Marked Response to Nivolumab by a Patient With SMARCA4-Deficient Undifferentiated Urothelial Carcinoma Showing High PD-L1 Expression: A Case Report.

BACKGROUND: SMARCA4 is a component gene of the SWI/SNF (SWItch/Sucrose NonFermentable) chromatin remodeling complex; undifferentiated tumors associated with its functional deletion have been described in several organs. However, no established treatment for these tumors currently exists. CASE: In this study, we report a case of a SMARCA4-deficient undifferentiated urothelial carcinoma with high PD-L1 expression that was effectively treated with nivolumab after early relapse following treatment for non-invasive bladder cancer. The histological morphology of the rhabdoid-like undifferentiated tumor of unknown primary led us to suspect a SWI/SNF-deficient tumor, and subsequent immunostaining led to the diagnosis of a SMARCA4-deficient undifferentiated tumor. This effort also led to the identification of the developmental origin of this SMARCA4-deficient undifferentiated tumor as a non-invasive bladder cancer. We also carried out a detailed immune phenotypic assay on peripheral T cells. In brief, a phenotypic change of CD8+T cells from naive to terminally differentiated effector memory cells was observed. CONCLUSION: Regardless of the organ of cancer origin or cancer type, SWI/SNF-deficient tumors should be suspected in undifferentiated and dedifferentiated tumors, and immune checkpoint inhibitors may be considered as a promising treatment option for this type of tumor. The pathogenesis of SMARCA4-deficient anaplastic tumors awaits further elucidation for therapeutic development.

Pre-first-line chemotherapy risk stratification for overall survival in advanced urothelial carcinoma in sequential therapy era.

PURPOSE: To explore pre-treatment risk factors for overall survival (OS) in advanced urothelial carcinoma (UC) patients treated with first-line (1L) chemotherapy in sequential therapy (ST) era. Additionally, to evaluate the proportion of patients who were not able to undergo subsequent immune checkpoint inhibitor (ICI) therapy according to the subgroups stratified by the risk factors. METHODS: A multicenter retrospective study was conducted. Metastatic or locally advanced UC patients treated between 2017 and 2022 were included. The Kaplan-Meier method with the log-rank test and multivariate Cox regression models were used to address OS. RESULTS: Three hundred and fourteen patients treated with 1L chemotherapy were included in the study and 57 (18.2%) patients were not able to proceed to subsequent ICI therapy. Pre-chemotherapy risk factors for OS in 314 patients were ECOG-PS 1 or more, having no primary site resection, C-reactive protein (CRP) level of 3 mg/dL or more, and non-cisplatin-based regimen. Patients having 3 or 4 risk factors had higher risk for not being able to receive ST (Mann-Whitney U test, P < 0.001). As risk factors for OS in 230 patients who were able to receive ST, having no primary site resection, a neutrophil to lymphocyte ratio of 3 or more, and the presence of liver metastasis were identified. CONCLUSION: We reported the risk factors for OS in advanced UC patients treated with 1L chemotherapy in ST era. Patients with high risk for OS may not be able to proceed to subsequent ICI therapy even in the ST era.

[A Case of Neuropathic Lower Urinary Tract Dysfunction Due to Spina Bifida Occulta Discovered at the Age of 19 Years and Successfully Treated with Multidisciplinary Therapy].

A 19-year-old man had been aware of dysuria and urinary incontinence since childhood but did not seek medical attention. He was diagnosed with acute pyelonephritis due to lower urinary tract dysfunction associated with spina bifida occulta and tethered cord syndrome (TCS) due to spinal cord lipoma. After placement of a urethral catheter and antibacterial chemotherapy, the patient was cured of acute pyelonephritis. He was treated with solifenacin and started clean self-intermittent catheterization (CIC). Shortly after the start of CIC, the acute pyelonephritis flared up again, and he was managed with a reinserted urethral catheter until an untethering operation. Preoperative video urodynamics showed that the bladder morphology was Ogawa classification grade III with vesicoureteral reflux (VUR) at 92 ml infusion. With the combination of an untethering operation and additional mirabegron, the functional bladder capacity was increased to 353 ml and VUR improved, allowing for safe urinary management of the CIC. TCS can be diagnosed at any age and requires appropriate urinary management and therapeutic intervention as early as possible after diagnosis.

Intrinsic and extrinsic factors causing hyperplasia of the prostate.

Prostatic hyperplasia is very common in elderly men and is a typical disease that reduces quality of life. Histologically, hyperplasia of the prostate gland causes obstruction at the bladder outlet, resulting in symptoms such as a weak urine stream. Various factors have been considered to cause histological enlargement of the prostate, but the underlying cause is still unknown. The factors that cause prostate hyperplasia can be broadly classified into intrinsic and extrinsic ones. Extrinsic factors include things that we directly come into contact with such as bacteria and food. On the other hand, intrinsic factors are those that cause changes in functions originally provided in the body due to some cause, including extrinsic factors, such as chronic inflammation and an imbalance of sex hormones. A large number of reports have been made to date regarding the etiology of prostatic hyperplasia, although they have not yet clarified the fundamental cause(s). The various factors currently known should be outlined for future research. Should it be possible to prevent this highly prevalent prostatic hyperplasia which is mainly cause of dcreasing quality of life, there is no doubt that it would be a huge contribution to humanity.

[Two Cases of Retroperitoneal Synovial Sarcoma].

We experienced two cases of renal primary synovial sarcoma. Case 1: A 29-year-old man underwent laparoscopic radical nephrectomy and was originally diagnosed with renal cell carcinoma. Case 2: A 25-year-old man was treated by open radical nephrectomy since radiographical findings indicated tumor invasion to the ureter causing hydronephrosis. Both cases were pathologically diagnosed as renal synovial sarcomas, and were followed using computed tomography. Recurrence was observed within a year in both cases.

Development and preliminary evaluation of a novel procedure for creation of an ileal conduit stoma aimed at preventing parastomal hernia.

OBJECTIVES: Our previous study suggested that the operative procedure is critical for the development of parastomal hernia. We developed a novel procedure for the creation of an ileal conduit stoma to prevent parastomal hernia. Herein we evaluate the efficacy and safety of the procedure. METHODS: A total of 113 Japanese patients underwent radical cystectomy and ileal conduit diversion for bladder cancer from January 2017 through December 2021 at our institution. After excluding those with incomplete data, 103 patients consisting of 46 (44.7%) with the conventional procedure and 57 (55.3%) with the novel procedure were consecutively enrolled. The main points of the novel procedure are as follows: (1) the passage of the ileal conduit is ≤2.4 cm in diameter in principle; (2) the posterior rectus sheath and peritoneum are vertically incised 2 cm laterally from the middle of the stoma site to make an oblique passage for the ileal conduit; and (3) the anterior rectus sheath and posterior rectus sheath with peritoneum are fixed to the ileal conduit separately. RESULTS: Radiography-based parastomal hernia was observed in 11 patients (10.7%) with a median follow-up of 22.0 months. The incidences of parastomal hernia were 3.5% and 19.6% in the novel and the conventional procedure groups, respectively (p = 0.011). The former had a significantly lower cumulative incidence of parastomal hernia (p = 0.008, log-rank test). No specific complications associated with the procedure were observed. CONCLUSIONS: The results of the preliminary cohort study suggest that the novel procedure is safe and effective for the prevention of parastomal hernia.

Maternal diet during gestation affect prostatic tissue component in SHR/Izm offspring.

BACKGROUND: Numerous studies have investigated the associations between maternal nutritional status and various diseases, with the underlying mechanism often attributed to epigenetic changes. However, limited research has been conducted on the relationship between maternal nutrition and benign prostatic hyperplasia (BPH). In this study, we aimed to explore the potential association between maternal nutrition and BPH using an animal experiment and evaluating the findings through fluorescent immunostaining and genetic analysis. METHODS: Female spontaneously hypertensive rats (SHR/Izm) were randomly assigned to three groups at the start of pregnancy: a standard diet group (SD; 17% protein, 7% fat), a low-protein diet group (LPD; 6% protein, 7% fat), and a high-fat diet group (HFD; 22% protein, 35% fat). The diets were maintained throughout gestation. After giving birth, both the mothers and their pups were exclusively fed a standard diet. Male pups were euthanized at 48 weeks, and their prostates were removed. The composition of the ventral prostate (VP) was evaluated using fluorescent immunostaining with antibodies for cytokeratin, vimentin, and Ki-67. Microarray analysis, real-time RT-PCR, and DNA methylation analysis using pyrosequencing were performed. Statistical analysis was conducted using one-way ANOVA and Tukey's multiple comparison test, with a significance level set at p < 0.05. RESULTS: Pups in the LPD group exhibited significant underweight from birth (1 day; SD vs. LPD vs. HFD: 4.46 vs. 4.08 vs. 4.35, p = 0.04) until weaning (21 days; SD vs. LPD vs. HFD: 30.8 vs. 27.4 vs. 29.2, p = 0.03). However, they exhibited catch-up growth, and there was no significant difference at 48 weeks (p = 0.84). The epithelial area in the ventral prostate was significantly increased in the LPD group (SD vs. LPD vs. HFD: 39% vs. 48% vs. 37%, p = 0.01), while the stromal area was significantly increased in the HFD group (SD vs. LPD vs. HFD: 11% vs. 11% vs. 15%, p < 0.01). Gene ontology analysis of the gene expression microarray showed increased activity in developmental processes (SD vs. LPD: p = 6.3E-03, SD vs. HFD: p = 7.2E-03), anatomical structure development (SD vs. LPD: p = 6.3E-03, SD vs. HFD: p = 5.3E-03), and cell differentiation (SD vs. LPD: p = 0.018, SD vs. HFD: p = 0.041) in both the LPD and HFD groups. Real-time RT-PCR revealed high expression levels of the transcription factors NFκB (p < 0.01) and Smad3 (p < 0.01) in both the LPD and HFD groups. XIAP, an apoptosis inhibitor, was increased in the LPD group (p = 0.02). The TGF beta pathway, associated with epithelial mesenchymal transition (EMT), and vimentin (p < 0.01) were upregulated in the HFD group. Pyrosequencing DNA methylation analysis of the TGF beta pathway indicated hypomethylation of TGFb1, TGFbR1, and Smad3 in all groups, although there were no significant differences. CONCLUSIONS: Our findings suggest that both maternal undernutrition and obesity influence the prostatic development of offspring. Maternal consumption of a low protein diet promotes epithelial hyperplasia through the upregulation of apoptosis inhibitors, while a high fat diet leads to increased stromal growth through the induction of EMT.

Durable response to pembrolizumab in microsatellite instability-high advanced adrenocortical carcinoma.

Introduction: Advanced adrenocortical carcinoma has a poor prognosis and is treated with chemotherapy that includes mitotane with etoposide, doxorubicin, and cisplatin as first-line therapy. However, second-line therapy has not been determined yet. Pembrolizumab has been approved for high microsatellite instability for which standard treatments have failed. Case presentation: Here, we present a patient with advanced adrenocortical carcinoma treated with complete surgical resection. 21 months later, he had local and metastatic recurrences. After four cycles of first-line therapy, we switched to pembrolizumab because microsatellite instability-high was detected in his tumor. He has received mitotane and pembrolizumab for 15 months, and this has exerted a radiographical response without severe adverse events. Conclusion: We presented a patient with microsatellite instability-high advanced adrenocortical carcinoma treated with pembrolizumab and mitotane.

[Evaluation of the Effects of Long-Term Indwelling Ureteral Stents on Preserving Renal Parenchyma and Function in Patients with Ureteral Stenosis].

Although ureteral stenting is a common conservative treatment for ureteral stricture, it is unclear whether a long-term indwelling ureteral stent protects the kidney against parenchymal atrophy and functional deterioration. In this study, we evaluated the changes in renal parenchymal thickness (RPT) and estimated the glomerular filtration rates (eGFR) in patients with indwelling ureteral stents for one year or more. As a control, we also evaluated changes in RPT associated with indwelling percutaneous nephrostomy (PNS) for one year or more. Polymer ureteral stents were used and replaced every three months. RPT was measured using computed tomography (CT). Totally, 69 renal units in 55 patients with baseline and follow up CT scans available were enrolled. The median follow-up period was 29 months. The etiologies of ureteral obstruction were malignant and benign disease in 27 and 28, respectively. RPT was reduced obviously in most cases. At 1 year, the median reduction rate of RPT was 17.3% in unilateral cases, which was significantly higher than that in the healthy contralateral kidney. There was a strong correlation between eGFR and total RPT including the contralateral kidney. The reduction rate of RPT in kidneys with ureteral stents including bilateral cases was also significantly higher than that in 39 renal units of 35 patients with PNS. The results of this study suggest that the long-term efficacy of indwelling ureteral stents in preserving renal function is limited. Regular imaging may be essential to evaluate the residual renal function.

[Urological Comorbidities in an HIV-Infected Patient: A Case Report].

We report a case of a patient who developed several urological comorbidities associated with HIV infection. A 53-year-old male was diagnosed with HIV infection and AIDS. After 13 years, microhematuria was found and computed tomography (CT) revealed urolithiasis and a left renal tumor suspected of being renal cell carcinoma. Initially, he underwent transurethral lithotripsy. Stone analysis indicated that the stone was made of atazanavir. Then he received laparoscopic left partial nephrectomy. The pathological diagnosis was papillary type 2 renal cell carcinoma. Three years later, follow-up CT revealed a right renal pelvic tumor. Since right ureteroscopy showed that the tumor was papillary we diagnosed it as renal pelvic cancer and decided to perform laparoscopic right radical nephroureterectomy. His renal pelvic tumor was determined to be urothelial carcinoma by the pathological diagnosis. Intravesical recurrence occurred twice after the nephroureterectomy. His renal function gradually deteriorated during follow-up and we suspected that HIV nephrosis was one of the reasons for the deterioration. Hemodialysis was initiated at the age of 71.

Prevention of local symptoms in muscle invasive bladder cancer patients: clinical significance of local radiation therapy.

PURPOSE: To evaluate the significance of local radiation therapy (LRT) for prevention of local symptoms (LSs) caused by muscle-invasive bladder cancer (MIBC). METHODS: We retrospectively reviewed the clinical records of 133 patients from 13 hospitals. MIBC patients with or without metastases who were treated with LRT alone from January 2015 through December 2020 were enrolled. Exclusion criteria were urinary diversion (UD) prior to LRT, non-MIBC, or lack of clinical information. LSs were defined as hematuria requiring invasive treatment or transfusion, UD after LRT, bladder tamponade, and opioid use for bladder pain. RESULTS: One hundred fourteen patients were finally enrolled in the study. During the median follow-up period of 13.5 months, 30 patients (26.3%) had LSs. Risk factors of LSs in multivariate analysis were a prior history of non-MIBC (NMIBC) (hazard ratio [HR] 2.99; 95% confidence interval [CI], 1.36 to 6.56; P < 0.01), radiation dose of less than 50 Gray (Gy) (HR 3.99; 95% CI, 1.80 to 8.82; P < 0.01), and tumor stage 3 or more (HR 2.43; 95% CI, 1.14 to 5.21; P = 0.02). Risk factors of overall survival (OS) in multivariate analysis were being female (HR 3.32; 95% CI, 1.68 to 6.58; P < 0.01), an age-adjusted Charlson Comorbidity index of 6 or more (HR 2.19; 95% CI, 1.18 to 4.10; P = 0.01), distant metastases (HR 3.20; 95% CI, 1.39 to 6.58; P < 0.01), and tumor size of 40 mm or more (HR 2.38; 95% CI, 1.34 to 4.52; P < 0.01). Toxicity (all grades) occurred in 40.4% of the patients, 4.8% with grade 3 or more and 95.2% with lower grades. CONCLUSIONS: We determined the risk factors for LSs in MIBC patients treated with LRT alone. An escalated-dose of 50 Gy or more may contribute to prevention of LSs caused by MIBC. Thus, dose-escalated LRT for MIBC patients who can expect favorable survival may be a good option to avoid future annoying LSs.

Difference in symptom manifestation between postmenopausal and premenopausal women in acute uncomplicated cystitis: A multi-institutional pilot study.

Objectives: We aimed to prospectively compare lower urinary tract symptoms in premenopausal and postmenopausal women with acute uncomplicated cystitis before and after antibiotic therapy. Materials and methods: This study included adult women with acute uncomplicated cystitis who visited 4 institutions between 2019 and 2020. After registration, we administered oral antibiotics and prospectively documented the changes in lower urinary tract symptoms from the first visit to a follow-up visit at 1 week using the Core Lower Urinary Tract Symptoms Score (CLSS) questionnaire. Results: After treatment, pyuria disappeared in 60 of the 66 patients (14 premenopausal and 46 postmenopausal). The CLSS total score (range) changed from 13 (3-29) to 4 (0-18) with a significant improvement in all CLSS items. At baseline, nocturia, urgency, and urgency incontinence were more prominent in postmenopausal women than in premenopausal women. In contrast, baseline urethral pain and quality of life index were more severe in premenopausal women than in postmenopausal women. After treatment, the CLSS total score was still higher in postmenopausal women, as reflected by the relatively higher scores for nocturia and urgency, irrespective of the comparable scores for urethral pain and the quality of life index in the 2 groups. Conclusions: Our results suggest that if storage symptoms persist, they should be carefully interpreted according to menopausal status.

Adjuvant nivolumab versus placebo following radical surgery for high-risk muscle-invasive urothelial carcinoma: a subgroup analysis of Japanese patients enrolled in the phase 3 CheckMate 274 trial.

BACKGROUND: The phase 3 CheckMate 274 trial demonstrated superiority of adjuvant nivolumab over placebo after radical surgery in patients with high-risk muscle-invasive urothelial carcinoma. However, the efficacy and safety of adjuvant nivolumab in Japanese patients with muscle-invasive urothelial carcinoma have not been clarified. METHODS: Patients with muscle-invasive urothelial carcinoma were randomized to adjuvant nivolumab 240 mg or placebo (every 2 weeks via intravenous infusion) up to 120 days after radical surgery in CheckMate 274. RESULTS: Of 49 patients in the Japanese subgroup, 27 and 22 patients were randomized to nivolumab and placebo, respectively. Eleven and 8 patients, respectively, had tumor PD-L1 expression level of 1% or more. The median disease-free survival times in the nivolumab and placebo groups were 29.67 months (95% confidence interval 7.79-not reached) and 9.72 months (95% confidence interval 4.73-not reached), respectively (hazard ratio 0.77, 95% confidence interval 0.35-1.69). The corresponding values in patients with tumor PD-L1 expression level of 1% or more were 29.67 months (95% confidence interval 2.63-not reached) and 25.95 months (95% confidence interval 5.59-not reached) (hazard ratio 1.10, 95% confidence interval 0.31-3.92), respectively. Treatment-related adverse events of Grade 3-4 occurred in 25.9 and 13.6% of patients in the nivolumab and placebo groups, respectively. The most common treatment-related adverse events in the nivolumab group were lipase increased, amylase increased and diarrhea. The changes in quality of life scores from baseline over time were similar in both groups. CONCLUSIONS: The efficacy and safety results in the Japanese subgroup were consistent with the overall population of CheckMate 274.

Clinical characteristics and treatment outcomes of patients with small cell carcinoma of the urinary bladder.

Background: Small cell carcinoma of the urinary bladder (SCUB) is rare. The optimal treatment for SCUB remains unclear. To address the problem of appropriate treatment for each case, we assessed single-modality and surgery-based multimodality treatments in patients with SCUB. Materials and methods: We retrospectively reviewed the medical records of 12 patients with SCUB between 1990 and 2013. All patients underwent transurethral resection of the bladder tumor and were diagnosed with SCUB. Their clinicopathological characteristics were assessed, and the outcomes were compared according to the treatment modality. Results: The median (range) age at diagnosis was 66 years (range, 53-85 years). T1-4N0M0 was observed in 8 patients (66%), N1-3M0 in 2 (17%), and NanyM1 in 2 (17%). After transurethral resection of the bladder tumor, 6 patients (50%) underwent cystectomy alone, and 4 (33%) underwent cystectomy and presurgical or adjuvant chemotherapy with etoposide and cisplatin. During the median follow-up period of 20.7 months, 6 patients (50%) died of cancer, and 2 patients (17%) died of other causes. The median overall survival period was 1.9 years. The 5-year overall survival rate in patients who underwent cystectomy and chemotherapy was 75%, whereas that in those who underwent cystectomy alone and transurethral resection alone were 22% and 0%, respectively (p = 0.012). Recurrence-free survival was significantly correlated with cause-specific survival (r = 0.95; 95% confidence interval, 0.81-0.99; p < 0.001). Conclusions: Radical cystectomy with chemotherapy using the etoposide and cisplatin regimen improved the prognosis of patients with SCUB and TxNxM0. The time from initial progression to death due to cancer was very short, indicating that the initial treatment strategy is crucial.

[Clinical Investigation of Immune Checkpoint Inhibitor-Associated Kidney Injury in Patients with Urologic Cancers].

We investigated the clinical characteristics of patients who developed kidney injury after starting treatment with immune checkpoint inhibitors (ICI) for urologic malignancies. The study included 118 patients who were treated with ICI at our hospital. They consisted of 65 with renal cell carcinoma, 52 with urothelial carcinomas and 1 with adrenocortical carcinoma with high-frequency microsatellite instability. Immune-related kidney injury was observed in 13 patients (11.0%), including stage 1, 2 and 3 kidney injuries in 9, 0 and 4 patients, respectively. In univariate analyses, ≥stage 4 chronic kidney disease (CKD) before ICI treatment and proton pump inhibitor use were significantly associated with all stages of kidney injury, whereas ≥stage 4 CKD and ICI combination therapy were significantly associated with kidney injury at ≥ stage 2. Of the 4 patients who developed ≥stage 2 kidney injury, histological examination was done only for 2 because renal biopsy was contraindicated in the other 2 due to prior nephrectomy. Steroid pulse therapy was performed for 3 patients but provided complete recovery only in 1. We should be aware of the risk for immune-related kidney injury in patients with baseline CKD (≥stage 4) and receiving ICI combination therapy. Precise diagnosis by histological examination can often be challenging due to a history of nephrectomy.

Are there differences in the characteristics of patients who respond to gemcitabine plus cisplatin chemotherapy and those who respond to pembrolizumab therapy for metastatic urothelial carcinoma? Multicenter retrospective study.

OBJECTIVES: To evaluate factors to predict overall survival of metastatic urothelial carcinoma patients treated with gemcitabine plus cisplatin chemotherapy or pembrolizumab therapy. METHODS: We retrospectively evaluated two metastatic urothelial carcinoma cohorts treated with (i) gemcitabine plus cisplatin or (ii) pembrolizumab. The gemcitabine plus cisplatin cohort was treated from December 2005 through December 2014 while the pembrolizumab cohort was treated from January 2018 through December 2020. Using multivariate analyses, we evaluated the risk factors for overall survival in each cohort and compared them. None of the gemcitabine plus cisplatin cohort patients were treated with pembrolizumab. All patients in the pembrolizumab cohort were treated with prior platinum-based chemotherapy. RESULTS: There were 184 patients in the gemcitabine plus cisplatin cohort and 91 in the pembrolizumab cohort. The mean follow-up periods were 714 and 284 days, respectively. In multivariate analysis, the risk factors for overall survival in the gemcitabine plus cisplatin cohort were liver metastasis, worse Eastern Cooperative Oncology Group performance status (1 or more), no primary site resection, and a high prognostic index (1 or more). In the pembrolizumab cohort, liver metastasis, bone metastasis, and worse Eastern Cooperative Oncology Group-performance status (1 or more), and high prognostic index (1 or more) were the risk factors for overall survival. In the pembrolizumab cohort, patients with a complete response or partial response during prior platinum-based chemotherapy had better overall survival with the following pembrolizumab treatment than those with stable or progressive disease (P = 0.004). CONCLUSIONS: Considering the similarity of these risk factors in two sequential treatments, it may be possible to predict the response to pembrolizumab according to the response to prior chemotherapy.

Management of hydronephrosis after radical cystectomy and urinary diversion for bladder cancer: A single tertiary center experience.

OBJECTIVES: To clarify the incidence of postoperative hydronephrosis and verify the validity of diagnostic and therapeutic approaches for hydronephrosis after cystectomy and urinary diversion for bladder cancer. METHODS: Totally, 290 patients receiving urinary diversion from 2005 through 2017 with complete data were enrolled, including 258 (89.0%) with an ileal conduit and 32 (11.0%) with an ileal neobladder. Postoperative radiographic images were reviewed. In patients with postoperative hydronephrosis, antegrade pyelography and ureteroscopy were performed to exclude malignant etiology. Balloon dilation and open surgical revision were performed according to the conditions. RESULTS: Forty-six patients (58 renal units) developed postoperative hydronephrosis. The cumulative incidence was 11.4% by a median follow-up of 59.5 months. Ureteral recurrence was detected by antegrade examinations in two patients, whereas malignant strictures were subsequently revealed in three patients. Thus, malignant etiology was found in hydronephrosis in five renal units (12.8%) of five patients (16.1%). The median times to diagnosis of hydronephrosis were 0 (interquartile range [IQR] 0-4) and 14 months (IQR 9-12) for benign and malignant strictures, respectively (p = 0.003). Of them, 31 patients (39 renal units) received interventions. Balloon dilation was performed in 13 renal units with benign strictures, and was successful in two (15.4%). Open surgical revision was performed in eight patients (11 renal units), including two with failed balloon dilation, all of which was successful. CONCLUSIONS: Postoperative hydronephrosis is potentially associated with recurrent disease. Accurate differential diagnosis is challenging although antegrade procedures may be helpful in some cases. Open surgical revision is highly effective to treat benign strictures.

A case of metastatic Xp11.2 translocation renal cell carcinoma showing a prolonged response to nivolumab as 6th-line treatment.

A female patient with Xp11.2 translocation renal cell carcinoma developed lung metastasis 24 months after partial nephrectomy that was performed at the age of 32. Sunitinib, everolimus, axitinib, temsirolimus and pazopanib were sequentially administered for 55 months and disease progression was observed. Then nivolumab was started as 6th-line treatment. After a transient increase in tumor size, metastatic tumors started to shrink. Eventually, nivolumab provided a partial response with a 35% reduction of tumor size at 50 months and freedom from progression for 60 months. The present case suggests that immune checkpoint inhibitors are effective in selected cases with Xp11.2 translocation renal cell carcinoma.

[A Case of Diagnosed Lynch Syndrome in a Patient with Ureteral Cancer].

Lynch syndrome (LS) is an autosomal dominant genetic disorder in which tumors are known to develop at an early age. Upper tract urothelial carcinoma is one of the tumors related to Lynch syndrome. A 49-year-old woman visited a urologic clinic due to left abdominal pain. She had a history of ovarian cancer. Her mother had a history of colorectal cancer and renal pelvic cancer, and her grandmother had had colorectal cancer. After detailed examination, she received laparoscopic left nephroureterectomy and she was pathologically diagnosed with left ureteral cancer. LS was suspected based on her past history, family history, and age. A microsatellite instability (MSI) test gave a positive result, and genetic analysis confirmed a mutation in the MSH2 gene, leading to the diagnosis of Lynch syndrome. Although LS has a high frequency of carcinogenesis, it is thought that an improved prognosis can be achieved by early discovery and treatment of cancer in LS patients. From our case report, we recommend screening of LS in patients with a past/family history, who have had an upper tract urothelial carcinoma. Once LS is diagnosed, the patient should be followed by a planned surveillance of cancer development.