[Klippel-Trénaunay syndrome. A rare cause of recurrent macrohematuria: case report]. / Klippel-Trenaunay-Syndrom. Eine seltene Ursache für rezidivierende Makrohämaturie - kasuistische Falldarstellung.

A 37-year-old man with Klippel-Trénaunay syndrome presented with an episode of painless severe gross hematuria. Magnetic resonance imaging (MRI) revealed vessels of significant diameter in the bladder wall. Diagnostic imaging is mandatory in order to be aware of the extent of the lesion as the bleeding identified intraoperatively may only be the "tip of the iceberg." If conservative means fail, laser coagulation should be the treatment of choice.

[Association of a positive family history with histopathology and clinical course in early-onset prostate cancer]. / Assoziation von familiärem Status mit Histologie und klinischem Verlauf beim frühen Prostatakarzinom.

BACKGROUND: In a large number of studies a positive family history is documented as one of the main risk factors for the development of prostate cancer. In a US population an association between early-onset prostate cancer among familial patients and a more differentiated tumour was shown. The aim of this study was to compare clinical parameters between sporadic and familial or hereditary patients with an age at diagnosis < or =55 years. MATERIAL AND METHODS: The clinical data of prostate cancer patients with an age at diagnosis < or =55 years and who were recruited between July 1999 and the end of June 2004 to the database "familial prostate cancer in Germany" were analysed. The following data were documented for all patients: PSA at diagnosis, histopathological stage, grading, Gleason score and progression-free survival. RESULTS: The clinical data of 685 patients could be completed: 222 (32.4%) had one first-degree relative with prostate cancer, 48 of whom (7.0%) were hereditary; 463 (67.6%) were sporadic. The median age at diagnosis in the hereditary patients was 51.6 (41-55) years, in the familial patients 51.1 (35-55) years and in the sporadic patients 52.0 (38-55) years. The median follow-up was 24 months in hereditary, 36 months in familial and 35 months in sporadic patients. An initial curative therapy with radical prostatectomy or radiotherapy/brachytherapy was planned in 657/685 (95.9%) of the patients. There were no clear differences regarding PSA at diagnosis, the postoperative parameters (organ-confined disease, lymph node involvement, Gleason score, grading) and the progression-free survival in sporadic and familial or hereditary patients. CONCLUSIONS: Patients with an age at diagnosis < or =55 years have a positive family history more often than all prostate cancer patients in Germany. No association could be shown between pathohistological stage or clinical course and a positive family history in patients with an age at diagnosis < or =55 years.

[Prospective evaluation of prostate cancer screening in men with a family history of the disease]. / Prospektive Evaluierung der Prostatakarzinomfrüherkennung bei Männern mit familiärer Disposition.

BACKGROUND: Family history is one of the strongest risk factors for prostate cancer. In this prospective study we evaluated the results of prostate cancer screening performed in healthy brothers of prostate cancer patients. The detection rate of prostate cancer and the positive predictive value of the examinations were determined. MATERIAL AND METHODS: The study population comprised 513 healthy men who were 38-75 years of age (median 62.0 years). Of these men, 268 having only one affected brother with prostate cancer were assigned to the sporadic group, and 245 probands having 2-10 affected relatives were assigned to the familial group. An abnormal PSA and/or a pathological digital rectal examination (DRE) was noted in 17.5% of familial (43/245) and 15.8% of sporadic probands (35/268). A biopsy of the prostate was performed in 60.5% of familial (26/43) and 71.4% of sporadic (25/35) men with pathological findings. RESULTS: Prostate cancer was found in 15 of 26 familial (57.7%) and 16 of 25 sporadic (64.0%) probands by prostate biopsy. The overall detection rate was 6.0% (31/513). CONCLUSION: Due to an increased prevalence the detection rate of prostate cancer and the positive predictive value of PSA and/or DRE are higher in men with a family history as expected in an unselected population. Our data suggest that in predisposed men prostate cancer screening should be recommended early. Furthermore an early indication for prostate biopsy is necessary. This recommendation should also be applied if only one first-degree relative has prostate cancer.

Radiosensitivity detected by the micronucleus test is not generally increased in sporadic prostate cancer patients.

The micronucleus test (MNT) has shown increased micronuclei (MN) frequencies in BRCA associated and sporadic breast cancer patients, Ataxia telangiectasia and Nijmegen Breakage Syndrome patients, demonstrating a common cellular phenotype of increased radiosensitivity. Some genes, causative of these diseases, have also recently been associated with prostate cancer. In order to investigate if prostate cancer exhibits the cellular phenotype of increased radiosensitivity, we performed MNT analysis on 22 sporadic prostate cancer patients and 43 male controls. We determined the baseline MN frequency, in order to see in vivo chromosomal damage without radiation, and induced (after irradiation with 2 Gy) frequency of MN, both in binucleated cells (BNC) obtained from cultured peripheral blood lymphocytes. An automated image analysis system was used to score the MN employing two different classifiers (Classifier A and B) for detection of BNC. The mean baseline frequencies were 48/43 MN/1000 BNC (A/B) for the controls and 42/50 (A/B) for prostate cancer patients. The induced MN frequencies amounted to 107/111 MN/1000 BNC (A/B) for controls and 111/114 MN/1000 BNC (A/B) for prostate cancer patients. The obtained MN frequencies did not result in a statistically significant difference between unselected cases and controls. However, restricting the analysis to young patients (50-60 years, N = 7) and age-matched controls (N = 7) revealed marginally significant higher MN frequencies in patients. We conclude that increased radiosensitivity is not a property of prostate cancer patients in general.

[Is the prostate cancer screening behaviour of men with familial predisposition predictable?]. / Ist das Vorsorgeverhalten bei Männern mit familiärer Prädisposition für ein Prostatakarzinom vorhersehbar?

Little is known about the motives of German men to attend or refuse preventive checkups for prostate cancer. The aims of this study were to investigate if in men with familial predisposition screening behaviours are influenced by epidemiological or clinical parameters of prostate cancer of their affected relatives. 476 probands with one and 312 probands with at least two affected relatives were advised in writing to have a PSA-test and DRE done at their local urologists. We evaluated if the response rate was correlated to the proband's age, to the number and the age of onset of their affected relatives and also to the clinical course of their disease. Our data implicate that in men with familial predisposition the acceptance of prostate cancer screening is influenced only by individual characteristics and personal attitude and not by factors within the family. To which extent the awareness of disease risk is modified by familial predisposition remains to be evaluated.

(CAG)nCAA and GGN repeats in the human androgen receptor gene are not associated with prostate cancer in a French-German population.

Alleles of the CAG and the GGC repeat in the first exon of the human androgen receptor (AR) gene have been shown to be associated with the risk of (advanced) prostate cancer. These studies had been carried out in the United States. We have analysed these polymorphisms in a French-German collection of 105 controls, 132 sporadic cases, and a sample of prostate cancer families comprising 85 affected and 46 not affected family members. The allele distributions were very similar in all four groups and chi square statistics on contingency tables did not detect any significant differences. The relative risk (odds ratio, OR) were calculated using logistic regression and did not reach significance despite sufficient numbers of patients and controls. Typical results were OR = 1.007; 95% Confidence Interval (CI) 0.97-1.1, P = 0.87 for CAG as continuous variable and OR = 1.2 (95% CI 0.7-2.0), P = 0.47 for CAG classes < 22 and > = 22 repeats. Similar results were obtained for subgroups defined by age or Gleason score. We conclude that these polymorphisms can not be used as predictive parameters for prostate cancer in the French or German population.

[Ileal neobladder with anastomosis to the female urethra]. / Die Ileumneoblase mit Anschluss an die weibliche Harnröhre.

Orthotopic reconstruction to the native urethra has revolutionized urinary diversion, allowing patients to void per the urethra. This form of urinary diversion was initially performed solely in male patients after cystectomy. More recently, however, with a better understanding of the female continence mechanism, including the urethral/vaginal support mechanism, and the ability to select appropriate female candidates properly for this type of surgery, orthotopic reconstruction has become a viable option in women. Since November 1986, 24 women aged 53 years (range 17-76) have undergone orthotopic reconstruction using the ileal neobladder. Indications for cystectomy included transitional cell carcinoma of the bladder (8), fibrotic radiated bladder (4), interstitial cystitis (5), tuberculotic bladder (2), urge incontinence (2), neurogenic fibrotic bladder (2), and fibrotic bladder of unknown etiology (1). Nineteen patients are available with a median follow-up of 48 months (range 3 to 109 months). There were no perioperative deaths, with few early and late complications. Two women previously irradiated developed a neovesicovaginal fistula and had to be diverted by an ileal loop. Three patients from the far East are no longer available for follow-up. Ten years of experience with 24 patients have led to a nerve- and urethral-support-sparing cystectomy technique with the ileal neobladder anastomosed to the proximal urethra. However, even then, retention in 20% of the patients rather than the expected incontinence is the critical issue. Incontinence has never been a problem. The advent of orthotopic lower urinary reconstruction in women is a major achievement in the evolution of urinary diversion. With our increasing understanding of the continence mechanism in women and with increasing evidence that the female urethra can be safely preserved after cystectomy, orthotopic lower urinary tract reconstruction by the ileal neobladder can now be offered safely not only to males, but also to female patients undergoing cystectomy, and the functional results are superb.

[Extracorporeal shockwave lithotripsy in children. Complications and long-term results]. / Extrakorporale Stosswellenlithotripsie bei Kindern. Komplikationen und Langzeitergebnisse.

ESWL of urinary stones is a well-established treatment in adult patients. The treatment of urinary stones in children has gained increasing importance in recent years. From 1987 to 1993, a series of 27 children with urolithiasis in all parts of the urinary tract were treated by ESWL. Treatment was performed with general anaesthesia or analgosedation. During the treatment no complications occurred. On average, 34 sessions of ESWL, with 2165 shockwaves and 22.3 kV energy, were performed. Minor early complications, such as fever, pain and hydronephrosis, were observed in 7 patients. The overall stone clearance rate was 92%. Stone recurrence occurred in only 1 patient. There were no late complications, such as malfunction of the kidney, skeletal deformation or hypertension after 38 months of follow up. In conclusion, ESWL is the treatment of first choice in paediatric urolithiasis.

[Familial prostate carcinoma in Germany]. / Das familiäre Prostatakarzinom in Deutschland.

Epidemiologic studies have shown that hereditary forms account for approximately 10% of all prostate cancers. The identification of several susceptibility loci harboring predisposing genes indicates the genetic heterogeneity of prostate cancer. The conflicting results of different linkage analyses may be explained by a varying contribution of each locus within different family collections and reflect differences of allele frequencies across different populations. In the present study we recorded the incidence of familial prostate cancer in Germany and performed descriptive analysis of the epidemiological data. In spite of a significant ascertainment bias, only 19% of all prostate cancers were familial. In 94% of families there were three affected relatives at most. Large prostate cancer families with at least five affected persons were rare (2%). Descriptive analysis revealed that only 42% of all pedigrees followed an autosomal-dominant pattern of transmission; the other pedigrees showed an X-chromosomal or recessive mode of inheritance. These data confirm the genetic heterogeneity of hereditary prostate cancer and imply that previously published epidemiological data cannot be transferred to the German population.

[Preventing prostate carcinoma in men with familial disposition]. / Prostatakarzinomvorsorge bei Männern mit familiärer Disposition.

A family history is one of the strongest risk factors for prostate cancer (PC). We evaluated the detection rate of PC in relatives of 119 German PC families that took part in ongoing linkage analyses. Brothers of patients with sporadic prostate cancer aged < 55 years at onset were included as well. Responses were received from 120/196 (61.2%) individuals of the familial and 67/120 (55.8%) of the sporadic group. Findings (DRE, TRUS, PSA) were more often suspicious for carcinoma in the PC families. Prostate cancer was diagnosed in 6 (5.0%) and 2 (2.99%) participants of the familial and the sporadic group, respectively. These detection rates tended to be higher than that of an age-matched subgroup of an unselected population in other European screening studies. The most important risk factor for the diagnosis of PC was a low average age at onset within the family. These data imply that prostate cancer screening in the high-risk group of men with familial predisposition cannot be assessed by population-based studies and should be evaluated separately.

[Familial versus sporadic prostate cancer in the German population. Clinical and pathological characteristics in patients after radical prostatectomy]. / Familiäres versus sporadisches Prostatakarzinom in der deutschen Population. Klinische und pathologische Charakteristika bei Patienten nach radikaler Prostatektomie.

Family history is one of the strongest epidemiological risk factors for the development of prostate cancer. The impact on the clinical presentation and prognosis, however, is controversial. In the present study, we analyzed 464 familial and 492 sporadic prostate cancer patients following radical prostatectomy. The average age at onset was 62.1 years in the familial group and 64.2 years in the sporadic controls (p<0.001). The screening attitude, DRE findings and the PSA values at diagnosis the pT- and pN-stages, and the tumor grade did not differ between both groups. With a median follow-up of 3.3 years, the 5- and 10-year progression-free survival rates were 76.2% and 56.5% in familial and 70.8% and 55.5% in sporadic patients, respectively (n.s.). A multiple logistic regression analysis revealed that family history did not have an influence on disease recurrence. In our population there was no association between a familial predisposition and clinical features or clinical course of the disease. Whether hereditary prostate cancer is distinct from sporadic forms cannot be determined before the underlying genetic alterations are identified.

[Genetic analysis of familial prostatic cancer: localization of a gene predisposing to prostatic cancer (PCaP) on chromosome 1q 42.2-43]. / Analyse génétique du cancer de la prostate familial: localisation d'un gène prédisposant au cancer de la prostate (PCaP) sur le chromosome 1q 42.2-43.

OBJECTIVES: To conduct genetic linkage analysis in order to localize predisposition genes for hereditary prostate cancer (CaP), as various epidemiological studies have demonstrated a family aggregation in 15 to 25% of cases, and the development of hereditary forms in 5 to 10% of cases of CaP. MATERIAL AND METHODS: A genetic study on 47 French and German families included 122 patients and 72 subjects considered to be healthy after PSA assay. This study was conducted by linkage analysis of 364 microsatellite markers distributed throughout the genome (on average every 10 cM). RESULTS: Parametric and nonparametric linkage analysis identified a locus on chromosome 1q 42.2-43, which could be with a gene predisposing to CaP (called PCaP). The primary site was confirmed by several markers, using 3 different genetic models. The maximum LOD score (probability of linkage between the locus and the disease) on two-point analysis was 2.7 for the D1S2785 marker. Parametric and nonparametric multipoint analysis provided an HLOD score and an NPL score of 2.2 and 3.1, respectively (with P = 0.001). Heterogeneity analysis with calculations of LOD scores by multipoint analysis estimated that up to 50% of hereditary CaPs were related to this locus, with a heterogeneity probability of 157/1. Analysis of a subgroup of 9/47 families characterized by early onset CaP (before the age of 60 years) confirmed the very high probability of localization of a predisposition gene at locus 1q42.2-43 for these families (multipoint LOD score and NPL score of 3.31 and 3.32, respectively; with P = 0.001). CONCLUSION: The identification of predisposition genes will eventually allow identification within certain families of those subjects who have inherited the genetic abnormality and who therefore present a high risk of CaP. It will then be possible to perform targeted screening of CaP in order to diagnose CaP as early as possible.

Mutation screening and association study of RNASEL as a prostate cancer susceptibility gene.

To date, germline mutations have been found in three candidate genes for hereditary prostate cancer: ELAC2 at 17p11, RNASEL at 1q25 and MSR1 at 8p22. RNASEL, encoding the 2',5'-oligoadenylate-dependant RNase L, seems to have rare mutations in different ethnicities, such as M1I in Afro-Americans, E265X in men of European descent and 471delAAAG in Ashkenazi Jews. In order to evaluate the relevance of RNASEL in the German population, we sequenced its open reading frame to determine the spectrum and frequency of germline mutations. The screen included 303 affected men from 136 Caucasian families, of which 45 met the criteria for hereditary prostate cancer. Variants were analysed using a family-based association test, and genotyped in an additional 227 sporadic prostate cancer patients and 207 controls. We identified only two sib pairs (1.4% of our families) cosegregating conspicuous RNASEL variants with prostate cancer: the nonsense mutation E265X, and a new amino-acid substitution (R400P) of unknown functional relevance. Both alleles were also found at low frequencies (1.4 and 0.5%, respectively) in controls. No significant association of polymorphisms (I97L, R462Q and D541E) was observed, neither in case-control analyses nor by family-based association tests. In contrast to previous reports, our study does not suggest that common variants (i.e. R462Q) modify disease risk. Our results are not consistent with a high penetrance of deleterious RNASEL mutations. Due to the low frequency of germline mutations present in our sample, RNASEL does not have a significant impact on prostate cancer susceptibility in the German population.

Does the option of the ileal neobladder stimulate patient and physician decision toward earlier cystectomy?

PURPOSE: The primary goal of bladder replacement is to attempt to improve patient quality of life, not to increase survival, affect cancer prognosis or decrease renal metabolic complications. Nevertheless, we retrospectively determined whether orthotopic bladder replacement has an impact on the decision to perform cystectomy. MATERIALS AND METHODS: From April 1986 to September 1994, 213 men a mean of 63 years old with stages pT2N0M0 to pT4N0M0 invasive bladder cancer were referred to our department for cystectomy. For 135 patients who underwent an ileal neobladder procedure and 78 who underwent conduit diversion median followup was 4.8 and 3.5 years, respectively. We evaluated the interval from the primary diagnosis of bladder cancer to cystectomy as well as the number of previous transurethral bladder resections. The 5-year cancer specific survival rates were calculated using the Kaplan-Meier method. The Wilcoxon and log rank tests, and the Cox proportional hazards model were used to determine statistical significance. RESULTS: In the neobladder and conduit groups an average of 2.1 (range 1 to 18) and 4.1 (range 1 to 15) transurethral bladder resections was performed, respectively. Interval from the primary diagnosis to cystectomy was 11.8 months in the neobladder and 16.7 months in the conduit group. Cystectomy was performed 4.1 months after the diagnosis of invasive cancer in the neobladder group, whereas radical surgery was delayed for 15.4 months in the conduit group. Cancer specific 5-year survival rates were 76.6 and 28.35% in the neobladder and conduit groups, respectively. After stratifying according to tumor stage the 5-year survival rate was significantly higher for all disease stages in the neobladder than in the conduit group. The proportional hazards model revealed that this difference was not due to patient age at disease stages pT3bN0 and pT4N0 or by American Society of Anesthesiologists score. Independent prognostic factors for survival were diversion type and age. Delayed cystectomy was a risk factor only in advanced disease stages. CONCLUSIONS: These data suggest that the ileal neobladder may decrease physician reluctance to perform cystectomy early in the disease process, increasing the survival rate. They also demonstrate that the ileal neobladder option significantly affects an earlier patient and physician decision in favor of cystectomy.

The ileal neobladder--updated experience with 306 patients.

From April 1986 through May 1995, 306 men with primary urothelial carcinoma underwent radical cystoprostatectomy and orthotopic bladder substitution via the ileal neobladder. Altogether, 7.5% of the patients suffered general early complications, including thrombosis, embolism, wound infection, and pneumonia. Specific early complications directly related to formation of the neobladder and requiring surgery included ileus (4%), abscess drainage (2%), and leakage of the ileal anastomosis (0.5%). The early reoperation rate was 6.5%. Early complications that required temporary percutaneous drainage were lymphocele formation (3%) or ureteral obstruction (6%). In all, 9% of our patients required prolonged catheter drainage for leakage of the ileouretheral anastomosis. Late complications requiring reoperation were ileus (2%), abscess drainage (1%), neobladder fistula to the colon (1.5%), ureteral reimplantation because of obstruction (3.6%), and nephrectomy for hydronephrosis (1%). A transurethral incision of the ileouretheral anastomosis was necessary in 7% of cases. Continence was separately addressed by sending each patient and his home physician a detailed questionnaire: Using our criteria (no diapers, no awakenings) the night and day continence rate increased from 67% at 6 months, to 72% at 1 year to 85% at 2 years, finally reacting 90% after 4 years. In part II of this presentation we address the question as to whether the option of orthotopic bladder replacement has any impact on the patient's and physician's decision toward earlier cystectomy. We compared our ileal neobladder cohort with a group of 137 patients that had been operated on during the same time span by the same group of surgeons. There was no negative selection with regard of the tumor stage of our patients. However, as compared with the conduit group, the neobladder cohort had a significantly improved survival rate. This phenomenon is explainable by the significantly lower number of previous transurethral resections of the bladder (TUR-Bs) performed in the neobladder group. The time span between primary diagnosis and cystectomy was 10 months in the neobladder group as compared with 18 months in the conduit patients. These data reinforce our belief that orthotopic bladder replacement using the ileal neobladder yields an extraordinary functional result that can be accomplished with a high degree of patient satisfaction and minimal complication. The availability of orthotopic bladder replacement does indeed stimulate the physicians and patients decision toward earlier cystectomy.

The ileal neobladder in women: 9 years of experience with 18 patients.

PURPOSE: We present our surgical and functional experience with orthotopic bladder replacement in women. MATERIALS AND METHODS: Since 1986, 18 women have undergone lower urinary tract reconstruction with an ileal neobladder. A nerve sparing cystectomy is done, and reservoirs are connected to the proximal urethra or urethrovesical junction. A total of 13 patients was available for complete followup as of March 1995. RESULTS: There were no perioperative deaths and few early complications. The only 2 failures were a neobladder vaginal fistula and these cases, which were converted to a conduit, are excluded from this study. Late complications requiring rehospitalization or reoperation in 2 patients included urethroileal stenosis that had to be dilated without further sequelae and bilateral ureteroileal stenosis that was treated endoscopically. At 3 months postoperatively excellent continence was achieved in 8 patients, while 2 had grade 1 stress incontinence and 3 were hypercontinent. As of March 1995 only 4 patients voided to completion while 9 required intermittent catheterization (continuously in 5 and twice daily for residual urine in 4). We were unable to demonstrate a functional difference of the various resection lines located at the proximal urethra or urethrovesical junction. CONCLUSIONS: Urethral support and nerve sparing cystectomy plus the ileal neobladder as a reservoir guarantee excellent continence in all patients. Despite our efforts, we have been unable to demonstrate any advantage of the nerve and urethral support sparing cystectomy technique as far as micturition is concerned. The development of hypercontinence in 70% of the patients with time demonstrates that our current understanding of the functional and anatomical basics of the voiding process is too limited to allow bladder replacement with a perfect functional result in all female patients. Our long-term experience, which is different from initial reports, justifies creation of an ileal neobladder in select female patients as long as they accept a 70% risk of clean intermittent catheterization in the long term. Overall patient satisfaction, including sexual life, is exceptional. However, disappointment is considerable when clean intermittent catheterization is required after periods of successful voiding per urethram.

High-dose pelvic irradiation followed by ileal neobladder urinary diversion: complications and long-term results.

OBJECTIVE: To determine the risk of post-operative complications in patients receiving high-dose pelvic irradiation before radical cystectomy and urinary diversion. PATIENTS AND METHODS: Of 300 patients who underwent orthotopic bladder replacement at our institution from 1986 to 1994, 11 patients (mean age 63 years, range 53-74) did so after receiving high-dose pelvic irradiation. The indication for cystectomy and urinary reconstruction was bladder cancer in seven men, prostate cancer in two men and a contracted bladder due to combined external pelvic irradiation and afterloading radiotherapy in two women. RESULTS: The post-operative course, including the duration of hospital stay, peri-operative complications and early functional results, did not differ from a control group of non-irradiated patients, and no patients died. The mean follow-up was 22 months (range 10-37) and revealed satisfactory results in seven of 11 patients. A neovesicoperitoneal fistula developed in one woman 10 months after surgery and was repaired by laparotomy. A neovesicovaginal fistula led to supravesical urinary diversion in the second woman. CONCLUSIONS: High-dose pelvic irradiation should not be a primary contra-indication for orthotopic urinary diversion using segments of small intestine. For patients who undergo combined external and afterloading radiotherapy, the indication for orthotopic bladder replacement should be considered critically.

The ileal neobladder: complications and functional results in 363 patients after 11 years of followup.

PURPOSE: Since 1986 orthotopic lower urinary tract reconstruction using the ileal neobladder has been our diversion of choice in patients of both sexes undergoing cystectomy. We report on experience and functional results of the first 363 men 11 years after this procedure. MATERIALS AND METHODS: Complications were assessed, tabulated, subdivided into early (3 months or less postoperatively) and late types, and further categorized with respect to relationship to neobladder construction. Continence and voiding pattern were individually evaluated via a detailed patient questionnaire. RESULTS: Perioperative death occurred in 11 patients (3%). Neobladder related early and late complications occurred in 56 (15.4%) and 85 (23.4%) of the 363 patients, respectively. Neobladder related early and late abdominal reoperation rates were 0.3 and 4.4%, respectively. Perioperative neobladder unrelated early complications were observed in 122 patients (33.6%) and 44 (12.1%) required operative treatment. Late postoperative complications unrelated to the neobladder occurred in 45 patients (12.4%) and 19 required open surgical revision. Of 290 evaluable patients 96.1% void spontaneously, 3.9% perform clean intermittent catheterization in some form and 1.7% perform regular intermittent catheterization. Daytime and nighttime continence was reported as good by 95.9% and satisfactory by 95% of the patients. Unacceptable daytime continence requiring more than 1 pad per day occurred in only 4.1% of the patients and only 5% are wetting more than 1 pad a night. CONCLUSIONS: The ileal neobladder produces good functional results and can be constructed with acceptable complications. Our data suggest that although it is not a complication-free procedure, we advocate its use when possible.

Association between the clinical presentation and epidemiological features of familial prostate cancer in patients selected for radical prostatectomy.

OBJECTIVE: We evaluated if epidemiological features of familial prostate cancer are associated with certain clinical or histopathological characteristics of the disease. METHODS: 463 German patients with familial prostate cancer who underwent radical prostatectomy were stratified according to several epidemiological criteria: (1). the apparent mode of disease transmission, (2). the average age of onset and (3). number of affected relatives/family, (4). whether or not they met the Johns Hopkins criteria of hereditary prostate cancer. The variables analysed included the Prostate Specific Antigen (PSA) and the digital rectal examination at diagnosis, histopathological characteristics of the prostatectomy specimen and relapse free 5-year survival rates. These characteristics were compared within the subsets of familial patients and compared to 492 control patients with sporadic prostate cancer. RESULTS: Age of onset was the only clinical parameter differentiating familial and sporadic prostate cancer. Otherwise there was no association between epidemiological features of familial predisposition and the clinical presentation or outcome of the disease. CONCLUSIONS: Familial and sporadic prostate cancer seem to be the same disease. Alternatively it may be concluded that the common epidemiological features of familial prostate cancer are not useful to tell tumours that are based on inherited susceptibility apart from those that are not. Whether hereditary prostate cancer is clinically distinct from sporadic forms cannot be determined before the underlying genetic alterations are identified.