[A Case of Inflammatory Myofibroblastic Tumor of The Bladder Treated with Partial Cystectomy].

A 60-year-old woman with extensive hematuria visited our hospital. Cystoscopy revealed a tumor with an edematous surface on the left lateral wall of the urinary bladder. To diagnose the tumor, we performed a transurethral resection of the bladder tumor. Pathological examination suggested leiomyoma. Four weeks after the transurethral resection, magnetic resonance imaging revealed an increase in the bladder tumor. She received partial cystectomy. Pathological analysis revealed an inflammatory myofibroblastic tumor. No recurrence was observed 9 months after the initial operation.

Short-term outcomes of risk-adapted upfront docetaxel administration in patients with metastatic hormone-sensitive prostate cancer: a multicenter prospective study in Japan.

We conducted a risk-adapted upfront docetaxel (DOC) in patients with metastatic hormone-sensitive prostate cancer (mHSPC). Here, we reported an interim analysis of the study. The study enrolled 68 patients with newly diagnosed mHSPC between 2016 and 2018. According to the presence of visceral metastasis, an EOD score ≥ 3, or prostate-specific antigen (PSA) level at 3 months of ≥ 1 ng/mL, patients were divided into low- and high-risk groups. Patients were treated with androgen deprivation therapy (ADT) with or without bicalutamide; those in the high-risk group received upfront treatment involving six cycles of DOC (70 mg/m2). Short-term treatment effect, adverse events, and quality of life (QOL) were evaluated. Fifty (73.5%) were classified in the high-risk group, and 46 (67%) received upfront ADT + DOC. In the ADT + DOC group, 43.5% (20/46) patients achieved a PSA level ≤ 0.2 ng/mL. PSA nadir and time to PSA nadir were 0.291 ng/mL and 288 days, respectively. In the ADT + DOC group, 76.1% (35/42) patients had adverse events (AEs) of grade ≥ 3. During a median follow-up of 18.5 months, 36.4% (8/22) patients in the ADT group and 43.5% (20/46) in the ADT + DOC group had CRPC. Two QOL scores including the physical status and appetite loss at 6 months significantly worsened in the ADT + DOC group but was resolved by 12 months. Upfront DOC achieved high PSA responses without long-term QOL deterioration. However, the short-term outcomes were limited. Longer follow-up is needed to determine the survival advantage.

[Tuberous Sclerosis Complex Detected by Spontaneous Rupture of a Giant Renal Angiomyolipoma : A Case Report].

A woman in her 30s was admitted with abdominal pain and nausea. CT scan revealed a spontaneous rupture of the right giant renal angiomyolipoma, and trans-arterial embolization was performed successfully. With further examination, she was found to be affected with tuberous sclerosis complex (TSC) and she finally wastreated with everolimusfor prevention of recurrent spontaneous-rupture of renal angiomyolipoma.

Efficacy and safety of bladder hydrodistension for decreased bladder capacity induced by intravesical BCG therapy.

OBJECTIVE: Intravesical BCG therapy is widely used for the treatment of high-risk, non-muscle-invasive bladder cancer. Among various reported side-effects, decreased bladder capacity is a serious side-effect that significantly worsens patients' quality of life. This article reports the efficacy and safety of bladder hydrodistension (BHD) in six patients with seriously decreased bladder capacity caused by BCG treatment. METHODS: Six patients with low bladder capacity (<100 ml in voiding diaries) and complaint of grade 3 irritative symptoms were diagnosed with decreased bladder capacity and treated with BHD. Alleviation of symptoms was defined as medication being discontinued or reduced after BHD. RESULTS: Five patients were male and one was female, and the mean age was 67.7 years. The mean interval between the last transurethral resection and BCG therapy was 26.0 days. Before BHD, all patients had been treated with antibiotics, anticholinergics and non-steroidal anti-inflammatory drugs (NSAIDs). The median bladder capacity before treatment was 40 ml (range 30-100 ml), and the median capacity increased to 200 ml (175-250 ml) within 2 weeks following BHD therapy. Four patients stopped NSAID use and three patients stopped anticholinergic use. One patient needed total cystectomy for recurrent symptoms. With a median follow-up period of 32 months, the bladder capacity remained stable without symptomatic deterioration in the remaining five patients. There was neither tumor spread nor disseminated tuberculosis infection. CONCLUSIONS: BHD appears to be an effective treatment option in patients with severely decreased bladder capacity. Its efficacy and safety were acceptable.

COMPARISON BETWEEN EXTRACORPOREAL SHOCK WAVE LITHOTRIPSY AT 120 AND 60 SHOCKWAVES PER MINUTE FOR TREATMENT OF URINARY STONES.

(Purpose) It has recently been suggested that a slow delivery rate of shockwaves by extracorporeal shock wave lithotripsy (SWL) improved treatment outcomes for urinary stones. We retrospectively analyzed the treatment outcomes of different shockwave delivery rates at 120 and 60 shockwaves per minute. (Patients and method) A total of 88 patients were treated at a fast delivery rate of 120 shockwaves per minute between July 2010 and April 2012, and 139 patients were treated at a slow delivery rate of 60 shockwaves per minute between May 2012 and May 2014 (n=227) using a Sonolith® Praktis lithotripter. The treatment outcome of stone-free rate (SFR) after one SWL session was assessed at four weeks. (Result) SWL at 60 shockwaves per minute resulted in a significantly higher SFR compared with SWL at 120 shockwaves per minute (39.8% and 59.0%, respectively, p=0.0047), particularly for upper ureter (U1) stones (53.1% and 72.0%, respectively, p=0.028). Multivariate analysis showed that younger age, stone sizes of 10 mm or less, U1 stones, and slow delivery rate were significant predictors of a stone-free outcome. There were fewer adverse events after the delivery rate of 60 shockwaves per minute (p=0.058). (Conclusion) Our study suggests that SWL at 60 shockwaves per minute should be recommended to successfully treat urinary stones using the Sonolith® Praktis lithotripter.

[PRIMARY MEDIASTINAL GERM CELL TUMOR ARISING IN A PATIENT WITH NEUROFIBROMATOSIS TYPE 1].

Neurofibromatosis type 1 (NF1) is a distinct genetic disorder due to the NF1 gene mutation which induces the aberrant activation of the RAS-signaling. Because RAS-related proteins function as oncogenic factors, NF1 patients frequently develop malignant tumors, especially of neural crest origin, such as peripheral nerve sheath. In addition, malignant tumors of the pancreas, colorectum, and lung have been reported to frequently arise in NF1 patients. However, the association between germ cell tumor and NF1 has not been clarified yet. A 29-year-old male with dyspnea was referred to our hospital because of the large mass in the anterior mediastinum and cervical lymph node swelling. The diagnosis was extragonadal germ cell tumor with cervical lymph node metastasis, and complete remission was obtained by multidisciplinary treatment consisted of combination chemotherapy and surgical resection. To our acknowledgement, this is the first case of extragonadal germ cell tumor in NF1 patients. We discuss the relevance between activation of the RAS-signaling and the development of germ cell tumor.

Clinical benefits of tubeless umbilical cutaneous ureterostomy.

INTRODUCTION: We assess a novel technique of tubeless bilateral cutaneous ureterostomy, with a single umbilical stoma, for bladder cancer patients with short ureters after cystectomy. The benefit of cutaneous ureterostomy is equal to other incontinent urinary diversions, when the tubeless procedure is successfully achieved. This simple technique makes it easy to monitor the upper urinary tract (UUT) and is beneficial to patients with a high risk of UUT recurrence. METHODS: This old and new surgical technique was used to perform total cystectomy and urinary diversion on three patients with bladder cancer at a high risk of UUT recurrence. RESULTS: Two men and one woman (mean age: 73 years) underwent this surgery and the mean follow-up period was 8.3 years. The surgical approaches were laparotomy (n = 2) and laparoscopy (n = 1). One case developed para-stomal erosion, whereas another developed ureteral stenosis requiring catheter reinsertion. Although postoperative hydronephrosis was observed in all cases, the mean preoperative and postoperative serum creatinine levels were 0.70 and 0.76, respectively. UUT recurrence was not observed during the follow-up period. CONCLUSION: This tubeless umbilical cutaneous ureterostomy procedure greatly improves the outcome of urinary diversion for cancer patients with short ureters at a high risk of UUT recurrence. The benefits are equivalent to other urinary diversions when the tubeless procedure is successfully achieved.

[Pazopanib for three patients with recurrence of retroperitoneal liposarcoma : initial clinical experience].

Pazopanib, a novel tyrosine kinase inhibitor, is an effective therapeutic agent for patients with advanced soft tissue sarcoma. Here we report three patients with recurrent retroperitoneal liposarcoma who were treated with pazopanib. Case 1: A 54-year-old male received three courses of combined chemotherapy consisting of doxorubicin and ifosfamide for recurrent left retroperitoneal dedifferentiated liposarcoma and liver metastasis following tumor excision. Because of the lack of response to chemotherapy, 400 mg/day of pazopanib was subsequently administered for two weeks. The patient died 3 weeks after the initiation of pazopznib therapy. Case 2: A 78-year-old male with right retroperitoneal dedifferentiated liposarcoma underwent irradiation for a recurrent tumor 16 months after the initial tumor excision. Pazopanib (600 mg/day) was partially effective for 2 months. Pazopanib was administered for 7 months, but the patient died 8 months after the initiation of pazopanib therapy. Case 3 : An 80-year-old male with locally recurrent right retroperitoneal myxoid liposacroma was treated with 600 mg/day of pazopanib from 5 months after tumor excision. He remains alive and has had stable disease for 17 months to date. In conclusion, pazopanib may be effective in a subset of patients with recurrent retroperitoneal liposarcoma.

Characterization of clinical and genetic risk factors associated with dyslipidemia after kidney transplantation.

We determined the prevalence of dyslipidemia in a Japanese cohort of renal allograft recipients and investigated clinical and genetic characteristics associated with having the disease. In total, 126 patients that received renal allograft transplants between February 2002 and August 2011 were studied, of which 44 recipients (34.9%) were diagnosed with dyslipidemia at 1 year after transplantation. Three clinical factors were associated with a risk of having dyslipidemia: a higher prevalence of disease observed among female than male patients (P = 0.021) and treatment with high mycophenolate mofetil (P = 0.012) and prednisolone (P = 0.023) doses per body weight at 28 days after transplantation. The genetic association between dyslipidemia and 60 previously described genetic polymorphisms in 38 putative disease-associated genes was analyzed. The frequency of dyslipidemia was significantly higher in patients with the glucocorticoid receptor (NR3C1) Bcl1 G allele than in those with the CC genotype (P = 0.001). A multivariate analysis revealed that the NR3C1 Bcl1 G allele was a significant risk factor for the prevalence of dyslipidemia (odds ratio = 4.6; 95% confidence interval = 1.8-12.2). These findings may aid in predicting a patient's risk of developing dyslipidemia.

Successful introduction of laparoendoscopic single-site donor nephrectomy after experience with laparoscopic single-site plus-one trocar donor nephrectomy.

PURPOSE: To assess the feasibility, safety, and efficacy of the laparoendoscopic single-site (LESS) donor nephrectomy (LESSDN) procedure after experience with the LESS-plus-one-trocar donor nephrectomy (LEPODN) procedure. PATIENTS AND METHODS: From 2009 to 2014, 126 left laparoscopic donor nephrectomies (LDNs) were performed, including 59 Standard (Std)-LDN, 30 LEPODN, and 37 LESSDN. In the LEPODN procedure, a 5-mm trocar was added as a right-hand working trocar to the LESSDN procedure. A GelPOINT(®) platform was applied on a pararectal single incision in both LEPODN and LESSDN procedures. After performing the LEPODN procedure several times, each surgeon performed the LESSDN procedure. RESULTS: Std-LDN, LEPODN, and LESSDN procedures were performed by 10, 10, and 7 surgeons, respectively. The mean operative time, estimated blood loss, warm ischemia time, time to ambulation, and length of postoperative hospital stay were the shortest for the LESSDN procedure (P<0.012, P=0.007, P<0.001, P=0.027, and P=0.001, respectively). No significant difference in the complication rate, delayed graft function rate, and mean 7-day post-transplant serum creatinine levels was observed among the three procedures. Individual results of the operative time and estimated blood loss for the LESSDN procedure were not significantly inferior to those of Std-LDN and LEPODN procedures for each surgeon. CONCLUSIONS: The LESSDN procedure can be introduced safely and effectively without compromising the operative time, complication rate, and graft function after experience with the LEPODN procedure among multiple surgeons. The LEPODN procedure may be an effective bridge from standard multiport LDN to LESSDN.

Effects of functional genetic polymorphisms in the CYP19A1 gene on prostate cancer risk and survival.

CYP19 catalyzes the conversion of androgens to estrogens and is a critical enzyme affecting the sex hormone milieu. In this study, we investigated the functions of CYP19A1 polymorphisms and their associations with prostate cancer risk and clinical outcome. This case-control study evaluated the effects of three single nucleotide polymorphisms (SNPs) in CYP19A1 on the risk of prostate cancer in 330 prostate cancer patients and 354 normal controls. The associations between each SNP and sex hormone levels were evaluated in 164 healthy male patients. The functions of the SNPs were determined by reporter gene assays in PC3 and DU145 cell lines. Prostate-specific antigen nadir was evaluated in 142 patients with metastatic prostate cancer treated with androgen deprivation therapy. Cancer-specific survival (CSS) was determined in 166 patients with metastatic prostate cancer, to evaluate the influence of the three SNPs. Each variant allele of the three SNPs significantly decreased the risk of prostate cancer. Haplotype analysis showed that the T-A-G haplotype (corresponding to rs2470152-rs10459592-rs4775936) increased the risk of prostate cancer, while the C-C-A haplotype decreased the risk. The estrone/androstenedione ratio was significantly higher in men with the C allele of rs2470152, the C allele of rs10459592, and the A allele of rs4775936 in a gene-dosage-dependent manner. Patients with the variant allele at rs4775936 had significantly shorter CSS. These results indicate that CYP19A1 polymorphisms may influence prostate cancer risk and survival by modifying promoter activity, with subsequent effects on the sex hormone milieu.

Renal subcapsular fluid collection caused by penetration of a pancreatic pseudocyst.

A 63-year-old man presented with left flank pain and spiked fever. Computed tomography revealed a pancreatic cyst and left renal subcapsular fluid collection that appeared to be connected to the cyst. High levels of amylase and lipase were observed in a test puncture of renal fluid collection. The cause of the fluid collection was diagnosed as penetration of the pancreatic pseudocyst. Endoscopic nasobiliary drainage was used to drain the pancreatic pseudocyst and renal subcapsular fluid collection. The present case demonstrated that renal subcapsular fluid collection may be caused by penetration of a pancreatic pseudocyst.

De novo renal cell carcinoma in an allograft kidney treated with nephron-sparing surgery: a case report.

The development of primary malignant tumors is a distressing complication of organ transplant. However, the emergence of de novo renal cell carcinoma from a kidney allograft is rare. A 60-year-old man underwent living kidney transplant from a spousal donor. Six years after the transplant surgery, computed tomographic evaluation confirmed the presence of a 2.8-cm-diameter solid mass in the lower pole of the allograft. Partial allograft nephrectomy was performed, and the margin surrounding the normal parenchyma was resected. The serum level of creatinine did not decrease. Here, we report a case of renal cell carcinoma in an allograft kidney that was successfully treated with nephron-sparing surgery.

Successful local control of recurrent penile cancer treated with a combination of systemic chemotherapy, irradiation, and mohs' paste: a case report.

Penile squamous cell carcinoma (pSCC) is a rare disease, making it difficult to establish a standard of care, particularly in the advanced stage. We report a case of pSCC with advanced lymph node metastasis treated with multimodal therapy consisting of combination chemotherapy, irradiation, and chemosurgery using Mohs' zinc chloride-containing paste. An 80-year-old male with a past history of local treatment for penile cancer presented with a large painful inguinal mass with an ulcer and exudates. The patient underwent multimodal treatment with combination chemotherapy, irradiation, and Mohs' paste. The combination chemotherapy consisted of cisplatin, 5-fluorouracil, and docetaxel. The patient received 50-Gy external-beam radiation therapy to the left inguinal region along with daily local treatment with Mohs' paste. After the initiation of treatment, the pain and bleeding in the inguinal region considerably ameliorated. The wound became dry and flattened 20 days after the initiation of chemotherapy. A CT scan showed that the tumor had decreased 70% in diameter 1 month after the initiation of chemotherapy. After the first course of chemotherapy, the patient and his family decided not to continue treatment because of socio-economic reasons. The patient underwent no additional treatments; nevertheless, he had no local progression of the inguinal tumors for 8 months. We report a case of successful local control of recurrent inguinal pSCC treated with multimodal therapy. Combination treatment with taxane-based chemotherapy, external-beam radiation therapy, and Mohs' paste is an option for the management of recurrent pSCC.

Successful mammalian target of rapamycin inhibitor maintenance therapy following induction chemotherapy with gemcitabine and doxorubicin for metastatic sarcomatoid renal cell carcinoma.

This study presents a case of metastatic sarcomatoid renal cell carcinoma (RCC) treated with systemic chemotherapy followed by mammalian target of rapamycin inhibitor maintenance therapy. A 63-year-old male presented with lumbago, and lumbar vertebral tumors were detected by magnetic resonance imaging. Subsequent computed tomography (CT) revealed a right renal tumor and CT-guided biopsy of the right renal and left sacroiliac tumors determined pure sarcomatoid carcinoma without a clear cell component. Two cycles of combination chemotherapy comprising of gemcitabine (1,500 mg/m2 on day one) and doxorubicin (50 mg/m2 on day one) resulted in a 20% reduction in the longest diameter of the right renal tumor. However, due to grade 3 neutropenia, the chemotherapy was discontinued and temsirolimus (25 mg once weekly), which binds to the cytoplasmic protein, FKBP-12, and inhibits mTOR, was administered. Stable disease was maintained for 19 months with temsirolimus and no major adverse events, with the exception of grade 2 nausea, were observed. The patient succumbed to their disease at 30 months following the initiation of treatment. These results suggested that systemic chemotherapy followed by temsirolimus maintenance is a feasible treatment option for patients with metastatic sarcomatoid RCC.

Functional mononucleotide repeat polymorphism in the promoter region of HGF is associated with risk and malignant aggressiveness of bladder cancer.

Increased expression of hepatocyte growth factor (HGF) has been shown to be associated with aggressiveness in several types of cancer. Shorter variants of deoxyadenosine tract element (DATE) located in the HGF promoter region have been reported to enhance the expression of HGF. In this study, we investigated the role of HGF DATE variants in bladder cancer risk, HGF expression and clinicopathological features. The frequency of individuals with a short DATE (<28 repeats) in peripheral blood lymphocytes (PBLs) was significantly higher in bladder cancer patients compared to controls (p<0.001). Somatic mutations were observed in 37 of 70 bladder tumor (BT) tissues and the frequency of mutation to long DATE was significantly higher than that to short DATE (p=0.047). The presence of the short DATE in BT tissue was significantly associated with higher tumor grade (p=0.015). HGF mRNA levels were significantly higher in pT2 tumors than pTa or pT1 tumors (p=0.019), and in grade 3 tumors than grade 1 or 2 tumors (p=0.020). Furthermore, BT tissues with the short DATE showed significantly higher levels of HGF mRNA (p<0.001). In patients who underwent radical cystectomy, those with higher HGF expression had a significantly shorter overall survival than those with lower HGF expression (p=0.012). In conclusion, HGF may be associated with the prognosis of patients who undergo radical cystectomy, and the HGF DATE may affect the risk and aggressiveness of bladder cancer by altering HGF expression.

Bone marrow-derived cells mobilized by granulocyte-colony stimulating factor facilitate vascular regeneration in mouse kidney after ischemia/reperfusion injury.

Bone marrow-derived cells (BMDC) play crucial roles in tissue regeneration. Granulocyte-colony stimulating factor (G-CSF) mobilizes BMDC and may facilitate the repair of kidney tissues after ischemia/reperfusion (I/R) injury. The tissue protective action of resveratrol, an antioxidant, might modify the regenerating potential of BMDC in I/R renal injury. This study examined whether G-CSF and/or resveratrol affect the recruitment of BMDC into vascular endothelial cells and renal tubular cells and the kidney function after I/R injury. I/R renal injury was induced in female mice that had been lethally irradiated and transplanted with male bone marrow cells. The mice were given saline, resveratrol or G-CSF, daily for 7 days. Non-irradiated and non-bone-marrow-transplanted female mice, which underwent the same kidney injury, were included as control. White blood cell (WBC) count and serum creatinine were monitored. Immunohistologic evaluation for renal tubular cells (cytokeratin) and endothelial cells (factor VIII-related antigen), and fluorescence in situ hybridization for mouse Y chromosome were performed. Although WBC was significantly higher in the G-CSF group, there was no significant difference in creatinine levels among all groups. Factor VIII-related antigen-positive cells with a Y-chromosome signal were identified in the capillary wall between renal tubuli and most frequently seen in the G-CSF group (p < 0.0001). Resveratrol did not affect kidney recovery in this model. No cytokeratin-positive renal tubular cells having a Y-chromosome signal were identified. In conclusion, BMDC are recruited into endothelial cell in I/R renal injury without apparent renal tubular cell regeneration, and G-CSF facilitates the endothelial cell regeneration.

[A case of organ sparing surgery for metachronous bilateral testicular tumor with maintaining testicular function].

A 28-year-old man, who had undergone right orchiectomy and prophylactic irradiation for stage I seminoma 6 years ago, developed left testicular tumor. Since the secondary tumor was localized in the lower pole of the testis, partial orchiectomy was performed with an attempt to preserve the testicular function. The pathological finding of the surgical specimen was a mixed type testicular tumor consisting of seminoma, embryonal carcinoma and teratoma elements. Postoperative chemotherapy with 3 courses of BEP regimen resulted in azoospermia, but the impaired spermatogenesis recovered to a normal range within 18 months with no evidence of tumor recurrence and his wife delivered a healthy baby 2 years later. For the synchronous or metachronous bilateral testicular tumors, the combination of organ sparing surgery and chemotherapy could be a treatment of choice.

[Rapidly growing renal angiomyolipoma during pregnancy with tumor thrombus into the inferior vena cava: a case report].

A case of renal angiomyolipoma rapidly growing during pregnancy with tumor thrombus into the inferior vena cava is reported. Angiomyolipoma in a 31-year-old woman was incidentally found by ultrasonography. CT scan revealed a fat-containing tumor in the right kidney with 4 cm in diameter. The patient was followed at outpatient clinic without any treatment. Fifteen months later, the post-delivery follow-up CT scan showed that tumor size had grown up to 11 cm in diameter. Although laparoscopic right nephrectomy was tried, open transperitoneal right nephrectomy was performed because the tumor thrombus extending into the inferior vena cava was found during the laparoscopic procedure. Pathological diagnosis was angiomyolipoma of the kidney. There are several reports that indicate angiomyolipoma grows rapidly during pregnancy. Our case demonstrates that careful follow-up is required for angiomyolipoma in women with possible conception and delivery.