Enzalutamide versus Abiraterone Plus Prednisolone for Nonmetastatic Castration-Resistant Prostate Cancer: A Sub-Analysis from the ENABLE Study for PCa.

Enzalutamide (ENZ) and abiraterone plus prednisolone (ABI) can improve the survival of patients with castration-resistant prostate cancer (CRPC). However, the agent that is more effective against nonmetastatic CRPC remains unclear. To evaluate the agent that can be used as the first-line treatment for CRPC, an investigator-initiated, multicenter, randomized controlled trial (ENABLE Study for PCa) including both metastatic and nonmetastatic CRPC was conducted in Japan. The prostate-specific antigen (PSA) response rate, overall survival, some essential survival endpoints, and safety of patients with nonmetastatic CRPC were also analyzed. In this subanalysis, 15 and 26 patients in the ENZ and ABI arms, respectively, presented with nonmetastatic CRPC. There was no significant difference in terms of the PSA response rate between the ENZ and ABI arms (80% and 64%, respectively; p = 0.3048). The overall survival did not significantly differ between the two arms (HR: 0.68; 95% CI: 0.22-2.14, p = 0.5260). No significant differences were observed in terms of radiographic progression-free survival and cancer-specific survival between the ENZ and ABI arms (HR: 0.81; 95% CI: 0.35-1.84; p = 0.6056 and HR: 0.72; 95% CI: 0.19-2.73; p = 0.6443, respectively). Only four and six patients in the ENZ and ABI arms, respectively, had ≥grade 3 adverse events. ABI and ENZ had similar efficacy and safety profiles in patients with nonmetastatic CRPC.

[Idiopathic Thrombocytopenia Purpura After Pembrolizumab Treatment Against Locally Recurrent Bladder Cancer : A Case Report].

A man in his 70s visited our hospital for gross hematuria. He was diagnosed with invasive urothelial carcinoma (cT3N2M0) and underwent total cystectomy and ileum conduit construction after three courses of neoadjuvant chemotherapy. Eight months after the operation, the disease reoccurred in the pelvic lesion. He received pembrolizumab therapy but developed idiopathic thrombocytopenic purpura (ITP) immediately before the ninth course of administration; and, treatment was discontinued. Recovery of symptoms and normalization of blood test data were achieved 3.5months after starting steroid treatment. Reduction of recurrent disease has been maintained for 2 years.

Mixed epithelial and stromal tumor of the kidney with long-term imaging follow-up.

Mixed epithelial and stromal tumor (MEST) of the kidney is a rare benign tumor with malignant potential, and is characterized by epithelial and stromal proliferation with a variety of cellularity and growth pattern. MEST of the kidney is often depicted as a well-defined, solid mass with a cystic component. However, due to the rarity of the disease, there are no reports of its progression in serial imaging examinations. This report presents the case of a 68-year-old woman with MEST who was followed for 13 years by computed tomography (CT). To the best of our knowledge, this is the first report of image findings of MEST of the kidney over a follow-up period longer than 10 years.

Discontinuation of pembrolizumab for advanced urothelial carcinoma without disease progression: Nationwide cohort study.

Pembrolizumab, an anti-programmed death 1 monoclonal antibody, has revolutionized the treatment of metastatic urothelial carcinoma. However, the optimal treatment duration for treatment responders has not been established. To address this, we retrospectively assess the treatment outcomes and duration of pembrolizumab for patients whose best response was complete response (CR) or partial response (PR) in a Japanese nationwide cohort of platinum-refractory metastatic urothelial carcinoma. Of 203 patients whose best response was CR or PR, 83 patients discontinued pembrolizumab before progression. The median pembrolizumab treatment duration was 6.9 months. The 2-year relapse-free survival (RFS), treatment-free survival, and OS rates after discontinuation were 49.0%, 57.4%, and 74.5%, respectively. CR, higher hemoglobin levels, and a better Eastern Cooperative Oncology Group performance status at the time of discontinuation were associated with significantly better RFS. Pembrolizumab was re-administered to 12 patients. Pembrolizumab re-challenge resulted in CR, PR, stable disease, and progressive disease in six, three, two, and one patient, respectively. Propensity score-matched landmark analysis revealed no significant OS difference between patients who continued or discontinued pembrolizumab at 6, 12, and 18 months (p = 0.91, 0.99, and 0.25, respectively). Our findings demonstrated that patients with objective responses had favorable survival outcomes and suggested that pembrolizumab could be discontinued safely in this population. This study should drive further efforts to optimize the treatment duration for pembrolizumab responders.

Impact of family history on clinicopathological variables and disease progression in Japanese prostate cancer patients undergoing robotic-assisted radical prostatectomy.

OBJECTIVE: We evaluated whether a first-degree family history (FH) of prostate cancer (PCa) in Japanese patients undergoing robotic-assisted radical prostatectomy (RP) is correlated with clinicopathological variables and disease progression. METHODS: We reviewed consecutive 392 localized PCa patients undergoing robotic-assisted RP at our institution between 2015 and 2020. Information on FH was obtained via a self-administered questionnaire. A positive FH was defined as having a first-degree FH: a father and/or one or more brothers with PCa prior to diagnosis. All patients had clinically localized PCa treated by robotic-assisted RP. We evaluated the relationship between clinical characteristics, pathological findings, and biochemical progression-free survival (bPFS) according to first-degree FH status. RESULTS: Median follow-up was 20.8 months. FH was identified in 42 (10.7%) patients. Patients in the FH group (median, 64.8 years) were diagnosed at a significantly younger age than patients in the non-FH (NFH) group (patients without FH) (median, 67.7 years) (p = 0.003). The 5-year bPFS in the FH and NFH groups was 72.0% and 78.1%, respectively (p = 0.90). A subgroup analysis revealed a significant difference in prostate-specific antigen (PSA) density between the FH group (median, 0.51 ng/ml/cm3 ) and the NFH group (median, 0.29 ng/ml/cm3 ) in patients younger than 60 years (p = 0.033). CONCLUSIONS: In this RP population, FH of PCa was not associated with worse clinical characteristics, pathological findings, or disease progression. Patients with a FH underwent surgery at a significantly younger age, and among patients <60 years, patients with a FH had significantly higher PSA density compared with patients without a FH.

Succinate dehydrogenase B-deficient renal cell carcinoma with a germline variant in a Japanese patient: a case report.

Succinate dehydrogenase (SDH)-deficient renal cell carcinoma (RCC) is a rare renal cancer. A 75-year-old Japanese female presented with gross hematuria. Computed tomography revealed two tumors in the left kidney, which were resected. Immunohistochemistry indicated negative staining for the B subunit of SDH (SDHB) in the resected specimen, leading to a final diagnosis of SDHB-deficient RCC. Genetic testing for SDHB showed a RCC germline variant in exon 6 (NM_003000.3:c.642 G > C) that was previously reported but associated with a novel phenotype (i.e., RCC). Twenty-six years prior, her daughter, who was 25 years old at the time, had undergone radical nephrectomy for a pathologic diagnosis of renal oncocytoma of the right kidney; SDHB immunostaining of her daughter's tumor was also negative retrospectively. We confirmed that her daughter carried the germline variant in SDHB exon 6, similar to the patient. The patient had no evidence of disease progression at 15 months after surgery.

Enzalutamide Versus Abiraterone plus Prednisolone Before Chemotherapy for Castration-resistant Prostate Cancer: A Multicenter Randomized Controlled Trial.

Background: Enzalutamide (ENZ) and abiraterone plus prednisolone (ABI) improve survival in castration-resistant prostate cancer (CRPC). However, which agent is better for patients with CRPC remains unclear. Objective: To evaluate whether ENZ or ABI is better as first-line treatment for CRPC. Design setting and participants: An investigator-initiated, multicenter, randomized controlled trial was conducted in Japan. The study enrolled 203 patients with CRPC before chemotherapy between February 20, 2015, and July 31, 2019. Patients were randomly assigned 1:1 to the ENZ or ABI arm. Outcome measurements and statistical analysis: The primary endpoint was time to prostate-specific antigen (PSA) progression. Secondary endpoints included the PSA response rate (≥50% decline from baseline), overall survival, and safety. A log-rank test was used for comparison of survival analyses between arms. Results and limitations: After randomization, 92 patients in each arm were treated and analyzed. Time to PSA progression did not significantly differ between the arms (median 21.2 mo for ENZ and 11.9 mo for ABI; hazard ratio [HR] 0.81, 95% confidence interval [CI] 0.51-1.27; p = 0.1732). There was a significant difference in the PSA response rate between the arms (72% for ENZ and 57% for ABI; p = 0.0425). There was no significant difference in overall survival (median 32.9 mo for ENZA and 35.5 mo for ABI; HR 1.17, 95% CI 0.72-1.88; p = 0.5290). Grade ≥3 adverse events were observed in 11% of patients in the ENZA arm and 21% in the ABI arm (p = 0.1044). Conclusions: ENZ did not show any survival benefit in comparison to ABI, but showed a better PSA response rate with a low rate of severe adverse events in CRPC. Patient summary: Results from our study suggest that use of enzalutamide before abiraterone may have potential clinical benefits for patients with castration-resistant prostate cancer.

Efficacy and safety of pembrolizumab for older patients with chemoresistant urothelial carcinoma assessed using propensity score matching.

BACKGROUND: We used real-world and large-scale data to assess the clinical efficacy and safety of pembrolizumab in older patients with advanced urothelial carcinoma (UC). METHODS: A total of 608 patients who received pembrolizumab for the treatment of chemoresistant UC were retrospectively analyzed. All patients were histologically diagnosed with pure UC. Using propensity score matching (PSM) (ECOG performance status, site of metastasis, hemoglobin level and neutrophil-to-lymphocyte ratio, 1:1 matching), the overall survival (OS) and adverse events (AEs) of patients <75 and ≥75 years old were compared. RESULTS: The median follow-up (IQR) period was 16.1 (9.9-20.5) months. After PSM, there were 215 patients each in the aged <75 years and aged ≥75-year-old groups. The median OS of all patients was estimated to be 10.4 months (95% confidence interval [CI] = 8.8-12.1). After PSM, the median OS was 7.8 months (95% CI = 5.2-10.4) in the <75-year-old group and 10.4 months (95% CI = 7.3-13.5) in the ≥75-year-old group (P = 0.186). Any-grade AEs were more frequently reported in the ≥75-year-old group in comparison to the age <75-year-old group (55.3% vs. 41.9%, P = 0.007), whereas there was no significant difference between the two groups in the incidence of grade ≥3 AEs (10.2% vs. 12.6%, P = 0.544). The objective response rate, defined as complete remission or a partial response, was 22.8% in the <75-year-old group and 25.1% in the ≥75-year-old group (P = 0.651). CONCLUSIONS: The present study demonstrates that age does not affect the efficacy and safety of pembrolizumab treatment for advanced chemoresistant UC. Pembrolizumab treatment should not be avoided based on chronological age; however, close monitoring for the development of treatment-related AE should be considered for older patients.

Pre-pembrolizumab neutrophil-to-lymphocyte ratio (NLR) predicts the efficacy of second-line pembrolizumab treatment in urothelial cancer regardless of the pre-chemo NLR.

Neutrophil-to-lymphocyte ratio (NLR) was reported to be associated with prognosis of urothelial cancer (UC) patients receiving systemic chemotherapy or immunotherapy. However, it has not been elucidated how preceding first-line chemotherapy affects NLR and subsequent second-line pembrolizumab treatment. This multicenter study analyzed 458 patients with metastatic UC who received first-line chemotherapy and second-line pembrolizumab with regard to pre-chemotherapy and pre-pembrolizumab NLR in association with the efficacy of chemotherapy and pembrolizumab treatment. NLR was increased in 47% while decreased in 53% of patients before and after first-line chemotherapy. High pre-chemotherapy NLR (≥ 3) was significantly associated with unfavorable overall (OS, P = 0.0001) and progression-free (P < 0.0001) survivals after first-line chemotherapy. However, pre-chemotherapy NLR showed only modest influence on radiological response and survival after second-line pembrolizumab treatment, whereas pre-pembrolizumab NLR showed higher association. NLR decrease was associated with partial response or greater objective response by first-line chemotherapy, while NLR increase was associated with higher patient age. In conclusion, immediate pre-chemotherapy and pre-pembrolizumab NLR was significantly associated with efficacy of the following treatment, respectively. However, even patients with high pre-chemotherapy NLR achieved favorable OS if they had their NLR reduced by chemotherapy, whereas those with high pre-chemotherapy NLR yielded unfavorable OS if they had their NLR remained high after chemotherapy, suggesting that chemotherapy may have differential effect on the efficacy of subsequent pembrolizumab treatment in UC patients.

[Presurgical Treatment with Axitinib and Pembrolizumab Reduced Operation Risk by Downsizing the Vena Cava Tumor Thrombus in Clear Cell Renal Cell Carcinoma : A Case Report].

A woman in her seventies complained of chest pain during exertion and visited a local hospital. Computed tomographic scan showed right renal cell carcinoma with inferior vena cava (IVC) tumor thrombus extending above the diaphragm, and the patient was referred to our hospital. She was diagnosed with right renal cell carcinoma cT3cN0M0, with level IV IVC thrombus by Mayo classification. Axitinib and pembrolizumab were administered against intractable advanced renal cell carcinoma. The dose of axitinib was reduced due to grade 3 liver dysfunction. Right nephrectomy together with IVC thrombectomy was performed because the primary lesion had shrunk, and the level of IVC thrombus had become level III. The pathological results were clear cell carcinoma, pT3c, G3, Fuhrman grade3, INFA, v1, and ly0. Axitinib and pembrolizumab might be a presurgical option against an intractable renal cell carcinoma with an IVC thrombus.

A case of adenocarcinoma of the rete testis with durable response to cisplatin-based chemotherapy.

INTRODUCTION: Adenocarcinoma of the rete testis is a rare malignancy with a poor prognosis. We report a case of adenocarcinoma of the rete testis with a durable response to cisplatin-based chemotherapy. CASE PRESENTATION: A 48-year-old man with Down syndrome (trisomy 21) presented with a 1-month history of painless swelling of the left scrotum. The physical examination revealed a left testis with a hydrocele associated with a tumor and enlarged pelvic and para-aortic lymph nodes. He underwent a radical orchiectomy. The specimen was diagnosed as adenocarcinoma of the rete testis. The patient received 7 cycles of chemotherapy (1 cycle of BEP and 6 cycles of EP) postoperatively. The metastatic lymph nodes were reduced in size for at least 12 months. Our patient with adenocarcinoma of the rete testis obtained an acceptable response to cisplatin-based chemotherapy. CONCLUSION: We treated a patient with an adenocarcinoma of the rete testis who had an acceptable response to platinum-based chemotherapy.

[Initial Experience of Photodynamic Diagnosis-Assisted Transurethral Resection of Bladder Tumor (PDD-TURBT)].

Photodynamic diagnosis (PDD) using 5-aminolevulinic acid (5-ALA) is expected to be useful in preventing oversight of non-muscle-invasive bladder cancer (NMIBC) and in reducing the intravesical recurrence rate after transurethral resection of bladder tumor (TURBT). We report our initial experience with28 cases of PDD-assisted TURBT (122 samples) performed at our hospital from February 2018 to April 2019. The median age of the patients was 74.5 years, and 18 of the 28 were primary cases. Each patient underwent TURBT with oral administration of 5-ALA 20 mg/kg 3 hours before endoscopic examination. The sensitivity was 89.8% when both white light and blue light were used, which was superior to the sensitivity of 67.8% when using only white light (p＜0.01, McNemar's test). Among the first several cases, we experienced high false positivity, which suggested that some experience may be required to discriminate tumors from inflammatory lesions. In fact, the specificity and the positive likelihood ratio improved with experience. No grade 2 or higher adverse events were observed among our cases. The median follow-up period was 738 days, and 9 of 28 patients (32. 1%) had recurrence within the follow-up period. In conclusion, our initial experience with PDD-assisted TURBT demonstrated its excellent diagnostic sensitivity and safety, as previously reported.

Predictive urodynamic factors for de novo stress urinary incontinence after laparoscopic sacrocolpopexy for pelvic organ prolapse.

OBJECTIVES: Laparoscopic sacrocolpopexy is often performed to treat pelvic organ prolapse. Preoperative stress urinary incontinence (SUI) can improve after laparoscopic sacrocolpopexy. In contrast, some patients without incontinence preoperatively develop SUI after pelvic organ prolapse repair (ie, de novo SUI). This study aimed to identify the preoperative predictive factors of de novo SUI after laparoscopic sacrocolpopexy. METHODS: A total of 858 patients underwent laparoscopic sacrocolpopexy between January 2013 and November 2017 at Kameda Medical Center. Urodynamic studies were performed pre- and postoperatively on 75 patients. This retrospective study included 43 of 75 patients who did not have SUI preoperatively. We assessed urethral function according to functional profile length and maximum urethral closure pressure. We evaluated voiding function in terms of maximum flow rate, detrusor pressure at maximum flow rate, and postvoid residual volume. Furthermore, each factor was compared between de novo SUI-positive and SUI-negative groups. RESULTS: After laparoscopic sacrocolpopexy, 30 (69.8%) patients presented with SUI. The preoperative maximum urethral closure pressure was significantly lower in the de novo SUI-positive group (36.0 cmH2 O) than in the de novo SUI-negative group (50.5 cmH2 O, P = .020). More patients with maximum urethral closure pressure <40 cmH2 O had de novo SUI than patients with maximum urethral closure pressure ≥40 cmH2 O (P = .004). There were no significant differences between preoperative maximum urethral closure pressure and postoperative maximum urethral closure pressure in either group. CONCLUSIONS: Low preoperative maximum urethral closure pressure, particularly maximum urethral closure pressure <40 cmH2 O, is a predictor of de novo SUI after laparoscopic sacrocolpopexy.

Neoadjuvant Chemohormonal Therapy before Radical Prostatectomy for Japanese Patients with High-Risk Localized Prostate Cancer.

BACKGROUND: Radical prostatectomy (RP) is the standard treatment in patients with high-risk prostate cancer (PCa). However, there is a high rate of recurrence, and new approaches are required to improve surgical efficacy. Here, we evaluated the feasibility and safety of neoadjuvant chemohormonal therapy (NCHT) before RP for Japanese patients with high-risk localized prostate cancer (PCa). METHODS: From February 2009 to April 2016, 21 high-risk patients were enrolled in this prospective study. Patients were treated with docetaxel (70 mg/m2) every four weeks for three cycles and luteinizing hormone-releasing hormone agonist. Patients with grade 3-4 toxicities had 25% dose reductions for the following course. RESULTS: Median follow-up was 88.6 months. The dose of docetaxel was reduced in 13 patients. The estimated five-year biochemical progression-free survival (bPFS) rate was 57.1%. National Comprehensive Cancer Network criteria (high-risk, but not very high-risk (nVHR) versus VHR) was associated with bPFS (p = 0.03). Five-year bPFS rates in the nVHR and VHR groups were 76.9% and 25.0%, respectively. There was a significant difference in bPFS between the nVHR and VHR groups (p = 0.023) by Kaplan-Meier analysis. CONCLUSIONS: Although our study included a small number of cases, at least in our exploration, NCHT was safe and feasible. However, more extensive treatment modalities are needed to improve outcomes, especially in VHR patients.

[Neoajuvant Chemotherapy with Gemcitabine and Cisplatin Plus S-1 for Primary Female Urethral Adenocarcinoma].

A 67-year-old female presented for evaluation of a left inguinal mass. Contrast-enhanced computed tomography revealed a tumor surrounding the urethra. Magnetic resonance imaging showed that the tumor had invaded the bladder neck on the anterior aspect of the urethra. The serum carbohydrate antigen 19-9 level was elevated. The clinical diagnosis was a primary adenocarcinoma of the female urethra (cT4N2M0). The initial treatment consisted of gemcitabine plus cisplatin (GC) and oral fluoropyrimidine (S-1). A total cysto-urethrectomy with anterior vaginal wall resection, pelvic and inguinal lymphadenectomy, and urinary diversion with ileal conduit formation were performed. The final diagnosis was urethral adenocarcinoma (ypT4ypN2, stage IV). Twelve months post-operatively, there was no evidence of recurrence or distant metastases.

[A Case of Extragonadal Germ Cell Tumor Treated by Induction Therapy with A Reduced Bep Regimen].

A 21-year-old man with chief complaints of left hypochondriac and chest pain was shown to have multiple masses in the lung, a pleural effusion in the right cavum thoracis, a mediastinal mass, and lymphadenopathy detected by computed tomographic scan. He was diagnosed with an extragonadal germ cell tumor based on pathologic findings from lung biopsies and elevation of the serum total human chorionic gonadotropin. He underwent a reduced chemotherapy regimen consisting of bleomycin, cisplatin, and etoposide (reduced BEP) to lower the risk of acute respiratory distress syndrome (ARDS), a manifestation of choriocarcinoma syndrome, which occurs at induction chemotherapy with the full-dose BEP regimen. Choriocarcinoma syndrome did not develop during chemotherapy, and he has been disease-free since salvage chemotherapy and subsequent retroperitoneal lymph node dissection.

Successfully Treated Lung and Renal Metastases from Primary Chondrosarcoma of the Scapula with Radiofrequency Ablation and Surgical Resection.

Since chondrosarcoma is a relatively rare type of malignant bone tumors characterized by its ability to produce a cartilage matrix and aggressive behavior, a consensus clinical management strategy has not been established. We report a 55-year-old woman who presented with renal metastasis arising from chondrosarcoma of the scapula. Chondrosarcoma of the left scapula was diagnosed 15 years earlier. After surgical resection of a local recurrence in the left scapula, she received focal radiofrequency ablation (RFA). She underwent focal RFA and surgical resection for a total of 21 times for lung metastases. Because invasion of the renal pelvis was suspected from urine cytology, she underwent laparoscopic nephroureterectomy. The histopathological findings showed metastatic chondrosarcoma involving the right renal parenchyma. The patient has remained clinically stable without recurrence for 18 months. To the best of our knowledge, this is the first report of metastatic chondrosarcoma of the lung and renal parenchyma with involvement of the renal pelvis in which remission was achieved with multimodal treatment including RFA and surgical resection.

Transvaginal surgery using self-cut mesh for pelvic organ prolapse: 3-year clinical outcomes.

OBJECTIVES: To evaluate subjective and objective outcomes, complication, recurrence, and reoperation rates after transvaginal mesh surgery for the management of pelvic organ prolapse. METHODS: This was a retrospective analysis of transvaginal mesh surgery carried out using self-cut mesh measuring subjective outcomes using validated questionnaires, and objective outcomes using Pelvic Organ Prolapse Quantification. Patients diagnosed with stage ≥2 pelvic organ prolapse were counseled about all possible surgical options. After thorough explanation about the benefits and risks during transvaginal mesh surgery, patients who gave signed consent were scheduled for surgery and evaluated at 1 and 3 years postoperatively. RESULTS: We included 101 patients who completed a minimum of 3-year follow up. One year and 3-year follow up showed significant improvement both on subjective and objective outcomes. Recurrences were observed in three patients (3%), with one (1%) patient undergoing reoperation. One case (1%) of intraoperative complication (bladder injury) and four cases (4%) of postoperative complications (two mesh exposure, one hematoma and one significant increase in post-voiding residual) were recorded. Overall patients' satisfaction was positive. CONCLUSIONS: Transvaginal mesh surgery using self-cut mesh is associated with significant improvement in both subjective and objective outcomes, offering low recurrence and complication rates, and high patient satisfaction rates. It can be a safe, effective and cost-efficient option not only for recurrence cases, but also as primary management of pelvic organ prolapse using a standardized technique and proper patient selection.

Laparoscopic lateral suspension for pelvic organ prolapse in a case with difficulty in performing laparoscopic sacrocolpopexy.

INTRODUCTION: Mesh fixation at the promontory is the most important procedure in laparoscopic sacrocolpopexy. We present a case of pelvic organ prolapse wherein sacrocolpopexy was converted to lateral suspension intraoperatively due to tissue weakness of the promontory. CASE PRESENTATION: A 66-year-old woman with a sensation of bulge in the vagina presented to our clinic. She was diagnosed with uterine prolapse (grade III). Laparoscopic sacrocolpopexy was planned; exposure of the promontory and mesh fixation at the vesicovaginal and rectovaginal space could be smoothly performed. However, handling the needle at the promontory was impossible due to tissue weakness of the anterior longitudinal ligament of the sacrum. Consequently, mesh fixation was converted to lateral suspension. CONCLUSION: Difficult mesh fixation at the promontory is not rare in laparoscopic sacrocolpopexy. Lateral suspension may be useful as a trouble-shooting procedure for laparoscopic sacrocolpopexy, and surgeons performing laparoscopic sacrocolpopexy should know this procedure.

[LEIOMYOSARCOMA ARISING FROM THE RENAL VEIN].

A 47-year-old female presented to a clinic complaining of right back pain. A CT scan revealed a right retroperitoneal mass and she was referred to our department for further evaluation. Contrast-enhanced CT and MRI revealed a right retroperitoneal mass (6 cm) in the hilum of the right kidney that invaded the right renal vein and inferior vena cava (IVC). Suspecting a tumor arising from retroperitoneal tissues involving the right renal vein and IVC, the decision was made to excise the tumor with the right kidney, renal vein, and a portion of the IVC. The histologic findings indicated that the tumor was a leiomyosarcoma originating from the renal vein wall. The tumor cells were spindle-shaped and stained positive for desmin, caldesmon and HHF35. The post-operative course was uneventful and she was recurrence-free 20 months after surgery. In addition to presenting a case of a leiomyosarcoma of the renal vein, a short review of the literature is provided.