Evaluation of incidence and histolopathological findings of soft tissue sarcomas in genitourinary tract: Uludag university experience.

PURPOSE: In this study we aimed to review urological soft tissue sarcomas of genitourinary tract that were diagnosed in our institution and their prognostic factors for survival. MATERIALS AND METHODS: The clinical and pathological records of 31 patients who had diagnosis of soft tissue sarcomas primarily originating from the genitourinary tract between 2005-2011 were reviewed. RESULTS: The most common site was kidney (17 cases, 54.8%), and most common diagnosis was leiomyosarcoma (11 cases, 35.4%). A total of 24 patients (77.4%) had surgical excision. The surgical margins were positive in 7 patients who presented with local recurrence after primary resection. Twelve patients developed metastatic disease. During follow-up (range 9-70 month), 26 of the 31 patients (88.9%) were alive. Significant survival differences were found according to histological type (p: 0.001), with lower survival rates for malignant fibrous histiocytoma. The tumor size, the presence of metastasis at the time of diagnosis and tumor localization were not statistically significant for overall survival. CONCLUSIONS: In our series, prostate sarcomas, paratesticular rhabdomyosarcoma and malignant fibrous histiocytoma had poor prognosis, especially in patients presenting with metastatic disease.

Comparison of two different suture techniques in laparoscopic partial nephrectomy.

OBJECTIVE: To comparatively evaluate the traditional interrupted knot-tying and running suture renorrhaphy with Monocryl® in laparoscopic partial nephrectomy (LPN). MATERIALS AND METHODS: A retrospective analysis of 62 consecutive patients undergoing LPN using traditional interrupted knot-tying suture renorrhaphy (Group 1; n=31) or running suture technique renorrhaphy with 2-0 monofilament polyglecaprone (Monocryl®, Ethicon) (Group 2; n=31) from December 2011 to October 2015 at the University. All patients underwent LPN performed by an experienced laparoscopic surgeon. The demographic, perioperative and postoperative parameters were compared between the groups, and the effect of both suture techniques on the warm ischemic time (WIT) and trifecta were evaluated. RESULTS: The running suture renorrhaphy with Monocryl® reduced WIT, estimated blood lost and length of hospitalization stay significantly without increasing postoperative complication rate during LPN in comparison with interrupted knot-tying suture. CONCLUSION: The renorrhaphy using the running suture with Monocryl® is an effective and safe technique with the advantage of shortening WIT even in more challenging and larger tumors during LPN.

Congenital nephrotic syndrome of NPHS1 associated with cardiac malformation.

Congenital nephrotic syndrome (CNS) is a rare disease inherited as an autosomally recessive trait and defined as proteinuria manifesting at birth or in the first 3 months of life. The classical form is the Finnish type of CNS (CNF), which is caused by mutations in the nephrin gene (NPHS1). The classical findings include prematurity, large placenta and massive proteinuria. Minor cardiac findings have been reported as a minor functional disorder but CNS with major cardiac malformation is rare. Here we report the case of a Turkish child with CNS with small indel mutation (c.614_621delCACCCCGGinsTT) in exon 6 of NPHS1 and also major cardiac malformation who did not develop end-stage renal disease until the age of 5 years.

Prognostic value of estrogen receptors in patients who underwent prostatectomy for non­metastatic prostate cancer.

Estrogen receptors in prostate cancer (PCa) are a subject of debate. The aim of the present study was to investigate whether estrogen receptor-α (ERα) and estrogen receptor-ß (ERß) impact the biochemical recurrence (BCR) of non-metastatic PCa after surgery. Following the application of the exclusion criteria, data from 108 patients who underwent laparoscopic radical prostatectomy between January 2011 and December 2019 were retrospectively evaluated. A total of 36 patients with BCR constituted the BCR group. The control group was formed using the Propensity Score Matching (PSM) method with a 1:2 ratio, including parameters with well-studied effects on BCR. The median follow-up time was 74.3 (range, 30-127.5) months in the BCR group and 66.6 (range, 31.5-130) months in the control group. Pathology specimens from the two groups were immunohistochemically stained with ERα and ERß antibodies. Logistic regression analysis and survival analysis were performed. No differences in clinicopathological characteristics were detected between the two groups. The patients with ERα(-)/ERß(+) staining results had a significantly fewer BCRs than other patients (P=0.024). In the logistic regression analysis, patients with ERα(-)/ERß(+) PCa also had a significantly lower risk of recurrence (P=0.048). In the survival analysis, the 5-year BCR-free survival rate of patients with ERα(-)/ERß(+) PCa was higher than that of other patients (85.7 vs. 66.1%; P=0.031). Excluding the effects of well-studied risk factors for recurrence by the PSM method, the present study showed that ERα and ERß have prognostic value for non-metastatic PCa. The 5-year BCR-free survival rate is significantly higher in patients whose PCa tissue has ERα(-)/ERß(+) staining results.

AA amyloidosis presenting with acute kidney injury, curable or not?

BACKGROUND: Amyloidosis is a group of disorders with extracellular accumulation of autologous fibrillary insoluble proteins in various tissues and organs such as the kidneys, liver, spleen, heart and gastrointestinal tract leading to impairment of normal organ function. Childhood amyloidosis is an exceedingly rare entity mainly caused by familial Mediterranean fever (FMF) and the other autoinflammatory diseases such as mevalonate kinase deficiency (MKD). CASE: A 16-year-old male was referred to pediatric nephrology for coincidentally discovered proteinuria. He had no significant findings on physical examination except for urochromic color. He had nephrotic range proteinuria with 109 mg/m2/h and serum creatinine was 1.35 mg/dl. Kidney biopsy was performed because of nephrotic range proteinuria with acute kidney injury. In hematoxylin-eosin-stained tissue sections, amyloid was suggested as extracellular amorphous material that is lightly eosinophilic in the glomeruli. Diagnosis was confirmed by Congo red positivity, with apple-green birefringence under polarized light. MEFV gene mutation was negative and a compound heterozygote mutation found in mevalonate kinase gene. A 6-monthtrial of colchicine, enalapril, and losartan combination was not successful; Canakinumab was started thereafter. Proteinuria and creatinine decreased to 7 mg/m2/h and 0.6 mg/dl respectively 4 years after treatment. CONCLUSIONS: Amyloidosis should be considered especially in children presenting with proteinuria and with a history of recurrent fever. This report also emphasizes the efficacy of canakinumab to prevent or decelerate chronic renal failure in these patients although it does not reduce tissue deposition in long-term use.

Laparoscopic Transperitoneal Radical Nephrectomy for Renal Masses with Level I and II Thrombus.

BACKGROUND: We aimed to report surgical and oncological outcomes of renal cell carcinoma (RCC) patients with level I and II thrombus treated with laparoscopic transperitoneal radical nephrectomy. PATIENTS AND METHODS: Medical records of 13 patients were reviewed retrospectively in a single center between 2005 and 2017. Hem-o-lok® clips were used to secure renal artery and renal vein, excluding 2 patients with an attached thrombus on the right side. RESULTS: Seven male and 6 female patients were treated laparoscopically, and conversion to open surgery was not required in any case. The mean age was 61.6 ± 7.8 years (range 45 to 75 years) and the mean body mass index was 27 ± 4.6 kg/m2 (range 18.8 to 34.5 kg/m2). Median tumor size was 9.5 × 7.3 cm (range 5 to 14 cm) and 5 patients who had preoperative metastases underwent cytoreductive nephrectomy. The mean operative time was 137.6 ± 45.8 minutes (range 60 to 200 minutes) and blood loss was 105 ± 34.9 mL (range 50 to 150 mL). The mean hospital stay was 4.4 ± 3.9 days (range 1 to 15 days). Splenic capsular injury occurred in 1 patient and the vena cava was injured in another. Three patients needed blood transfusions. The mean follow-up period was 25 months (range 12 to 86 months). CONCLUSION: Laparoscopic transperitoneal radical nephrectomy can be reliable option for surgical management of RCC with level I and II thrombus in experienced centers.

T2E (TMPRSS2-ERG) fusion transcripts are associated with higher levels of AMACR mRNA and a subsequent prostate cancer diagnosis in patients with atypical small acinar proliferation.

Genetic rearrangements involving androgen-regulated transmembrane protease serine 2 (TMPRSS2) and genes from the ETS transcription factor family, most commonly ERG and ETV1, result in alteration that responsible for oncogenic activity in prostate cancer (PC). The aims of the present study were to: 1) investigate the frequency of these fusion transcripts in prostate tissue samples obtained from patients diagnosed with atypical small acinar proliferation (ASAP), 2) determine any clinical significance of T2E expression at the RNA level in predicting PC detection in subsequent biopsies, and 3) evaluate expression of the PC marker, alpha-methylacyl-CoA racemase (AMACR), according to T2E status by real-time quantitative reverse transcription PCR (RT-qPCR). T2E transcripts were detected in 31.7% (n=20) of the patients examined, and this was significantly associated with subsequent detection of PC in ASAP patients with a prostate specific antigen (PSA) level of 4-10ng/ml (p=0.045). AMACR expression was also significantly higher in the patients who were diagnosed with PC in subsequent biopsies than in the patients who were not diagnosed with PC (p=0.034) and in T2E-positive ASAP patients (p=0.002) compared to T2E-negative ASAP patients. Although these results need to be further clinically validated, we suggest that the presence of T2E transcript, in association with higher AMACR expression, is an indicator of PC risk from a T2E-positive focus or an unsampled malignant gland adjacent to a T2E-positive site in ASAP lesions.

The protective effect of L-arginine, tadalafil, and their combination in rat testes after ischemia and reperfusion injury.

INTRODUCTION: Nitric oxide (NO) plays an important role in the ischemia and reperfusion process. In this study, we aimed to examine the effect of L-arginine, tadalafil, and their combination for prevention of the ischemia reperfusion injury after testis torsion in rats. METHODS: A total of 40 adult, male Sprague-Dawley rats were allocated into five groups. Three hours of left testicular torsion was performed in each group, excluding the control group. While the ischemia reperfusion (I/R) group had no treatment, I/R + Arg group received L-arginine, I/R + Td group received tadalafil and I/R + Arg + Td group received tadalafil and L-arginine 30 minutes before the detorsion. Then the left testis was untwisted for four hours of reperfusion. After bilateral orchiectomy, lipid peroxidation (LPx) and glutathione (GSH) activities were examined in testicular tissue. Spermatogenesis was evaluated with Johnsen's score. RESULTS: LPx levels of the I/R group were found to be significantly higher than for groups that received drugs for both testes (p<0.001). GSH levels of the combination group were higher than I/R group in ipsilateral testis (p<0.01) and it was significantly higher than other groups for contralateral testis (p<0.001 for I/R group, p<0.01 for I/R + Arg, p<0.05 for I/R + Td). Mean Johnsen's score of the I/R group was found to be significantly lower than treatment groups in ipsilateral testis (p<0.001 for I/R + Arg + Td group, p<0.01 for other treatment goups) and contralateral testis (p<0.001). The mean Johnsen score of the combination group was significantly higher than that of other treatment groups in ipsilateral testis (p<0.05) and it was significantly higher than in the I/R + Td group in the contralateral testis (p<0.05). CONCLUSIONS: L-arginine, tadalafil, and combination of these two molecules showed protective effect against ischemia/reperfusion injury for both testes after unilateral testis torsion.

Clinical outcomes and survival in AA amyloidosis patients.

AIM: Amyloid A amyloidosis is a rare complication of chronic inflammatory conditions. Most patients with amyloid A amyloidosis present with nephropathy and it leads to renal failure and death. We studied clinical characteristics and survival in patients with amyloid A amyloidosis. METHODS: A total of 81 patients (51 males, 30 females) with renal biopsy proven amyloid A amyloidosis were analyzed retrospectively. The patients were divided into good and poor outcomes groups according to survival results. RESULTS: Most of the patients (55.6%) had nephrotic range proteinuria at diagnosis. Most frequent underlying disorders were familial Mediterranean fever (21.2%) and rheumatoid arthritis (10.6%) in the good outcome group and malignancy (20%) in the poor outcome group. Only diastolic blood pressure in the good outcome group and phosphorus level in the poor outcome group was higher. Serum creatinine levels increased after treatment in both groups, while proteinuria in the good outcome group decreased. Increase in serum creatinine and decrease in estimated glomerular filtration rate of the poor outcome group were more significant in the good outcome group. At the time of diagnosis 18.5% and 27.2% of all patients had advanced chronic kidney disease (stage 4 and 5, respectively). Median duration of renal survival was 65±3.54 months. Among all patients, 27.1% were started dialysis treatment during the follow-up period and 7.4% of all patients underwent kidney transplantation. Higher levels of systolic blood pressure [hazard ratios 1.03, 95% confidence interval: 1-1.06, p=0.036], serum creatinine (hazard ratios 1.25, 95% confidence interval: 1.07-1.46, p=0.006) and urinary protein excretion (hazard ratios 1.08, 95% confidence interval: 1.01-1.16, p=0.027) were predictors of end-stage renal disease. Median survival of patients with organ involvement was 50.3±16 months. CONCLUSION: Our study indicated that familial Mediterranean fever constituted a large proportion of cases and increased number of patients with idiopathic amyloid A amyloidosis. Additionally, it was observed that patient survival was not affected by different etiological causes in amyloid A amyloidosis.

Open vs Laparoscopic Simple Prostatectomy: A Comparison of Initial Outcomes and Cost.

INTRODUCTION: We compared the cost-effectiveness of laparoscopic simple prostatectomy (LSP) vs open prostatectomy (OP). PATIENTS AND METHODS: A total of 73 men treated for benign prostatic hyperplasia were enrolled for OP and LSP in groups 1 and 2, respectively. The findings were recorded perioperative, including operation time (OT), blood lost, transfusion rate, conversion to the open surgery, and the complications according to the Clavien Classification. The postoperative findings, including catheterization and drainage time, the amount of analgesic used, hospitalization time, postoperative complications, international prostate symptom score (IPSS) and International Index of Erectile Function (IIEF) scores, the extracted prostate weight, the uroflowmeter, as well as postvoiding residual (PVR) and quality of life (QoL) score at the postoperative third month, were analyzed. The cost of both techniques was also compared statistically. RESULTS: No statistical differences were found in the preoperative parameters, including age, IPSS and QoL score, maximum flow rate (Qmax), PVR, IIEF score, and prostate volumes, as measured by transabdominal ultrasonography. No statistical differences were established in terms of the OT and the weight of the extracted prostate. No differences were established with regard to complications according to Clavien's classification in groups. However, the bleeding rate was significantly lower in group 2. The drainage, catheterization, and hospitalization times and the amount of analgesics were significantly lower in the second group. The postoperative third month findings were not different statistically. Only the Qmax values were significantly greater in group 2. While there was only a $52 difference between groups with regard to operation cost, this difference was significantly different. CONCLUSION: The use of LSP for the prostates over 80 g is more effective than the OP in terms of OT, bleeding amount, transfusion rates, catheterization time, drain removal time, hospitalization time, consumed analgesic amount, and Qmax values. On the other hand, the mean cost of the LSP is higher than OP. Better effectiveness comes with higher cost.

Evaluation of incidence and histolopathological findings of soft tissue sarcomas in genitourinary tract: Uludag university experience

ABSTRACT Purpose: In this study we aimed to review urological soft tissue sarcomas of genitourinary tract that were diagnosed in our institution and their prognostic factors for survival. Materials and Methods: The clinical and pathological records of 31 patients who had diagnosis of soft tissue sarcomas primarily originating from the genitourinary tract between 2005-2011 were reviewed. Results: The most common site was kidney (17 cases, 54.8%), and most common diagnosis was leiomyosarcoma (11 cases, 35.4%). A total of 24 patients (77.4%) had surgical excision. The surgical margins were positive in 7 patients who presented with local recurrence after primary resection. Twelve patients developed metastatic disease. During follow-up (range 9-70 month), 26 of the 31 patients (88.9%) were alive. Significant survival differences were found according to histological type (p: 0.001), with lower survival rates for malignant fibrous histiocytoma. The tumor size, the presence of metastasis at the time of diagnosis and tumor localization were not statistically significant for overall survival. Conclusions: In our series, prostate sarcomas, paratesticular rhabdomyosarcoma and malignant fibrous histiocytoma had poor prognosis, especially in patients presenting with metastatic disease.

Clinical outcomes and survival in AA amyloidosis patients / Desfechos clínicos e sobrevida em pacientes com amiloidose AA

Abstract Aim Amyloid A amyloidosis is a rare complication of chronic inflammatory conditions. Most patients with amyloid A amyloidosis present with nephropathy and it leads to renal failure and death. We studied clinical characteristics and survival in patients with amyloid A amyloidosis. Methods: A total of 81 patients (51 males, 30 females) with renal biopsy proven amyloid A amyloidosis were analyzed retrospectively. The patients were divided into good and poor outcomes groups according to survival results. Results: Most of the patients (55.6%) had nephrotic range proteinuria at diagnosis. Most frequent underlying disorders were familial Mediterranean fever (21.2%) and rheumatoid arthritis (10.6%) in the good outcome group and malignancy (20%) in the poor outcome group. Only diastolic blood pressure in the good outcome group and phosphorus level in the poor outcome group was higher. Serum creatinine levels increased after treatment in both groups, while proteinuria in the good outcome group decreased. Increase in serum creatinine and decrease in estimated glomerular filtration rate of the poor outcome group were more significant in the good outcome group. At the time of diagnosis 18.5% and 27.2% of all patients had advanced chronic kidney disease (stage 4 and 5, respectively). Median duration of renal survival was 65 ± 3.54 months. Among all patients, 27.1% were started dialysis treatment during the follow-up period and 7.4% of all patients underwent kidney transplantation. Higher levels of systolic blood pressure [hazard ratios 1.03, 95% confidence interval: 1-1.06, p = 0.036], serum creatinine (hazard ratios 1.25, 95% confidence interval: 1.07-1.46, p = 0.006) and urinary protein excretion (hazard ratios 1.08, 95% confidence interval: 1.01-1.16, p = 0.027) were predictors of end-stage renal disease. Median survival of patients with organ involvement was 50.3 ± 16 months. Conclusion Our study indicated that familial Mediterranean fever constituted a large proportion of cases and increased number of patients with idiopathic amyloid A amyloidosis. Additionally, it was observed that patient survival was not affected by different etiological causes in amyloid A amyloidosis.

Resumo Objetivo: A amiloidose AA é uma complicação rara de condições inflamatórias crônicas. A maior parte dos pacientes com amiloidose AA apresenta nefropatia, que leva à insuficiência renal e à morte. Estudaram-se as características clínicas e a sobrevida em pacientes com amiloidose AA. Métodos: Analisaram-se retrospectivamente 81 pacientes (51 homens, 30 mulheres) com amiloidose AA comprovada por biópsia renal. Os pacientes foram divididos em grupos de desfecho bom e ruim de acordo com os resultados de sobrevida. Resultados: A maior parte dos pacientes (55,6%) tinha proteinúria na faixa nefrótica no momento do diagnóstico. Os distúrbios subjacentes mais frequentes foram a febre familiar do Mediterrâneo (FFM, 21,2%) e a artrite reumatoide (10,6%) no grupo de desfecho bom e a malignidade (20%) no grupo de desfecho ruim. Somente a pressão arterial diastólica no grupo de desfecho bom e o nível de fósforo no grupo de desfecho ruim foram mais elevados. Os níveis séricos de creatinina aumentaram após o tratamento em ambos os grupos, enquanto a proteinúria diminuiu no grupo de desfecho bom. O aumento na creatinina sérica e a diminuição na TFGe do grupo de desfecho ruim foram mais significativos no grupo de desfecho bom. No momento do diagnóstico, 18,5% e 27,2% de todos os pacientes tinham doença renal crônica avançada (estágios 4 e 5, respectivamente). A duração média da sobrevida renal foi de 65 ± 3,54 meses. Entre todos os pacientes, 27,1% iniciaram tratamento de diálise durante o período de seguimento e 7,4% de todos os pacientes foram submetidos a transplante renal. Níveis elevados de pressão arterial sistólica [taxas de risco (HR) 1,03, intervalo de confiança (IC) de 95%: 1 a 1,06, p = 0,036], creatinina sérica (HR 1,25, IC 95%: 1,07 a 1,46, p = 0,006) e excreção urinária de proteínas (HR 1,08, IC 95%: 1,01 a 1,16, p = 0,027) foram preditores de doença renal terminal. A mediana da sobrevida de pacientes com comprometimento de órgãos foi de 50,3 ± 16 meses. Conclusão: O presente estudo indicou que a FFM constituiu uma grande proporção de casos e crescente quantidade de pacientes com amiloidose AA idiopática. Adicionalmente, observou-se que a sobrevida do paciente não foi afetada pelas diferentes causas etiológicas na amiloidose AA.