[Epithelioid angiomyolipoma of the kidney after successfully treated malignant melanoma]. / Epitheloides Angiomyolipom der Niere nach stattgehabtem malignem Melanom.

BACKGROUND: Therapy of epitheloid angiomyolipomas (eAML) may be challenging, since unlike classical angiomyolipomas this rare subclass of benign mesenchymal angiomyolipomas may present with lymph node metastases, local recurrent disease, and/or systemic metastatic disease in up to 30% of cases. OBJECTIVES: We report here for the first time in Germany a case of eAML after successful treatment of malignant melanoma. MATERIALS AND METHODS: Clinical and histological findings as well as results of the genetic analysis of the angiomyolipoma are presented. RESULTS: A somatic, truncating mutation of the TSC2 gene was found in the angiomyolipoma. CONCLUSION: The relationship to histologically similar tumor entities are presented and therapeutic options based on the genetic classification are discussed.

Impact of surgeon's experience on outcome parameters following ureterorenoscopic stone removal.

Within the BUSTER trial, we analyzed the surgeon's amount of experience and other parameters associated with URS procedures regarding the stone-free rate, complication rate, and operative time. Patient characteristics and surgical details on 307 URS procedures were prospectively documented according to a standardized study protocol at 14 German centers 01-04/2015. Surgeon's experience was correlated to clinical characteristics, and its impact on the stone-free rate, complication rate, and operative time subjected to multivariate analysis. 76 (25%), 66 (21%) and 165 (54%) of 307 URS procedures were carried out by residents, young specialists, and experienced specialists (> 5 years after board certification), respectively. Median stone size was 6 mm, median operative time 35 min. A ureteral stent was placed at the end of 82% of procedures. Stone-free rate and stone-free rate including minimal residual stone fragments (adequate for spontaneous clearance) following URS were 69 and 91%, respectively. No complications were documented during the hospital stays of 89% of patients (Clavien-Dindo grade 0). According to multivariate analysis, experienced specialists achieved a 2.2-fold higher stone-free rate compared to residents (p = 0.038), but used post-URS stenting 2.6-fold more frequently (p = 0.023). Surgeon's experience had no significant impact on the complication rate. We observed no differences in this study's main endpoints, namely the stone-free and complication rates, between residents and young specialists, but experienced specialists' stone-free rate was significantly higher. During this cross-sectional study, 75% of URS procedures were performed by specialists. The experienced specialists' more than two-fold higher stone-free rate compared to residents' justifies ongoing efforts to establish structured URS training programs.

[Detection of papillomavirus DNA in the prostate: a virus with underestimated clinical relevance?]. / Die Detektion von Papillomavirus-DNA in der Prostata : Ein Virus mit unterschätzter klinischer Relevanz?

BACKGROUND: Human papillomaviruses (HPV) are the most frequent pathogens of sexually transmitted diseases. They have been associated with an increased incidence of several anogenital tumors. Whether oncogenic HPV are involved in the pathogenesis of prostate cancer has been a subject of great controversy. This study's purpose was to investigate the association between HPV infection and prostate cancer (PCA). MATERIAL AND METHODS: The study included 213 consecutive patients with an average age of 65.7 (+/-8.4) years. Within the framework of transrectal, ultrasonic-guided multibiopsy of the prostate, one additional core was examined by means of polymerase chain reaction (PCR) in relation to bacterial, fungal, and viral (including HPV) DNA, with subsequent DNA sequencing. The collected data were correlated with the histological results and with diverse clinical variables. The influence of several predictors for the existence of PCA was verified with a logistic regression model. RESULTS: No general bacterial DNA (16S rDNA) was detected. Of the 213 patients, 145 (68.1%) showed HPV DNA. In 64% (n=137), high-risk HPV DNA were depicted; these were 18% of the total in each case of HPV genotypes 16 and 18. From our examinations, no significant positive correlation existed between the HPV evidence and the histologically verified PCA that was found in 23.5% of the patients (n=50; odds ratio 1.45; 95% confidence interval 0.71-2.91). The BK virus was not found in any of the cores confirmed through PCR. CONCLUSION: Although no positive correlation between HPV infection and PCA existed in our study, data from the literature suggest an influence of the papillomavirus on PCA oncogenesis. Future studies should highlight to what extent HPV DNA is inserted in the genome of prostate cells and is able to cause subsequent malignant transformation of particular genes.

[The general practitioner's part in the initiation of diagnostic procedures in prostate cancer]. / Die Rolle des Hausarztes bei der Initiierung der Diagnostik des Prostatakarzinoms.

AIM: The aim of the study is the analysis of diagnostic procedures performed by general practitioners (GPs) leading to the detection of prostate cancer (PCA). PATIENTS AND METHODS: A retrospective evaluation was performed on all 406 patients who underwent ultrasound-supported transrectal multibiopsy of the prostate in our department between January 2004 and August 2005. The GPs were interviewed by use of standardised questionnaires concerning the indication for specific examinations, referrals to the specialist, the clinical findings and results, and the GPs own urological qualification. The rate of returned questionnaires was 72.9 % (105 of 144 GPs), therefore a study group (SG) of 295 patients (72.7 %) was formed. The mean age was 67.2 (40 - 90) years. RESULTS: In 190 patients (64.4 %) primary diagnostic measures were performed by the GP herself/himself. All the other patients had already been in urological specialist treatment (n = 43), were either referred directly to an urologist for primary diagnostics (n = 42) or were diagnosed during a hospital stay (n = 3). The reasons for the performed diagnostic measures were not to be found in 17 cases. In 50.8 % of all cases the initiation of preventive medical check-ups or determination of PSA levels was done at the patient's own request. 91 of 176 digital rectal examinations (DRE) performed by GPs showed suspect findings, leading to referral to the urologist in 62 cases. PSA levels of 105 patients measured by the GPs ranged between 0.0 and 1662 ng/mL with a mean level of 29.81 and a median of 6.20 ng/mL. In 75 cases the PSA level led to a referral to the urologist. In the case of 27 patients the referral was decided because of clinical symptoms. In 39.0 % (n = 115) of the SG a PCA was detected by biopsy. In 43.0 % (n = 64) of the patients originally examined by the GPs the suspected diagnosis of a PCA based on a pathological PSA level and/or DRE proved to be correct. The GPs stated in 35.2 % (n = 37) that they had spent a certain period of time in a urological department during their medical training. Concerning referrals and the positive predictive value of DRE, there was no statistically significant different to be found in GPs without urological training. CONCLUSION: Every fifth patient in the study group showed a histologically proven PCA that had initially been suspected by a GP based on a pathological PSA level and/or DRE. This underlines the value of preventive exams in the hands of GPs. In addition, the correct and sensible use of PSA levels controls by GPs as well as a pronounced desire for preventive medical check-ups on the patient's side was shown.

Treatment of varicocele with reference to age: a retrospective comparison of three minimally invasive procedures.

BACKGROUND: This study investigated whether the therapeutic efficacy and morbidity of three minimally invasive techniques for varicocele correction--laparoscopic varicocelectomy (LV), antegrade sclerotherapy (AS), and retrograde embolization (RE)--differed between children and adults. METHODS: During a 10-year period, 356 procedures for varicocele correction, including 122 cases of LV, 108 cases of AS, and 126 cases of RE, were performed for 314 patients at our institution. Of these patients, 223 were 19 years of age or younger (group 1), and 133 were older than 19 years (group 2). Diagnosis and postoperative results were established clinically and with the use of Doppler ultrasonography. The failure rates and complications for each procedure were retrospectively evaluated and compared between the two age groups. RESULTS: The median follow-up period was 69 months (range, 6-122 months). For 25 patients (19.8%), RE was not feasible for technical reasons. In both groups, LV had a lower failure rate than AS or RE, but the difference between LV and AS was not significant in group 1 (7.7(% vs 11.9%; p > 0.5). Also in group 1, AS was associated with fewer complications than LV 1 (4.5% vs 15.4%; p < 0.05). In group 2, LV was significantly more effective in correcting varicoceles than the other two techniques (p < 0.01). In this group, the complication rates for all three procedures did not differ significantly (p > 0.05). CONCLUSIONS: In our experience, LV was more effective than AS or RE in correcting varicoceles. For children and adolescents, AS may be more indicated because of the slightly lower complication rate and similar recurrence rates, as compared with LV, for this age group. The higher incidence of postoperative hydrocele formation after LV warrants more refined techniques such as the lymphatic-sparing approach.

[Multicystic renal tumor in a patient with WAGR syndrome]. / Multizystischer Nierentumor bei einem Patienten mit WAGR-Syndrom.

The WAGR syndrome is a combination of Wilms' tumor, aniridia, genitourinary anomalies, and mental retardation. We report on a 2-year-old boy, who had a deletion of the aniridia gene PAX6 and the Wilms' tumor gene 1 (WT1 gene). At the age of 23 months, a 1.7 x 1.9 cm-sized intrarenal tumor was detected by ultrasound examination. According to the protocol of the SIOP study, a cycle of neoadjuvant chemotherapy was prescribed followed by a left-sided nephrectomy. However, postsurgical histomorphology failed to confirm the suspected diagnosis of Wilms' tumor and instead revealed dysgenetic cysts of the kidney. Based on the image morphology in connection with the deletion of the WT1 gene, the tentative diagnosis of a nephroblastoma had to be made. The study protocol of the SIOP does not permit another therapy algorithm.

[Radical cystectomy in the treatment of bladder cancer always in due time?]. / Stellen wir die Indikation zur radikalen Zystektomie immer zeitgerecht?

INTRODUCTION: The aim of this study was to examine how the survival rates for patients with muscle-invasive bladder carcinoma are influenced by the tumor stage at initial presentation. PATIENTS AND METHODS: This study examined the clinical course of 452 patients who underwent radical cystectomy for bladder carcinoma from 1992 to 2004. The patients were divided into three groups according to the histological results of the initial and final transurethral tumor resection (TURB). In group 1 (n=114) patients who presented with a superficial bladder carcinoma which had a high likelihood of progressing underwent radical cystectomy. Group 2 included (n=92) patients who displayed a superficial tumor stage when they first presented and developed progressive muscle-invasive bladder carcinoma under conservative treatment. Group 3 (n=246) comprised patients who were already at the muscle-invasive tumor stage in the course of primary TURB. The histopathological characteristics of all transurethral tumor resections and radical cystectomy were recorded. Progression-free survival rates and overall survival rates in the three groups were then compared. RESULTS: The average patient age at cystectomy was 64.3 (35-80) years, and the average follow-up period was 49 months. Progression-free survival and overall survival of all 452 patients were 56.1 and 53.6%, respectively, after 5 years. The best outcome was a progression-free 5-year survival rate of 78.4% with organ-confined, lymph node-negative tumors (n=213). This result was statistically significant (p<0.01) compared with the progression-free 5-year survival rate of 42.3% for non-organ-confined, lymph node-negative tumors (n=112). Lymph node-positive patients (n=127) achieved a progression-free 5-year survival rate of 29.0% regardless of the tumor infiltration. Group 1 patients achieved a progression-free survival rate of 71.3% and an overall survival rate of 69.1% after 5 years. Group 2 patients achieved a progression-free survival rate of 52.9% and an overall survival rate of 51.4% after 5 years. Group 3 patients achieved a progression-free survival and overall survival of 50.2% and 47.1%, respectively, after 5 years. There was no significant difference between groups 2 and 3 with regard to their progression-free or overall survival rates (p>0.45). However, both groups displayed significantly poorer progression-free and overall survival rates compared with group 1 (p<0.01). CONCLUSION: Our results show that patients with superficial bladder carcinoma with tumor progression to muscle invasion do not have a better prognosis after radical cystectomy than patients presenting initially with muscle-invasive bladder carcinoma. Survival rates in this group can only be improved by singling out patients on the basis of risk factors at an earlier stage and carrying out cystectomy. Due to these results we must expect that waiting for a muscle invasion in patients with superficial bladder carcinoma with a high risk profile results in a significant impairment of prognosis.

[High-dose chemotherapy with transplantation of autologous stem cells in advanced germ-cell poor-risk testicular tumors].

Testicular tumors illustrate curable cancer, but 25% patients are resistant to standard therapy. High-dose chemotherapy (HDC) is promising therapy for germ-cell tumors with poor prognosis. HDC and transplantation of autologous stem cells were performed in 13 patients with germ-cell testicular tumors (GTT). In 6 patients of group 1 HDC was first-line treatment in poor prognosis, in 7 patients (group 2) it was a salvage treatment after recurrences. Patients of group 1 had longer mean survival than those of group 2 (31.3 and 11 months, respectively; p = 0.136). Two patients died of HDC complications. Neurological, hematological and other complications occurred. In spite of 50-90% remission after HDC, multicenter prospective randomized trials will give final conclusion on effectiveness of HDC which must be performed in special clinics having many specialists in their staff (urologists, oncologists, chemotherapists, etc.).

[Coincidence of testicular germ cell tumor and sarcoidosis: A diagnostic challenge]. / Testikulärer Keimzelltumor und Sarkoidose: Eine diagnostische Herausforderung.

Several case reports and small case series have described a total of 66 patients with sarcoidosis and testicular cancer so far. This report describes three additional cases. We highlight the association of sarcoidosis and testicular cancer and comment on the potential impact of this connection on the interpretation of the radiological and pathological findings in suspected cancer relapse. Sarcoidosis, a condition that can be combined with testicular cancer, should always be considered in the differential diagnosis.

[Gemcitabine and carboplatin chemotherapy in advanced transitional cell carcinoma in regard to patients with impaired renal function]. / Gemcitabine/Carboplatin-Chemotherapie in der Behandlung des metastasierten Urothelkarzinoms unter besonderer Berücksichtigung von Patienten mit eingeschränkter Nierenfunktion.

PURPOSE: The aim of this analysis is the evaluation of the activity and toxicity of gemcitabine and carboplatin in patients with advanced urothelial transitional carcinoma (TCC) with special regard to patients with impaired renal function. PATIENTS AND METHODS: 30 consecutive patients with metastatic TCC [mean age: 68 (range: 47 - 82) years, median ECOG-PS:1] were treated with gemcitabine (1000 mg/m (2) on days 1 and 8 of a 21-day schedule) and carboplatin (AUC 4.5 day 1). In 15 patients (considered as renal unfit) a creatinine clearance of less than 60 mL/min (range: 31 - 59 mL/min) was seen. RESULTS: Concerning the survival rate, no significant difference noticed between the two subgroups of renal impaired patients and patients with normal renal function was detected (median 13 vs. 14 months, p = 0.901). An overall response rate of 50 % was obtained. In 16.7 % and 33.3 % of all cases a complete or a partial response was noted. Median time to progression was 5.34 months. The 1-year-survival rate has been calculated as 51.8 %. There was no restriction of renal function under chemotherapy in any single patient. CONCLUSIONS: The chemotherapy combination of gemcitabine and carboplatin is definitely powerful for a first-line-therapy in patients with advanced TCC. Toxicity is well manageable. Due to the dosage for carboplatin by AUC an adaptation to the glomerular filtration rate is possible. Decreases of effectiveness in cases of impaired renal function were not detected. Patients with metastatic TCC should be entered onto well designed, randomised clinical trials with the gemcitabine/carboplatin combination to afford a tailored chemotherapy.

Do we need new high-risk criteria for surgically treated renal cancer patients to improve the outcome of future clinical trials in the adjuvant setting? Results of a comprehensive analysis based on the multicenter CORONA database.

BACKGROUND: Since there is still an unmet need for potent adjuvant strategies for renal cancer patients with high progression risk after surgery, several targeted therapies are currently evaluated in this setting. We analyzed whether inclusion criteria of contemporary trials (ARISER, ASSURE, SORCE, EVEREST, PROTECT, S-TRAC, ATLAS) correctly identify high-risk patients. METHODS: The study group comprised 8873 patients of the international CORONA-database after surgery for non-metastatic renal cancer without any adjuvant treatment. Patients were divided into potentially eligible high-risk and assumable low-risk patients who didn't meet inclusion criteria of contemporary adjuvant clinical trials. The ability of various inclusion criteria for disease-free survival (DFS) prediction was evaluated by Harrell's c-index. RESULTS: During a median follow-up of 53 months 15.2% of patients experienced recurrence (5-year-DFS 84%). By application of trial inclusion criteria, 24% (S-TRAC) to 47% (SORCE) of patients would have been eligible for enrollment. Actual recurrence rates of eligible patients ranged between 29% (SORCE) and 37% (S-TRAC) opposed to <10% in excluded patients. Highest Hazard Ratio for selection criteria was proven for the SORCE-trial (HR 6.42; p < 0.001), while ASSURE and EVEREST reached the highest c-index for DFS prediction (both 0.73). In a separate multivariate Cox-model, two risk-groups were identified with a maximum difference in 5-year-DFS (94% vs. 61%). CONCLUSION: Results of contemporary adjuvant clinical trials will not be comparable as inclusion criteria differ significantly. Risk assessment according to our model might improve patient selection in clinical trials by defining a high-risk group (28% of all patients) with a 5-year-recurrence rate of almost 40%.

Is it possible to provide a prognosis after radical prostatectomy for prostate cancer by means of a PSA regression model?

BACKGROUND: For over 15 years, studies have been done to evaluate the elimination kinetics of the prostate-specific antigen (PSA) after radical prostatectomy. Even though evaluation of PSA regression in the two-compartment model has become established, no clear data are currently available as to whether a statement can be made with regard to tumor prognosis from a computation of the PSA half-life (PSA-HL). This study focuses on the determination of the PSA-HL in the two-compartment model and on its correlation with the biochemical recurrence-free survival. In addition, a computer program is being developed to simplify the determination of PSA-HL. MATERIAL AND METHODS: Seventy-seven prospective patients were examined who subsequently had a radical prostatectomy at our facility without neoadjuvant or adjuvant hormone deprivation. In addition to preoperative measurement of the PSA value (dO), PSA determinations were carried out postoperatively on days 5, 10 and 60, and at four-monthly intervals thereafter (mean follow-up: 16 months). By means of the computer program developed for this purpose, CTK.TumW, the PSA half-lives for the first (d0-d5, PSA-HL1) and second (d5-d10, PSA-HL2) compartments were subsequently determined and their effect on biochemical recurrence-free survival was assessed. RESULTS: PSA-HL1 and PSA-HL2 were 1.89 (+/- 0.03) and 3.39 (+/- 0.14) days, respectively. Whilst PSA-HL1 did not permit any prognostic statement, the median PSA-HL in the second compartment between patients with and without disease progression differed significantly (4.44 versus 3.12 days; p < 0.001). Discrimination analysis produced a cutoff of 3.8 days for the second compartment; patients with a PSA-HL2 > or = 3.8 days had a significantly worse biochemical recurrence-free survival after 18 months than the other patients (27% versus 93%; p < 0.001). CONCLUSION: The PSA regression kinetics after radical prostatectomy follows a two-compartment model in which the prognostic value of the PSA-HL1 is limited. When a cutoff of 3.8 days is used, evaluation of the PSA-HL in compartment 2 (d5-10) appears to permit a prognostic statement. Due to the limited postsurgical follow-up, the disease process was only assessed as biochemical recurrence-free survival, and a longer follow-up will be necessary to generate data on progression-free survival.

[Silica-containing urinary stones--clinical issues to keep in mind]. / Silikathaltige Harnsteine im Klinikalltag. Was gibt es zu beachten?

Formation of calculi in efferent urinary passages is always due to supersaturation of urinary calculi substances and associated increased crystallization. Apart from the typical calculi, consisting of calcium oxalate, inorganic phosphates, uric acid or cystine, there are occasional signs of rare substance classes. Although more than 50 silicate stones have already been reported internationally, this stone entity remains relatively unknown. In particular, the occurrence of silicate stones in the absence of magnesium trisilicate abuse is extremely rare. A medium-sized left-sided ureterolith was removed from a 54-year-old male patient using a ureteroscope. X-ray diffraction showed it to be a compound stone consisting of 40% silicate. The patient, who in 1986 was living close to the nuclear reactor accident in Chernobyl, showed no signs of a constant uptake of magnesium trisilicate. However, he had undergone partial (2/3) gastrectomy 4 months before for a drug-refractory gastric ulcer, which had been diagnosed at the end of the 1980s and treated with excessive dosages of a magnesium trisilicate antacid preparation until the time of the operation. The patient had also been suffering from unstable angina pectoris since 1986 and treated with Pentalong (pentaerythrityltetranitrate) for 17 years. We were also able to detect silicium dioxide in components of this drug using X-ray diffraction. Silicate uroliths are extremely rare but they can be clearly identified by X-ray diffraction or infrared spectroscopy and distinguished from artifacts or quartz pebbles. Formation of calculi can be prevented by increasing diuresis as well as switching to a different drug and reducing the dosage.

[Solitary renal cell carcinoma metastasis to the thyroid gland--a paradigm of metastasectomy?]. / Die solitäre Nierenzellkarzinommetastase in der Schilddrüse. Ein Paradigma der Metastasenchirurgie?

We investigated the usefulness of thyroidectomy for solitary metastases from renal cell carcinomas in ten patients. In the absence of postoperative morbidity and mortality, a mean survival time of 3.4 years was observed. Subsequently, four patients developed intracerebral metastases. Swelling of the neck and the discovery of a nodule in the thyroid of patients who have undergone nephrectomy for renal cell carcinoma should raise suspicion of a metastasis, possibly after a long latency period. With the aid of modern immunohistochemical methods, renal cell carcinoma metastasis can now be identified unequivocally, with differentiation from a primary follicular carcinoma of the thyroid rendered possible by a combination of TTF-1, thyroglobulin, and CD 10. In the event of a solitary lesion with no extrathyroidal tumour manifestation, an R0 resection of the metastasis should always be attempted. If tumour dissemination has occurred, palliative measures and endoscopic intervention (e.g. placement of an endotracheal stent) with the aim of improving quality of life by preventing obstruction of the airways are justified.

[Outlet of a megaureter with aplastic kidney into a seminal vesicle cyst. Case report of laparoscopic intervention]. / Mündung eines Megaureters mit aplastischer Niere in eine Samenblasenzyste. Fallbericht einer laparoskopischen Intervention.

Unilateral cyst of the seminal vesicle, ipsilateral ectopic ureter, and ipsilateral renal aplasia are the components of a rare congenital abnormality affecting the male urogenital tract. The clinical picture is characterized by a retrovesical cystic tumor, which may cause nonspecific symptoms. Differential diagnosis includes cysts of the müllerian duct, the urogenital sinus/ejaculatory duct, and the prostate. We report a case of a left seminal vesicle cyst associated with aplasia of the left kidney in a 14-year-old boy. Preoperatively, an ipsilateral renal agenesis was suspected, but a renal aplasia with an ectopic urinary megaureter into the seminal vesicle was found intraoperatively. An 8-cm cystic left seminal vesicle and an ipsilateral renal aplasia was successfully removed by operative laparoscopy. The advantages of the laparoscopic approach over open surgery include excellent exposure of the deep pelvic structures, a short hospitalization, and a rapid recovery for the patient. We searched the literature to review the clinical presentation, diagnostic procedures, differential diagnosis, and therapeutic treatment options for this anomaly.

[Impact of immunochemotherapy on survival of patients with metastatic renal cell carcinoma. A retrospective study comparing interferon-alpha-2a/vinblastine versus interferon-alpha-2a/interleukin-2/5-fluorouracil]. / Bedeutung der Immunochemotherapie für das Uberleben von Patienten mit metastasiertem Nierenzellkarzinom. Eine retrospektive Studie der Therapiegruppen Interferon-alpha2a/Vinblastin vs. Interferon-alpha2a/Interleukin-2/5-Fluorouracil.

The prognosis for patients with metastatic renal cell carcinoma (RCC) remains unsatisfactory to date. Combined immunochemotherapy (ICT) strives for a synergistic effect avoiding a substantial increase of therapy-related adverse events. The combination therapy regimes consisting of either interferon-alpha-2a/vinblastine (IFN-alpha2a/VBL) or interferon-alpha-2a/interleukin-2/5-fluorouracil (IFN-alpha2a/IL-2/5-FU) demonstrated objective remission rates, surpassing the results obtained with the administration of single immunotherapeutic agents. Despite the data from a recently published study, the role of these two therapy combinations did not seem clearly defined. Therefore, we compared the impact of IFN-alpha2a/VBL and IFN-alpha2a/IL-2/5-FU on remission and survival as well as the safety profile in a retrospective study in patients with metastatic RCC. In a retrospective single-center study, 105 patients with metastatic RCC having received treatment between 1992 and 2002 with either s.c. IFN-alpha2a/ i.v. VBL ( n=70, group 1) or s.c. IFN-alpha2a/ s.c. IL-2/ i.v. 5-FU ( n=35, group 2) were evaluated. At a median follow-up of 17 months, remission and survival rates as well as the toxicity profiles of the respective groups were documented and compared. The median age throughout the entire patient population was 61 years. Patients in the IFN-alpha2a/VBL group reached a median overall survival of 20 months compared to 17 months for the patients in the IFN-alpha2a/IL-2/5-FU population ( p=0.850). The objective response rate in the first patient group reached 25.7%, whereas the tumor remission rate of group 2 amounted to 22.9% ( p=0.680). Patients showing an objective response reached a significantly higher survival rate than patients without response reaction (median survival was 36 vs 10 months, p=0.0001). The incidence of each therapy-induced adverse event was higher throughout the second treatment group. These differences were significant with respect to flu-like symptoms (85.7 vs 57.1%, p=0.003), grade 3/4 elevations of liver enzymes (14.3 vs 1.4%, p=0.007), nausea/vomiting (74.3 vs 50%, p=0.017), the severity of erythemas (74.3 vs 10%, p<0.001), and patients with lung edema (17.1 vs 2.9%, p=0.009). Eight patients discontinued the ICT, two of whom died of a myocardial infarction.Despite an overall limited prognosis, patients showing a tumor remission seem to benefit from ICT in terms of overall survival. While both treatment options offer comparable remission and survival rates, the IFN-alpha2a/VBL regimen induces fewer adverse events than the treatment with IFN-alpha2a/IL-2/5-FU.

[Bilateral testicular masses in the scope of adrenogenital syndrome]. / Beidseitige testikuläre Raumforderungen im Rahmen des adrenogenitalen Syndroms.

Testicular masses in male individuals with the adrenogenital syndrome (AGS) are a clinical and pathological diagnostic dilemma. The major differential diagnosis of gonadal nodules in this setting includes interstitial Leydig cell tumors and secondary benign tumors possibly of adrenal origin. We report a case of adrenogenital syndrome occurring in a 14-year-old boy. Examinations to clarify the cause of his dwarfism and bilateral testicular masses revealed 21-hydroxylase deficiency. The testes were not tender and were firm and nodular on palpation. The serum levels of adrenocorticotrophic hormone (ACTH), 17 alpha-hydroxyprogesterone (17-alpha-OHP), testosterone, and aldosterone were found to be elevated. Under corticosteroid therapy the serum marker abnormalities were corrected and there was gradual regression of the tumor lesions in both testes. Testicular tumors with adrenogenital syndrome are typically bilateral and develop in untreated or inadequately treated males with AGS.

[Locally recurrent malignant fibrous histiocytoma of the kidney and spermatic cord]. / Das lokal rezidivierende maligne fibröse Histiozytom der Niere oder des Samenstrangs.

OBJECTIVE: In this study, 26 patients with locally recurrent malignant fibrous histiocytoma of the kidney and spermatic cord after initial R0 resection were reviewed with regard to therapeutic options and prognosis. PATIENTS AND METHODS: Based on a literature query in the PubMed database, we identified 24 cases with locally recurrent malignant fibrous histiocytoma of the kidney and spermatic cord after initial R0 resection. Two of our own patients were included and afterwards the entire patient group was analyzed with regard to the time period of the first local recurrence and the overall survival rate. RESULTS: The average patient age was 58 years; in 17 cases (65%) the left side was affected. After primary therapy the 5-year survival rate was 25%; adjuvant therapy did not achieve any significant improvement in survival time ( p=0.259). The local recurrence was on average diagnosed after 13 months (with a median of 12 months). The prognosis of malignant fibrous histiocytoma after detection of the local recurrence was extraordinary poor, only 4 of 26 patients survived for longer than 3 years. The 1-, 2- and 5-year survival rates then were 34%, 28%, and 14%, respectively. The mean survival time was 31 months with a median survival of 9 months. Patients with a locally recurrent malignant fibrous histiocytoma of the spermatic cord showed a significant better survival prognosis than patients with a local recurrence of a renal malignant fibrous histiocytoma ( p=0.04). CONCLUSION: Malignant fibrous histiocytoma of the genitourinary tract are rare tumors with a high rate of local recurrence. If there are no distant metastases a R0 resection can result in a curative objective. Nevertheless, the prognosis of this disease is poor. Even early detection of local therapy failure and promptly initiated aggressive salvage therapy may offer the chance of long-term survival only in selected cases. Lifelong follow-up is necessary for patients with a malignant fibrous histiocytoma of the kidney or spermatic cord.

[Angiomyolipoma of the kidneys as a rare cause of retroperitoneal hemorrhage. Two case reports with tuberous sclerosis Bourneville-Pringle]. / Renale Angiomyolipome assoziiert mit tuberöser Hirnsklerose als seltene Ursache einer abundanten retroperitonealen Blutung. Report von 2 Fällen.

Tuberous sclerosis (Bourneville-Pringle-disease, TSC) is an autosomal dominant disorder characterized by seizures, mental retardation and hamartomatous tumours in multiple organs, including subependymal giant cell astrocytomas, cardiac rhabdomyomas and renal angiomyolipomas. Recent population-based studies suggest a prevalence of 1 case per 25,000 individuals. Renal angiomyolipomas, which may be found sporadically or associated with TSC, become evident as an acute retroperitoneal haemorrhage or by symptoms of a flank mass. Ultrasound and computed tomography provide clear evidence of lipomatous formation while, in rare instances, angiography can demonstrate the existence of multiple vascular tumour compartments. In view of two cases which were admitted with the clinical picture of an acute abdomen on the basis of retroperitoneal haemorrhage, the therapeutic strategies for TSC patients with renal angiomyolipomas are discussed, paying regard to the literature in this field.

[Superior vena cava syndrome with bilateral jugular and subclavian vein thrombosis. Paraneoplastic manifestion of renal cell carcinoma]. / V. -cava-superior-Syndrom mit beidseitiger Jugularis- und SubklaviavenenthromboseParaneoplastische Manifestation eines Nierenzellkarzinoms.

At present thrombosis of the superior vena cava is an uncommon event that is now more frequently associated with diagnostic or therapeutic catheterization. If an apparent spontaneous thrombosis occurs, malignancy should be considered in the differential diagnosis. One case of clinically symptomatic thrombosis of the internal jugular, subclavian, and superior vena cava is presented. We detected an asymptomatic left renal cell carcinoma in a 54-year-old patient and nephrectomy was performed. Increased blood coagulability as part of a paraneoplastic syndrome was considered to be the possible etiology. In patients with otherwise unexplained superior vena cava thrombosis, examination not only of the head and neck but also of the abdomen, retroperitoneum, and pelvis should be pursued. A review of the literature pertinent to this rare case is provided.