature, patients suffering from Parkinson’s disease (PD) or stroke (ST) show adverse continence outcomes after prostate surgery and, therefore, constitute a challenging cohort for continence surgery. However, little is known with respect to the results of AUS surgery in these patients. A retrospective analysis of our institutional, prospectively maintained AUS database aims to address this aspect with a focus on surgical and functional outcomes. Methods and Patients: All data of patients with an AUS implantation were prospectively collected in our database since 2009. The AUS was implanted according to a standardized protocol and activated at 6 weeks postoperatively at our institution. Further follow-up (FU) consisted of pad-test, uroflowmetry, residual urine, and radiography as well as a standardized questionnaire including the Incontinence Quality of Life questionnaire (I-Quol) and International Consultation on Incontinence questionnaire (ICIQ-SF) and is scheduled at 6 and 24 months and every 2 years thereafter. Patients received a preoperative urodynamic evaluation (UD). Patients with normal voiding and storage function were considered for AUS implantation. All patients performed a preoperative test for manual dexterity. Patients with a history of ST or PD were grouped and compared to nonneurological patients. Primary/secondary endpoints of the study were complications/continence. Results: 234 patients were available for analysis. The median FU was 24 months (interquartile range 7–36). Twenty-four patients (10%) had a neurological history (PD and ST). Neurological patients showed significantly worse outcomes regarding continence (objective/subjective/social continence; p = 0.04/p = 0.02/p = 0.1). Significant differences concerning explantation rates were not observed (p = 1). Kaplan-Meier analysis showed no significant difference regarding explantation-free survival (log-rank p = 0.53). Conclusion: AUS implantation shows significantly worse continence rates for neurological patients, despite the fact that all patients showed normal UD results and sufficient manual dexterity. Although neurological patients showed worse outcomes for continence, AUS implantation seems to be a safe and viable treatment for patients with a history of neurological disease.

Background Little is known about patient-reported intensity and quality of pain at the receiver site as well as postoperative complications following one-stage buccal mucosal graft urethroplasty (BMGU). Objective To evaluate perineogenital pain intensity and quality as well as short-term complications after BMGU, and to describe the impact of pain and complications on stricture recurrence. Design, setting, and participants A secondary analysis of a randomized controlled trial including 135 patients, who underwent BMGU from 2014 to 2015, was performed. Intervention One-stage BMGU. Outcome measurements and statistical analysis Patient-reported perineogenital pain was assessed by the Numeric Pain Rating Scale and the Short-form McGill Pain Questionnaire preoperatively and at 1, 5, and 21 d, and 3, 6, and 12 mo postoperatively. Complications were assessed by the Clavien-Dindo classification and the Comprehensive Complication Index. Pain and complications were compared according to stricture recurrence. Results and limitations At a mean follow-up of 21 ± 15 mo, 29 patients (21%) had stricture recurrence. Pain intensity as well as sensory and affective pain quality decreased over time, reaching a minimum at 6 mo. Postoperative complications at days 5 (95% of patients) and 21 (27% of patients) were predominantly “minor” (Clavien-Dindo classification grade ≤ IIIa). Neither patient-reported perineogenital pain nor cumulative morbidity burden was different between patients with and those without stricture recurrence (all p ≥ 0.05). Conclusions Perineogenital pain is frequent after BMGU, but pain intensity and quality decrease over time. The same holds true for postoperative complications, which are frequent but mostly present as minor events. Current findings allow for thorough preoperative patient counseling regarding the expected perineogenital pain intensity and quality over time as well as complications following BMGU. Patient summary In this study, we looked at pain intensity and pain quality as well as complications following buccal mucosal graft urethroplasty. We found that pain in the perineogenital region is frequent, but pain intensity and quality decrease over time. Similarly, postoperative complications are frequent, but mainly present as minor events.

This consensus-based guideline was developed by all relevant German pediatric medical societies. Ultrasound is the standard imaging modality for pre- and postnatal kidney cysts and should also exclude extrarenal manifestations in the abdomen and internal genital organs. MRI has selected indications. Suspicion of a cystic kidney disease should prompt consultation of a pediatric nephrologist. Prenatal management must be tailored to very different degrees of disease severity. After renal oligohydramnios, we recommend delivery in a perinatal center. Neonates should not be denied renal replacement therapy solely because of their age. Children with unilateral multicystic dysplastic kidney do not require routine further imaging or nephrectomy, but long-term nephrology follow-up (as do children with uni- or bilateral kidney hypo-/dysplasia with cysts). ARPKD (autosomal recessive polycystic kidney disease), nephronophthisis, Bardet-Biedl syndrome and HNF1B mutations cause relevant extrarenal disease and genetic testing is advisable. Children with tuberous sclerosis complex, tumor predisposition (e. g. von Hippel Lindau syndrome) or high risk of acquired kidney cysts should have regular ultrasounds. Even asymptomatic children of parents with ADPKD (autosomal dominant PKD) should be monitored for hypertension and proteinuria. Presymptomatic diagnostic ultrasound or genetic examination for ADPKD in minors should only be done after thorough counselling. Simple cysts are very rare in children and ADPKD in a parent should be excluded. Complex renal cysts require further investigation.Die Leitlinie wurde im Konsens aller relevanten deutschen Fachgesellschaften zusammen mit Patientenvertretern erstellt. Standard der prä- und postnatalen Bildgebung von Nierenzysten ist die Sonografie, die auch im Abdomen und inneren Genitale extrarenale Manifestationen ausschließen soll. Die MRT hat einzelne Indikationen. Bei Verdacht auf zystische Nierenerkrankungen ist eine kindernephrologische Vorstellung indiziert. Die pränatale Betreuung muss auf sehr unterschiedliche Schweregrade zugeschnitten werden. Bei renalem Oligohydramnion wird eine Entbindung in einem Perinatalzentrum der höchsten Stufe empfohlen. Neugeborenen sollte eine Nierenersatztherapie nicht allein aufgrund des Alters vorenthalten werden. Bei unilateraler multizystischer Nierendysplasie ist keine funktionelle Bildgebung oder Nephrektomie notwendig, aber (wie auch bei uni- oder bilateraler Nierenhypo-/dysplasie mit Zysten) eine langfristige nephrologische Überwachung. Bei der ARPKD (autosomal rezessive polyzystische Nierenerkrankung), Nephronophthise, Bardet-Biedl-Syndrom und HNF1B-Mutationen müssen extrarenale Manifestationen beachtet werden; eine genetische Testung ist hier sinnvoll. Kinder mit tuberöser Sklerose, Tumorprädispositionen (z. B. von Hippel Lindau Syndrom) oder hohem Risiko für erworbene Nierenzysten sollten regelmäßige Nierensonografien erhalten. Auch asymptomatische Kinder von Eltern mit ADPKD (aut. dominanter polyzystischer Nierenerkrankung) sollten regelmäßig auf Hypertonie und Proteinurie untersucht werden. Eine präsymptomatische sonografische oder genetische Diagnostik dieser Minderjährigen sollte nur nach ausführlicher Aufklärung erwogen werden. Einfache (isolierte) Zysten sind bei Kindern sehr selten und eine ADPKD eines Elternteils sollte ausgeschlossen sein. Komplexe Nierenzysten bedürfen weiterer Abklärung.

INTRODUCTION AND OBJECTIVE:Although radical cystectomy (RC) is increasingly indicated in the geriatric patient, evidence regarding comparative morbidity after different diversions is scarce and mos...

Cryptorchism is one of the most frequent diagnoses in early childhood with an incidence of up to 3 - 8 %. The goal of any treatment is scrotal positioning of the testis until the end of the first year of life to prevent degenerative changes of the testis. This often requires a surgical procedure. The surgical treatment for a palpable testis in the inguinal area is orchidopexy, combined with laparoscopy in case of a non-palpable testis. Orchidopexy can be done via inguinal or scrotal access with inguinal access being the gold standard.Der Hodenhochstand stellt eine der häufigsten Erkrankungen im Kindesalter dar und wird bei 3 – 8 % der männlichen Patienten diagnostiziert. Das Ziel der Therapie des Hodenhochstandes ist der Deszensus in den Hodensack innerhalb des ersten Lebensjahres, um anhaltende degenerative Veränderungen des Hodens zu vermeiden. Oft ist dazu eine operative Behandlung notwendig.Die chirurgische Therapie des tastbaren Hodens im Bereich der Leiste ist die Orchidopexie und bei nicht tastbaren Hoden ein laparoskopischer Ansatz mit ggf. inguinaler Freilegung. Die Orchidopexie für einen tastbaren Hoden kann durch einen traditionellen Leistenschnitt (inguinal) oder transskrotal behandelt werden. Der inguinale Zugangsweg gilt für die Orchidopexie als operative Methode der Wahl.

Die Leitlinie wurde im Konsens aller relevanten deutschen Fachgesellschaften zusammen mit Patientenvertretern erstellt. Standard der pra- und postnatalen Bildgebung von Nierenzysten ist die Sonografie, die auch im Abdomen und inneren Genitale extrarenale Manifestationen ausschliesen soll. Die MRT hat einzelne Indikationen. Bei Verdacht auf zystische Nierenerkrankungen ist eine kindernephrologische Vorstellung indiziert. Die pranatale Betreuung muss auf sehr unterschiedliche Schweregrade zugeschnitten werden. Bei renalem Oligohydramnion wird eine Entbindung in einem Perinatalzentrum der hochsten Stufe empfohlen. Neugeborenen sollte eine Nierenersatztherapie nicht allein aufgrund des Alters vorenthalten werden. Bei unilateraler multizystischer Nierendysplasie ist keine funktionelle Bildgebung oder Nephrektomie notwendig, aber (wie auch bei uni- oder bilateraler Nierenhypo-/dysplasie mit Zysten) eine langfristige nephrologische Uberwachung. Bei der ARPKD (autosomal rezessive polyzystische Nierenerkrankung), Nephronophthise, Bardet-Biedl-Syndrom und HNF1B-Mutationen mussen extrarenale Manifestationen beachtet werden; eine genetische Testung ist hier sinnvoll. Kinder mit tuberoser Sklerose, Tumorpradispositionen (z. B. von Hippel Lindau Syndrom) oder hohem Risiko fur erworbene Nierenzysten sollten regelmasige Nierensonografien erhalten. Auch asymptomatische Kinder von Eltern mit ADPKD (aut. dominanter polyzystischer Nierenerkrankung) sollten regelmasig auf Hypertonie und Proteinurie untersucht werden. Eine prasymptomatische sonografische oder genetische Diagnostik dieser Minderjahrigen sollte nur nach ausfuhrlicher Aufklarung erwogen werden. Einfache (isolierte) Zysten sind bei Kindern sehr selten und eine ADPKD eines Elternteils sollte ausgeschlossen sein. Komplexe Nierenzysten bedurfen weiterer Abklarung.

Nierenzellkarzinome stellen eine seltene Erkrankung im Kindesalter dar. Dennoch sollten sie differentialdiagnostisch bei Nachweis einer renalen Raumforderung in Betracht gezogen werden. Die Fragestellung bezieht sich auf die Inzidenz und Mortalitat von Nierenzellkarzinomen im Kindesalter sowie die Darstellung der klinischen Symptome, Diagnostik, Histologie, Therapie und Verlauf der Erkrankung. Eigene Daten einer kleinen Patientenkohorte von 8 Patienten werden ausgewertet, sowie der aktuelle Literatur zu dieser Tumorentitat diskutiert. Der durchschnittliche Nachbeobachtungszeitraum lag bei 27 Monaten. Praoperative Staginguntersuchungen zeigten keine Metastasen. Der histologisch primar vorherrschende Subtyp war das papillare Nierenzellkarzinom. Tumorstadium und Lymphknotenstatus waren die wichtigsten Prognosefaktoren in unserer Studie.