A case of primary aldosteronism with excessive secretion of renin that was unmasked by kidney transplantation.

A 42-year-old man showed marked hypokalemia after kidney transplantation. He was diagnosed with hypertension and suffered from acute myocardial infarction at 33 and 38 years of age. At 40 years of age, hemodialysis was introduced. A left adrenal tumor was noted and suspected as a non-functional adrenal adenoma at that time. Therefore, he received a living-donor kidney transplant at 42 years of age. After kidney transplantation, the serum creatinine level dropped. His blood pressure remained high, and the serum potassium level decreased. The PRA and PAC were elevated, and ARR was not elevated. Based on the results of various confirmatory tests and vein sampling, he was diagnosed with excessive secretion of renin from the native kidneys that was complicated by primary aldosteronism (PA), and left nephrectomy and adrenalectomy were performed. The overproduction of aldosterone in the resected adrenal adenoma and over secretion of renin in the kidney with arteriolosclerosis were immunohistologically confirmed. After surgery, the PAC decreased, but the PRA did not decrease. The postoperative serum potassium level improved, and the blood pressure was well controlled with a small dose of medication. This is the first reported case of PA with hyperreninemia after kidney transplantation. It should be noted that PA in dialysis patients and kidney transplant recipients may not fulfill the usual diagnostic criteria of an elevated ARR. In such patients, PA should be suspected based on the absolute value of the PAC and responsiveness to ACTH stimulation, and adrenal and renal vein sampling is required for a definitive diagnosis.

Hepatic and lung methotrexate-associated polymorphic lymphoproliferative disorders arising during postoperative follow-up of renal cell carcinoma: a case report.

INTRODUCTION: Methotrexate induces lymphoproliferative disorders on rare occasions; however, its pathogenesis remains unknown. A clinical diagnosis based on imaging studies alone is often difficult. CASE PRESENTATION: A 57-year-old Japanese woman was referred to our department for the evaluation of multiple lung and hepatic nodules that developed during methotrexate treatment for rheumatoid arthritis. Since she had a history of nephrectomy for localized renal cell carcinoma, multiple lung and hepatic metastases were initially considered. However, pathological diagnosis of the lung nodules (needle biopsy) revealed methotrexate-associated polymorphic-type lymphoproliferative disorders. After methotrexate discontinuation, continuous smooth shrinkage of the lung and liver lymphoproliferative disorders was observed. CONCLUSION: Methotrexate-associated lymphoproliferative disorders should be considered in the event of newly appearing neoplastic lesions, even during follow-up for renal cell carcinoma, if methotrexate is being administered.

Epithelioid Angiomyolipoma with Tumor Thrombus into Inferior Vena Cava Presurgically Treated with Combination Therapy of Pembrolizumab and Axitinib: A Case Report.

Epithelioid angiomyolipoma (EAML) is a rare variant of AML with malignant potential. It is occasionally difficult to distinguish EAML from renal cell carcinoma (RCC) on imaging. A 72-year-old woman was admitted to our hospital for the treatment of a left renal tumor with relatively high blood flow and a tumor thrombus extending to the inferior vena cava, suggesting RCC. The patient underwent presurgical combination therapy with axitinib and pembrolizumab. This treatment significantly shortened the thrombus, and radical nephrectomy was performed. The pathological findings were compatible with EAML, and the treatment effects were observed. We report a case treated pre-surgically with a combined therapy of pembrolizumab and axitinib, with a favorable response as a treatment option for EAML.

A case of von Hippel-Lindau disease with renal cell carcinoma treated by partial nephrectomy with pre- and post-surgical axitinib therapy.

von Hippel-Lindau (VHL) disease is an autosomal dominant hereditary disease with benign and malignant tumors occurring in various organs including the kidneys. In patients with renal cell carcinoma (RCC) lesions in both kidneys, it is difficult to determine the treatment strategy. We report a case of VHL disease with RCC treated via partial nephrectomy after 6 months of axitinib therapy. Then, the patient continued to receive low-dose axitinib therapy without any signs of tumor progression for 3 years after surgery. Axitinib combined with surgery might be a treatment option for patients with VHL disease harboring bilateral RCC.

[A Case of Stauffer Syndrome-Like Findings Associated with Metastatic Renal Cell Cancer Improved by Molecular Targeted Therapy].

We report a case of Stauffer syndrome-like findings in a patient with metastatic renal carcinoma treated by surgery and molecular targeted therapy. The patient was a 58-year-old woman diagnosed with renal carcinoma with multiple metastases. She had hepatosplenomegaly and hepatic dysfunction with elevated serum liver enzyme and IL-6 levels. Treatment with temsirolimus and axitinib reduced the size of the local and metastatic tumors and simultaneously improved the hepatosplenomegaly. The local tumor was excised by laparoscopic nephrectomy, treated with axitinib and then with nivolumab. With the reduction in the metastatic tumor size, serum liver enzyme and IL-6 levels decreased. It was suggested that molecular targeted therapy is an effective treatment when the findings of metastatic renal cell carcinoma, are similar to those of Stauffer syndrome.

[A Case of Neuroendcrine Carcinoma of the Prostate Diagnosed by Prostate Re-Biopsy during Hormonal Treatment and Effectively Treated by Multidisciplinary Therapy].

A 73-year-old Japanese man visited the urology clinic with the chief complaint of gross hematuria in June 2015. His prostate specific antigen (PSA) level was 146.7 ng/ml and he was diagnosed with prostate adenocarcinoma with a Gleason Score of 5+4. With bone metastasis in the right femur (cT3aN0M1), he was treated by orchiectomy and bicalutamide. He had gross hematuria in October 2017 and a prostate tumor was detected by computed tomography (CT) and magnetic resonance imaging without increasing PSA levels. Prostate re-biopsy showed prostate neuroendocrine carcinoma and local radiation therapy (74 Gy) was performed. Follow-up CT revealed a left adrenal tumor with a positive positron emission tomographic scan in October 2018. Under the diagnosis of metastatic neuroendocrine carcinoma, chemotherapy using cisplatinum and etoposide was performed. The tumor shrunk after five courses of treatment, followed by regrowth in April 2019. Radiation therapy (50 Gy) was added to the left adrenal tumor and it shrunk again. However, a left retroperitoneal tumor was detected in July 2019 and it was resected under laparoscopic surgery and diagnosed as metastatic neuroendocrine carcinoma. Since then, no recurrence has been observed.

Trans-tract electrocoagulation can decrease the need for postoperative nephrostomy tube after endoscopic combined intrarenal surgery.

AIM: To assess the efficacy and safety of trans-tract electrocoagulation at the end of endoscopic combined intrarenal surgery for renal or ureteral stones. METHODS: The present study included patients who underwent endoscopic combined intrarenal surgery from May 2010 to March 2018. After June 2013, the trans-tract electrocoagulation procedure, to coagulate bleeding from the access tract using a resectscope was carried out at the end of the operation. We compared the patients' background and surgical outcomes between patients with and without trans-tract electrocoagulation. RESULTS: Between the trans-tract electrocoagulation (n = 225) and non-trans-tract electrocoagulation (n = 72) groups, the stone number was significantly smaller (1:2:3 or more, 126:72:27 vs 59:10:3, P = 0.001) and the initial stone-free rates were significantly higher (80% vs 72%, P = 0.006) in the trans-tract electrocoagulation group than in the non-trans-tract electrocoagulation group. Patients experienced a higher nephrostomy tube-free rate (67% vs 26%, P < 0.0001), shorter postoperative catheterization time (2.8 ± 3.8 vs 5.4 ± 5.0 days, P = 0.002) and shorter hospital stay (6.5 ± 3.6 vs 8.8 ± 5.0 days, P = 0.0001) in the trans-tract electrocoagulation group than in the non-trans-tract electrocoagulation group. CONCLUSIONS: Trans-tract electrocoagulation in endoscopic combined intrarenal surgery is a safe and efficient procedure that decreases the need for nephrostomy tube placement after surgery.

The long-term prognosis of nephropathy in operated reflux.

OBJECTIVE: To investigate the postoperative long-term prognosis and the factors predicting the renal function of patients with reflux nephropathy. As the serum creatinine (s-Cr) level tends to increase during infancy, the degree of reflux and renal parenchymal damage are thought to be more important factors in pediatric patients than in older patients. MATERIALS AND METHODS: This study examined s-Cr, urinary protein, and blood pressure of patients who underwent anti-reflux surgery 10 years before. It also calculated the postoperative estimated glomerular filtration rate (eGFR) and examined the correlation between the eGFR and preoperative factors (age, gender, number of urinary tract infections [UTIs], primary diagnosis, reflux grade, percentage of dimercaptosuccinic acid uptake, degree of renal parenchymal damage, s-Cr abnormality, proteinuria, and hypertension), and analyzed the factors associated with the long-term prognosis. RESULTS: The study population was 51 infants (37 boys and 14 girls). The mean age of the patients before surgery and at the follow-up examination was 3.41 ± 3.61 and 14.63 ± 3.74 years, respectively. After surgery, the s-Cr, urinary protein, and blood pressure values showed (44.7%, 26.7%, and 18.2%, respectively) were abnormal. The postoperative eGFR was a mean 90.27 ± 20.42 ml/min/1.73 m2 and primary correlated with an older age (P = 0.0361), no UTI at the primary diagnosis (P = 0.0044), reflux grade ≥8 (P = 0.0180), degree of renal parenchymal damage (group ≥2b, P < 0.0001), s-Cr abnormality (P < 0.0001), and proteinuria (P = 0.0001) at baseline. A total of 20 patients had chronic kidney disease (CKD; Fig. 1). The multiple regression analysis of these factors revealed that an older age (P = 0.0021), reflux grade ≥8 (P = 0.0134), and degree of renal parenchymal damage (group ≥2b, P < 0.0001) were significantly associated with the long-term postoperative prognosis of reflux nephropathy. Using these three factors, this study derived a multiple regression equation estimating eGFR in the 10th year after surgery (Fig. 1). DISCUSSION: In this study, severe vesico-ureteral reflux (reflux grade ≥8) and severe renal parenchymal damage (group ≥2b) were associated with a long-term decrease in the eGFR. In particular, renal parenchymal damage was closely correlated with the postoperative eGFR; thus, this was clearly a critical factor. The age at surgery showed a better correlation with the postoperative eGFR in the multiple regression analysis; thus, age was regarded as an independent prognostic factor. CONCLUSIONS: The age, reflux grade, and degree of renal parenchymal damage at baseline were factors that affected the long-term postoperative prognosis of reflux nephropathy. Patients with high-grade reflux and severe renal parenchymal damage were more likely to show a reduced CKD level at 10 years after anti-reflux surgery.

Safe Resection of Renal Cell Carcinoma with Liver Invasion Using Liver Hanging Technique Supported by Preoperative Portal Vein Embolization.

In cases of RCC with liver involvement, partial hepatectomy is known to provide a better chance of survival for patients. For this reason, complete resection with clear surgical margin is thought to be necessary to achieve favorable outcome. Anterior liver hanging maneuver was extremely useful during hemihepatectomy in this rare type of RCC. A 63-year-old male was diagnosed with a large right renal cell carcinoma. The tumor measured 10 cm in diameter with tumor thrombus toward the inferior vena cava (IVC). In addition, we observed direct infiltration to the liver. We attempted a preoperative portal vein embolization (PVE) to preserve residual liver volume and function after right lobectomy. After PVE the resected volume decreased from 921 cm3 (71%) to 599 cm3 (53.4%). During the procedure, a nasogastric tube was placed in the retrohepatic space for liver hanging maneuver according to the original Belghiti's maneuver after dissection of the renal artery and vein. After hepatic parenchymal transection exposing vena cava, the right hepatic veins were safely transected using vascular stapler; right nephrectomy and hemihepatectomy were performed. The patient recovered without postoperative hepatic or urinary complications and has remained free of local recurrence and any de novo metastasis for 18 months.

Accompanying role of hepato-biliary-pancreas surgeon in urological surgery.

INTRODUCTION: The present case reports demonstrated the accompanying surgical support from hepato-biliary-pancreas (HBP) surgeons for urological surgery to secure operative safety because HBP surgeons are well experienced in dissecting techniques for mobilization of the liver or pancreas. We experienced 9 consecutive patients who underwent nephrectomy, adrenectomy or resection of retroperitoneal tumors by urological surgeons. Cardiovascular intervention was also required in cases of long tumor thrombus into the vena cava. CASES: All patients had no severe co-existing diseases except the main tumor. Reverse T-shape incision was performed in 7 cases and thoracolaparotomy in two. Dissection and mobilization at the site of severe compression by the urinary tumors were performed in three cases. Partial liver resection was performed for testicular liver metastases in two, and right hepatectomy for right renal cancer was performed in one. Encircling the vena cava and preparation of transection for tumor thrombi were performed in three, and among these, cardiovascular intervention was necessary in two because of extension into the right atrium. During admission, all patient outcomes were uneventful without severe complications. We herein showed the representative two cases of combined surgery. DISCUSSION: and conclusion The point of this case report is the coordination between each surgeon and anesthesiologist under precise perioperative planning or management. The role of HBP surgeons is to provide information as a specialist on the operative field for urological or cardiovascular surgery to achieve operative safety.

Surgical intervention for renal cell carcinoma with inferior vena cava extension combined with laparoscopic procedure.

Clear cell renal cell carcinoma (ccRCC) shows extreme hypervascularity, which may cause significant bleeding during surgery. For this reason, control of arterial blood supply is an important factor in the choice of operative procedure and in avoiding perioperative complications. This case series reports the successful dissection of renal artery in the preliminary stage of laparoscopic procedure in three ccRCC patients with inferior vena cava (IVC) extension. Patient 1 had right renal cell carcinoma (RCC) with level I tumor thrombus through two renal veins, and the renal artery was successfully dissected by retroperitonealscopic approach. Patient 2 had right invasive, immobilized RCC with significant infiltration to IVC and liver. Ligation of renal artery was performed by transperitoneal laparoscopic procedure. Patient 3 had left RCC with level III tumor thrombus and lung metastasis. Ligation of left renal artery and mobilization of peritoneal organs and kidney were performed by transperitoneal laparoscopic surgery. These cases suggest that combined laparoscopic-open surgery for RCC with IVC extension may facilitate early control of arterial blood supply.

Myopericytoma in urinary bladder: a case report.

BACKGROUND: Myopericytoma is reported to occur mainly in the skin and superficial soft tissue of the extremities. In contrast, occurrence in the urinary bladder is extremely rare. CASE PRESENTATION: We describe a 75-year-old Japanese man who developed a submucosal tumor at the right trigone of his bladder that led to interference with the discharge of right ureteral calculus. No invasive growth was observed by magnetic resonance imaging. Transurethral resection was successfully performed; histopathological analysis revealed perivascular proliferation of spindle-shaped to oval-shaped, cytologically bland tumor cells with eosinophilic cytoplasm. On immunohistochemical examination, the tumor cells were positive for alpha-smooth muscle actin, desmin, CD34 and h-caldesmon. CONCLUSION: Cystoscopic and pathological findings were compatible with a diagnosis of myopericytoma of the urinary bladder.

Expression of human kallikrein 1-related peptidase 4 (KLK4) and MET phosphorylation in prostate cancer tissue: immunohistochemical analysis.

Resistance to or relapse after androgen deprivation therapy (ADT) remains a significant problem in patients with prostate cancer. Several studies have hypothesized that the overexpression of MET and the activating signaling axis in prostate cancer cells are associated with castration-resistant prostate cancer (CRPC). On the other hand, the expression of human kallikrein 1-related peptidase (KLK) 4, which activates plasma HGF activator zymogen, in prostate cancer patients with bone metastasis or advanced stage has also been reported. In this study, we analyzed the expression and phosphorylation of MET along with KLK4 by immunohistochemistry in 62 formalin-fixed paraffin-embedded sections of prostate cancer collected by needle biopsy at our hospital between 2006 and 2011. As a result, the phosphorylation of MET was observed in 56% (35 of 62 cases) and positively correlated with worsening PSA relapse rate in a group of ADT-treated patients (P = 0.0445), suggesting significant correlation with CRPC. Overexpression of KLK4 was observed in patients with high T stage (P = 0.0001) and high Gleason score (P = 0.0146), and the expression was correlated with the phosphorylation of MET (P = 0.0002). Pathologically, strong MET phosphorylation observed in specific architectures in prostate cancer, such as cribriform structures, ill-defined glands or solid patterns, suggested the significance of MET activation in promoting the architectural formation of prostate cancer. In addition, high positivity rate (80%) of phospho-MET staining at high-grade prostatic intraepithelial neoplasia (HGPIN) may indicate the importance of the activating signaling axis in the carcinogenesis of prostate cancer.

[Analysis of multimodal treatment outcome in renal cell carcinoma patients with bone metastasis].

We retrospectively reviewed the treatment outcome of 12 renal cell carcinoma (RCC) patients with bone metastasis who had undergone targeted therapy along with a regimen of multimodal treatment. We performed metastatectomy following nephrectomy on patients with resectable foci. Patients with unresectable bone metastasis underwent radiation therapy. With the exception of two patients, zoledronic acid was also administered. As a result, the overall survival rates of 83.3% at 1 year and 72.9% at 2 years in our study compared favorably with reported cases. Patients with complete resection of solitary bone metastasis with or without extrabone lesions had favorable outcomes. As differences in the response of the targeted therapy can be observed between bone and extrabone metastases, control of bone metastasis may be a key factor for the prognosis of metastatic RCC patients.

[Yoke technique for severe proximal hypospadias].

UNLABELLED: PROPOSED: Various techniques have so far been reported for the repair of hypospadias, however, a one-stage procedure for the repair of severe proximal hypospadias still remains difficult to perform. We recently have begun to use the Yoke hypospadias repair technique for the treatment of severe proximal hypospadias. PATIENTS AND METHODS: As the chief surgeon, I performed a one-stage hypospadias repair on 40 proximal hypospadiac patients with severe fibrous chordee between July 1992 and December 2004. During the early period, eleven patients underwent urethroplasty by the Transverse Preputial Island Flap techinique (TPIF). Next, 10 patients underwent One-stage Urethroplasty with Parameatal Foreskin flap technique (OUPF IV). Finally, the most recent 19 had their hypospadias repaired by the Yoke technique. RESULTS: With the TPIF technique in the early periods, only 6 out of 11 patients underwent a successful repair (54.5%). With the OUPF IV technique, the success rate was only 60.0% (6 out of 10 cases). In contrast, 17 out of 19 cases treated by the Yoke technique in the most recent period had a successful repair, although proximal urethrocutaneous fistula and urethral stenosis occurred in one patient, respectively. A relatively high success rate was therefore obtained using the Yoke technique for the repair of severe proximal hypospadias. CONCLUSION: The Yoke techniques for the repair of hypospadias is therefore considered to be a safe and effective technique for the repair of proximal hypospadias because of the continuous skin flap of the ventral urethral plate and the prepuce with a blood supply from the circumferential vascular pedicle. We consider this technique to be very useful for the treatment of severe proximal hypospadias.