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Background: Renal pelvic urothelial carcinoma with multiple histological variants co-occurs with hydronephrosis and pyelonephritis extremely rarely. The diagnosis of renal pelvic urothelial carcinoma can be complicated by these conditions, leading to delayed or missed diagnosis or misdiagnosis., Case Presentation: We report the evolutionary course of a rare case of renal pelvic urothelial carcinoma containing squamous and sarcomatoid differentiation associated with hydronephrosis and pyelonephritis in a low-functioning kidney. Preoperative clinical and imaging manifestations were highly suggestive of hydronephrosis and pyelonephritis; however, all corresponding therapeutic measures failed. Eventually, nephrectomy was performed, and postoperative pathological examination revealed renal pelvic urothelial carcinoma with squamous and sarcomatoid differentiation. Further residual ureter and bladder cuff resection was refused by the patient. Regular cisplatin-based postoperative chemotherapy and bladder perfusion were arranged, and cystoscopy and imaging examinations were performed regularly for follow-up., Conclusion: Hydronephrosis and pyelonephritis can mask the typical clinical and imaging manifestations of renal pelvic urothelial carcinoma. Renal pelvic malignancy should be considered in patients with intractable pyelonephritis and hydronephrosis. There is no standard treatment for renal pelvic carcinoma with multiple pathological types. Radical surgery is considered an appropriate option and should be performed as early as possible. Chemotherapy and immunotherapy may improve patient survival., Competing Interests: Declarations Ethics approval and consent to participate This case was approved by the Ethics Committee of Jiangsu Provincial Hospital of Integrated Chinese and Western Medicine. Consent for publication Written informed consent was obtained from the patient for publication of this case report. Competing interests The authors declare no competing interests., (© 2024. The Author(s).)

Primary mucinous tumors of the renal pelvis are extremely rare and pose challenges in terms of diagnosis and treatment. This study reviewed the clinical and pathological characteristics of mucinous tumors of the renal pelvis, including mucinous cystadenocarcinomas and mucinous cystadenomas. Immunohistochemical analysis was conducted in three cases, along with KRAS gene detection using the Amplification Refractory Mutation System (ARMS) method. The results revealed mucinous epithelium with acellular mucinous pools in all cases, and acellular mucinous pools were observed in the renal parenchyma and perirenal fat capsules. All tumors expressed CK20 and CDX2, and one case showed KRAS gene mutation. The study suggests that mucinous cystadenomas of the renal pelvis may exhibit borderline biological behaviors. This study is the first to report a KRAS gene mutation in a mucinous cystadenoma of the renal pelvis, offering valuable insights into the diagnosis and treatment of this rare condition., Competing Interests: Declaration of competing interest All authors do not have any potential conflicts (financial, professional, or personal) that are relevant to the manuscript to disclose., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

The incidence rate of malignancy in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is higher than that in the general population. Malignancy has been indicated to be a risk factor or inducer of AAV. Herein, we report the case of a healthy 84-year-old man with seronegative microscopic polyangiitis (MPA) after the diagnosis of renal pelvic carcinoma. Four weeks before admission, his estimated glomerular filtration rate (eGFR) was 85 ml/min/1.73 m 2 , and no hematuria or proteinuria was detected. Renal biopsy on admission revealed invasive urothelial carcinoma of the right renal pelvis. On day 15, his eGFR decreased to 30 ml/min/1.73 m 2 without any incitement. The renal specimen extracted via right robot-assisted nephroureterectomy indicated the presence of ANCA-associated glomerulonephritis. On day 37, urinary protein/urinary creatinine level of 6.48 g/gCre, serum albumin level of 2.1 mg/dL, and eGFR of 20 ml/min/1.73 m 2 indicated the presence of nephrotic syndrome. His blood sputum was analyzed via chest computed tomography, which revealed alveolar hemorrhage. Although his myeloperoxidase-ANCA was negative, he was diagnosed with MPA based on the 2022 American College of Rheumatology/European League Against Rheumatism classification criteria. This is the first case report of  MPA or AAV complicated with renal pelvic carcinoma. The clinical indicators demonstrated that renal pelvic carcinoma preceded the onset of MPA. The spatial proximity of both diseases indicated that renal pelvic carcinoma had some influence on MPA development via the mechanism of inflammatory cytokines or neutrophil extracellular traps. Our report may be useful in elucidating the mechanism of MPA development., (© 2024. The Author(s), under exclusive licence to Japanese Society of Nephrology.)

Extraosseous bone formation of the upper urothelial tract is an unusual phenomenon with limited documentation in the uropathology literature, reported in only 2 clinical series of patients undergoing percutaneous nephrolithotomy for the management of renal stones. While speculations regarding the pathogenesis of this occurrence have been published, heterotopic ossification is still poorly understood. We report the finding of extraosseous bone formation in the renal pelvis of a 30-year-old male patient with a history of kidney stones. Histologic sections of the ureter and renal pelvis showed submucosal nodules of woven bone. Ancillary fluorescence in-situ hybridization studies were negative for MDM2 amplification and USP6 rearrangement., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Collagen 17A1 (COL17A1), an epidermal hemidesmosome component, is ectopically induced in the urothelium of mouse and human renal pelvis (RP) in parallel with urinary tract-associated lymphoid structure development. Here, COL17A1 was induced in obstructive uropathy-prone ureter of humans and cats. To ascertain its function, murine urinary organs with unilateral ureteral obstruction (UUO) were analyzed during 1 week after surgery. One day after UUO, COL17A1 expression increased in urothelial cells of RP and ureter, and was positively correlated with renal tubulointerstitial lesions. A portion of RP where the smooth muscle layer from the ureter was interrupted was sensitive to urothelium deciduation and COL17A1 induction, showing urine leaked from the RP lumen into the parenchyma. After urine stimulation, cultured immune cells expressed Cxcl2, also up-regulated in CD11b + cells following COL17A1 stimulation. One day after UUO, CXCL2 + CD11b + cells infiltrated the urothelium-disrupted area. However, these numbers were significantly lower in Col17a1-deficient mice. COL17A1 + urothelial cells partially co-expressed cytokeratin-14, a progenitor cell marker for urothelium, whereas Col17a1-deficient mice had lower numbers of cytokeratin-14 + cells. Gene Ontology analysis revealed that expression of epithelial- and immune-associated genes was up-regulated and down-regulated, respectively, in the ureter of Col17a1-deficient mice 4 days after UUO. Thus, COL17A1 maintains urothelium integrity by regulating urothelial cell adhesion, proliferation, and differentiation, and activates local immune responses during obstructive uropathy in mammals., Competing Interests: Disclosure Statement None declared., (Copyright © 2024 American Society for Investigative Pathology. Published by Elsevier Inc. All rights reserved.)

Renal pelvic carcinoma is a common upper urothelial cancer. The lack of an ideal in vitro model seriously hinders the research progress in the treatment for this disease. This study established a pipeline for the culture of renal pelvic carcinoma organoids based on the tumor tissue samples derived from the patients and tested the organoids to chemotherapeutic drugs. The results of immunohistochemistry and fluorescence experiments confirmed that the renal pelvic carcinoma organoids obtained from culture presented obvious nuclear heteromorphism, which was consistent with the tissue samples from renal pelvic carcinoma patients. The tumor marker molecule CD44 and the cell proliferation marker molecule Ki67 were positive in the organoids, indicating that the organoids were enriched with tumor stem cells and had strong proliferative ability. The renal pelvic carcinoma organoids were highly sensitive to pirarubicin, which had obvious killing effects. In brief, this study successfully established an in vitro model of renal pelvic cancer organoids and tested the sensitivity of the model to chemotherapeutic drugs. The results provide a new laboratory model for the individualized diagnosis and treatment of epithelial carcinomas represented by renal pelvic carcinoma.

The intrarenal reflux is caused by impaired emptying of the renal pelvis, that leads to increased intrarenal pressure. Increased pelvis pressure can be the result of a variety of states. The most common causes are acute upper urinary tract obstruction or overfilling of the pelvis during retrograde intrarenal surgery (RIRS) or retrograde pyelography. In rare cases, it is due to impaired neuromuscular tone of the upper urinary tract that leads to hyperkinetic state. We have presented case with a 73-year-old patient with intrarenal reflux into the peripelvic renal cysts. Since no such cases were found in the literature, this observation is unique.

Objectives: Sarcomatoid renal pelvis carcinoma (SRPC) is a rare variant of RPC. We aimed to summarize the clinicopathological features and prognostic factors of SRPC., Methods: In this retrospective study, we collected data from 24 patients with SRPC who were treated at the Department of Urology, Affiliated Hospital of Qingdao University between 2008 and 2021. The clinicopathological features of the patients were obtained from their medical records to evaluate the diagnosis, prognostic factors, and response to systemic therapy., Results: Immunohistochemical staining revealed that cytokeratin was expressed in 19 patients with SRPC, while vimentin was expressed in all patients. Computer tomography showed these tumors as low-density (n = 12) or mixed-density masses, with or without necrotic areas (n = 12). All patients showed different degrees of enhancement on computed tomography. Lymph node metastasis was present in 6 patients and distant metastasis in 5. The median survival of all patients was 28 months. Patients without metastasis had a median survival of 46 months compared with 18 months in those with metastasis (P < 0.05). Necrosis had no significant influence on prognosis (P > 0.05). The median survival of patients with and without hydronephrosis was 18 and 104 months (P < 0.05). Among patients without metastasis, those without hydronephrosis survived longer than those with hydronephrosis (104 vs 18 months, P < 0.05), and necrosis had no effect on prognosis. In patients with metastasis, necrosis and hydronephrosis had no effect on prognosis (P > 0.05)., Conclusion: The prognosis of SRPC is poor, and the clinical stage, particularly the presence of distant metastasis, has a significant impact on prognosis., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2024 Published by Elsevier Ltd.)

Cancers of the kidney and renal pelvis are among the most prevalent types of urinary cancers. We aimed to outline the incidence trends of kidney and renal pelvis cancers by age, sex, race/ethnicity, and histology in the United States (US) from 2000 to 2020. The data was obtained from the Surveillance, Epidemiology, and End Results (SEER) 22 database. The identification of patients with kidney and renal pelvis cancers with morphologies of renal cell carcinoma, nephroblastoma, sarcoma, and neuroendocrine tumor was conducted utilizing the International Classification of Diseases for Oncology version 3. The average annual percent change (AAPC) were presented. All estimates were given in the form of counts and delayed age-standardized incidence rates (ASIRs) per 100,000 people. From 2000 to 2019, a total of 490,481 cases of kidney and renal pelvic cancer were recorded across all age groups in the US. The majority of them were among Non-Hispanic Whites (NHWs) (69.75%) and those aged 55-69 years (39.96%). The ASIRs per 100,000 for kidney and pelvis cancers were 22.03 for men and 11.14 for women. Non-Hispanic Black men had the highest ASIR (24.53 [24.24, 24.81]), and increase in ASIR over the 2000-2019 period (AAPC: 2.19% [1.84, 2.84]). There was a noticeable increase in incidence of kidney and renal pelvis cancers. Individuals aged 70-84 years had the highest ASIR for kidney and renal pelvis cancers. The COVID-19 era has resulted in a significant reduction in incidence rates across all demographics., (© 2024. The Author(s).)

Ureteral endometriosis is rare and can be a silent clinical entity, which can potentially lead to serious complications such as obstructive uropathy, sepsis and renal failure. A high clinical suspicion is required especially in childbearing age groups due to non-specific presentation such as renal colic, recurrent urinary tract infection (UTI), renal failure or asymptomatic hydronephrosis.A woman in her 40s presented with febrile UTI and flank pain. She reportedly suffered from recurrent UTIs in the past. Initial workup revealed an infected, obstructed left renal collecting system with gross hydronephrosis and hydroureter to the distal ureter on a significant gynaecological background of severe endometriosis requiring hysterectomy in the past.CT showed chronic obstructive changes and soft tissue nodules within the renal pelvis with no radio-opaque stones. She underwent emergent ureteric stent insertion. Functional imaging demonstrated only 1% contribution of the left kidney with a preserved estimated glomerular filtration rate of 65 mL/min/1.73 m 2 Endoscopic evaluation of ureters found extensive soft tissue lesions throughout the dilated left collecting system with biopsy-confirmed endometriosis. Subsequently, she underwent laparoscopic nephroureterectomy due to extensive ureteric involvement and chronically obstructed non-functioning kidney. Histopathology demonstrated completely obstructing ureteral endometriosis.Ureteric obstruction secondary to endometriosis can be due to extrinsic or intrinsic disease. In addition to initial assessment with CT urogram MRI may be helpful to evaluate soft tissue thickening. Endoscopic assessment with ureteroscopy and biopsy is required for tissue diagnosis. Surgery is often the treatment of choice, ranging from ureteroureterostomy, ureteroneocystostomy or nephroureterectomy in severe cases.Ureteral endometriosis is a rare clinical entity, clinicians should remain vigilant about common presentations of this rare entity, early diagnosis and prompt treatment is crucial to prevent progression to renal failure., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Introduction: Upper urinary tract stones combined with parenchymal infiltrative renal pelvic cancer are challenging to detect on imaging and to evaluate the differential diagnosis., Case Presentation: The symptoms and diagnoses in three cases of parenchymal infiltrative renal pelvic cancer and upper urinary tract stones that occurred between June 2019 and June 2022 were reviewed. Primary symptoms of lumbar discomfort and hematuria were evident in all 3 patients. Preoperative computed tomography (CT) abdominal imaging revealed that all three cases had hydronephrosis along with renal stones, while the other two cases only had localized hypoenhancement of the renal parenchyma, which was only thought to be limited inflammatory changes in the renal cortex as a result of the combination of renal pelvis infection. After percutaneous nephrolithotomy or ureteroscopic lithotripsy, a combined renal pelvis tumor was discovered in all of these instances. Radical tumor surgery was later performed. One patient who had several tumor metastases passed away 6 months after surgery. A case with multiple metastases was discovered 15 months after surgery and survived with the help of the current chemotherapy. A case with a bladder tumor recurrence was discovered 16 months after surgery and had transurethral bladder tumor electrosurgery and routine bladder perfusion chemotherapy., Conclusion: Upper urinary tract stones and parenchymal infiltrative pyel carcinoma have atypical imaging, easily confused with infectious diseases. CT or computed tomography urography (CTU) must be considered by urologists. Patients who have a CT with local renal parenchyma density should be suspected of having parenchymal invasive renal pelvis carcinoma; a needle biopsy ought to be performed; and repeat biopsies may be performed if necessary. High-risk individuals need multiple, sufficient biopsies as needed and a comprehensive intraoperative assessment of the renal pelvic mucosa., (© 2024 The Author(s). Published by S. Karger AG, Basel.)

Urothelial carcinoma of the upper urinary tract (UTUC) presents a significant clinical challenge, often requiring aggressive surgical intervention for optimal management. We present a case of an 84-year-old woman with recurrent high-grade papillary UTUC of the left renal pelvis, refractory to prior endourologic interventions, who underwent neoadjuvant treatment with pembrolizumab and enfortumab vedotin (Pembro/EV) due to contraindications to cisplatin therapy. Following a favorable response to neoadjuvant therapy, the patient underwent laparoscopic left radical nephroureterectomy, achieving a pathologic complete response. We discuss the utility of Pembro/EV in the perioperative management of patients with UTUC, particularly in those ineligible for cisplatin-based therapy. In addition, we highlight the potential role of somatic mutation testing and the integration of novel therapeutic agents such as olaparib in personalized treatment strategies for UTUC. This case underscores the importance of exploring innovative treatment approaches and optimizing patient selection for kidney preservation strategies in the management of UTUC. Further research and clinical trials are warranted to elucidate the full therapeutic potential of Pembro/EV and other emerging therapies in this setting., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: P.C., MD, serves on an advisory board for Eisai, Merck, Taiho, Pfizer, Astellas, and Sirtex.

Background: Upper urinary tract urothelial carcinoma (UTUC) is often locally advanced at initial diagnosis and is associated with high recurrence and mortality rates after radical nephroureterectomy (RNU). Adjuvant platinum-based chemotherapy has shown a recurrence-free survival benefit in a randomised phase III trial, while neoadjuvant treatment seems promising in retrospective series. On the contrary, little is known about the role of perioperative immunotherapy and its combination with chemotherapy for UTUC patients, although initial positive results have been published for muscle-invasive bladder cancer., Study Design and Endpoints: Against this backdrop, we are running a multi-centre single-arm phase 2 trial of neoadjuvant Durvalumab, a monoclonal antibody targeting programmed cell death ligand 1, combined with Gemcitabine and Cisplatin or Carboplatin for high-risk UTUC patients. The primary outcome is pathological complete response rate at RNU. Secondary endpoints include the partial pathological response rate, safety, as well as disease-free and overall survival. A biomarker analysis is also planned., Patients and Interventions: Included patients must have a good performance status and harbour a non-metastatic UTUC, considered at high risk of progression, defined as either biopsy-proven high-grade disease or invasive features at imaging with or, more recently, without high-grade cytology at the multidisciplinary team discretion, as specified in the latest amendment. Enrolled patients receive 3 cycles of neoadjuvant immuno-chemotherapy before RNU, and the standard of care thereafter. The trial is registered as NCT04617756 and is supervised by an independent data monitoring committee., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: The impact of body mass index (BMI) on patients with upper urinary tract urothelial carcinoma (UTUC) undergoing radical nephroureterectomy (RNU) is controversial. Increasing evidence suggests an age-dependent relationship between obesity and outcomes for some solid organ tumors. Herein, we aimed to assess the prognostic value of preoperative BMI in UTUC patients treated with RNU in Taiwan., Methods: This was a retrospective single-center study of 468 UTUC patients undergoing RNU during January 2010-December 2017, with preoperative BMI classification and subgroup analysis based on ages of < or ≥ 70 years. All UTUC patients underwent RNU and bladder cuff excision. Overall survival (OS), cancer-specific survival, and disease-free survival (DFS) were analyzed. Fisher's exact test, Mann-Whitney U test, Kaplan-Meier method, and Cox regression model were used for data analysis., Results: The median follow-up duration was 36 months. Patients with higher versus lower BMI (cutoff: 25 kg/m 2 ) showed no differences in OS; older patients had poor OS (hazard ratio [HR] 1.74; 95% confidence interval [CI] 1.24-2.40; p < 0.001). Older age was an independent predictor of poor OS in multivariate Cox regression analysis (p = 0.001). Younger patients with higher BMI (p = 0.02) had better DFS than older patients with no BMI-related survival differences. Higher BMI was an independent predictor of favorable DFS in younger patients in multivariate Cox regression analysis (HR, 0.53; 95% CI 0.28-0.99; p = 0.043)., Conclusion: Younger UTUC patients with higher BMI were independently associated with a favorable DFS., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

The goal of this study is exploring the disparity of incidence, mortality and survival outcome among transitional cell carcinomas (TCCs) in the 4 parts of urinary system. This study comprehensively evaluates these disparities using the Surveillance, Epidemiology, and End Results (SEER) (2000-2018) database. According to the SEER database, the urinary tract is divided into 4 parts: urinary bladder, renal pelvis, ureter, and urethra. The joinpoint regression was used to analyze the secular trend of incidence and incidence-based mortality (IBM). The Kaplan-Meier method with the log-rank test is performed to evaluate survival outcomes. The bladder TCC has the highest age-adjusted incidence and mortality rate compared with TCC in other 3 locations. A slight decrease in incidence is shown in the both bladder and urethra TCCs during 2000-2018. The age-adjusted mortality rate similarly presents an initial increase among 4 locations TCCs at the beginning of study period. The survival curves demonstrate that patients with bladder TCCs have better overall survival (OS) and cancer-specific survival (CSS), whereas those with renal pelvis TCCs have the worse OS and CSS. In addition, patients with bladder TCC have the highest 1-year, 3-year, 5-year relative survival rate, and those with renal pelvis TCC have the lowest. These disparities are especially essential when we explore tumor characteristics and treatment, extrapolated from the literature on bladder TCC for upper tract urothelial carcinoma (UTUC). Notably, patients with bladder TCC especially for localized stage have better survival outcomes than those with UTUC., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

The prognosis of renal pelvis cancer is poor and therapeutic options are limited, especially for patients with advanced disease. In this report, we present a case of advanced renal pelvis carcinoma in a male patient in his 60s, characterized by an activating mutation in ERBB2 . Clinical evaluation resulted in a pathological diagnosis of renal pelvis carcinoma with liver metastasis. Immunohistochemistry staining results suggested that CK, P63, and PAX8 were positively expressed, while Sy, CK7, CK20, S100, PAX8, and HEP1 were negatively expressed. Furthermore, next-generation sequencing results showed an activating mutation in the ERBB2 gene. The patient initially received a trastuzumab-based combination therapy, which led to a significant reduction in ERBB2 mutation frequency and a stable condition after three treatment cycles. However, following continuous treatment for 4 months, the patient developed drug resistance that resulted in disease relapse. Subsequently, the patient received apatinib treatment, but the therapeutic response was not satisfactory. The patient's condition underwent rapid deterioration and he ultimately succumbed to the disease. This case underscores the potential benefit of trastuzumab for treating ERBB2 -mutated advanced renal pelvis cancer, but further highlights that overcoming drug resistance remains a crucial challenge for long-term efficacy., Competing Interests: Declaration of conflicting interestsThe authors declare that there is no conflict of interest.

Background: UGN-101 can be used for chemoablation of low-grade upper tract urothelial carcinoma (UTUC). The gel can be administered via a retrograde route through a ureteral catheter or an antegrade route via a nephrostomy tube., Objective: To report outcomes of UGN-101 by route of administration., Design, Setting, and Participants: We performed a retrospective review of 132 patients from 15 institutions who were treated with UGN-101 for low-grade UTUC via retrograde versus antegrade administration., Outcome Measurements and Statistical Analysis: Survival outcomes are reported per patient. Treatment, complications, and recurrence outcomes are reported per renal unit. Statistical analysis was performed for primary endpoints of oncological response and ureteral stricture occurrence., Results and Limitations: A total of 136 renal units were evaluated, comprising 78 retrograde and 58 antegrade instillations. Median follow-up was 7.4 mo. There were 120 cases (91%) of biopsy-proven low-grade UTUC. Tumors were in the renal pelvis alone in 89 cases (65%), in the ureter alone in 12 cases (9%), and in both in 35 cases (26%). Seventy-six patients (56%) had residual disease before UGN-101 treatment. Chemoablation with UGN-101 was used in 50/78 (64%) retrograde cases and 26/58 (45%) antegrade cases. A complete response according to inspection and cytology was achieved in 31 (48%) retrograde and 30 (60%) antegrade renal units (p = 0.1). Clavien grade 3 ureteral stricture occurred in 21 retrograde cases (32%) and only six (12%) antegrade cases (p < 0.01). Limitations include treatment bias, as patients in the antegrade group were more likely to undergo endoscopic mechanical ablation before UGN-101 instillation., Conclusions: These preliminary results show a significantly lower rate of stricture occurrence with antegrade administration of UGN-101, with no apparent impact on oncological efficacy., Patient Summary: We compared results for two different delivery routes for the drug UGN-101 for treatment of cancer in the upper urinary tract. For the antegrade route, a tube is inserted through the skin into the kidney. For the retrograde route, a catheter is inserted past the bladder into the upper urinary tract. Our results show a lower rate of narrowing of the ureter (the tube draining urine from the kidney into the bladder) using the antegrade route, with no difference in cancer control., (Copyright © 2023 European Association of Urology. Published by Elsevier B.V. All rights reserved.)

Inflammatory fibroid polyps (IFP) are rare and benign mesenchymal tumours of the gastrointestinal tract. They are submucosal spindle cell lesions with an eosinophilic-rich inflammatory infiltrate and mutations in the platelet-derived growth factor receptor alpha (PDGFRA) gene. In this report, we present the case of a 74-year-old female with a solid tumour of the kidney, which presented as a bland proliferation of spindle cells with thin-walled blood vessels and an inflammatory infiltrate with eosinophilic granulocytes. Immunohistochemistry revealed a positivity for vimentin and a weak staining for CD99 and CD34 in the spindle cells. Because of the morphological similarity to IFPs of the gastrointestinal tract, a molecular pathology analysis was performed. This identified an oncogenic mutation in exon 18 of the PDGFRA gene, which is characteristic for inflammatory fibroid polyps of the gastrointestinal tract. To the best of our knowledge, this is the first case of an IFP in the urogenital tract., (© 2023. The Author(s).)

Immunoglobulin (Ig) G4-related diseases are very rare diseases and are difficult to diagnose and treat. Here, we report a rare condition, an IgG4-related disease involving the bilateral renal pelvises, which was successfully treated with steroid therapy. A 64-year-old female was admitted with left flank pain and hematuria. Computed tomographic images showed irregular enhancing masses around the bilateral renal pelvises and lymph nodes in the left para-aortic area. A histopathological examination of a renal needle biopsy revealed numerous cells that were positive for IgG and more than 10 IgG4-positive cells per high-powered field. The IgG4/IgG ratio was more than 40%. Her serum IgG4 level was elevated to 1440 mg/dL, and her IgG4/IgG ratio was 82.1%. After a diagnosis of IgG4 sclerosing disease, a double-J ureteral stent was inserted and steroid therapy was performed for 20 weeks. Her symptoms dramatically improved, and the follow-up images showed a resolved state. There was no relapse of the disease for more than 6 months after the removal of the stent., (Copyright © 2023 Copyright: © 2023 Saudi Journal of Kidney Diseases and Transplantation.)

GATA3 is a transcription factor involved in the embryogenesis of multiple human tissues and organs and in maintaining cell differentiation and tissue homeostasis in the adult organism. GATA3 is also involved in carcinogenesis and is regarded as a sensitive marker for urothelial and breast carcinomas, although its expression in carcinomas of non-breast/urothelial origin has been frequently reported. In this study, we sought to examine the extent and intensity of GATA3 expression in various carcinomas, mainly lung, urothelial, breast, and various other primary sites. Patients with breast carcinoma (n=40), carcinoma of the urinary bladder/renal pelvis (n=40), lung carcinoma (n=110), and various other origins (n=45) were included in the study. 165 patients had a primary tumor diagnosis, and 70 cases had a metastatic tumor diagnosis. Our results showed that GATA3 expression was significantly more common in carcinomas of the breast, urinary bladder, and renal pelvis compared to all other origins. All primary and 93% of metastatic urinary bladder carcinomas and 94% of primary and 80% of metastatic breast carcinomas expressed GATA3. Expression was lower in the non-urothelial histology of urinary primaries and in triple-negative breast carcinomas (TNBC). Focal staining, mostly faint, was seen in 5.6% of the primary lung adenocarcinomas and 35% of the primary lung squamous cell carcinomas. More extensive and intense staining was seen in 3.7% of the primary lung adenocarcinomas and 12% of the primary lung squamous cell carcinomas. Expression, mostly focal, was also seen in 30% of the metastatic lung carcinomas. Finally, high expression was seen in 12.5% of the other tumors (one metastatic pancreatic carcinoma, one metastatic salivary gland adenocarcinoma not otherwise specified, one metastatic squamous cell carcinoma of the skin, one primary uterine cervix serous carcinoma, and one squamous cell carcinoma of the head and neck), and focal expression was present in another 22% of them. No ideal cut-off for positivity for GATA3 staining could be identified, as increasing the cut-off in either the extent or the intensity of staining increased specificity but decreased sensitivity. In conclusion, our study shows that although GATA3 staining is very helpful in everyday practice in determining the breast/urothelial origin of carcinomas, there are two caveats to its use: the first is that nonclassical histologies of urothelial carcinomas and TNBC may be negative for the marker, and secondly, carcinomas of various origins may show (although rarely) intense positivity.

Background: Increased usage of cross sectional imaging for a variety of indications, in particular CT imaging, has led to an increased detection of renal and ureteric masses. Benign ureteric masses are rare, with 95% of identified tumours consisting of transitional cell carcinoma (TCC). IgG4-related disease is a recognised clinical systemic autoimmune, inflammatory condition with a propensity for multi-organ manifestation. Nephritis and pseudo-tumour formation can occur when kidneys are involved. Ureteric involvement is more rare., Case Presentation: Forty nine-year-old Korean male was found to have an incidental invasive renal pelvis mass during investigation for chronic back pain and fatigue. Appearance of the tumour was consistent with an invasive malignancy, and consensus from multidisciplinary meeting was to have the tumour removed. Procedure involved a prolonged open surgery with reconstruction of contralateral renal blood supply and was complicated by a long recovery process. Final histopathology confirmed IgG4 renal pseudo tumour diagnosis., Conclusion: IgG4-related disease is a rare but potentially morbid disease that can mimic various cancers, including lung, pancreas and renal malignancies. A high index of suspicion is required to accurately diagnose this condition, through a targeted history taking, examination and investigation which should include biopsies. Failing to do so may result in unnecessary procedures being performed and exposing a patient to its associated risks., (© 2023. BioMed Central Ltd., part of Springer Nature.)

Current guidelines recommend endoscopic management (EM) for patients with low-risk upper urinary tract urothelial carcinoma, as well as those with an imperative indication. However, regardless of the tumor risk, radical nephroureterectomy is still mainly performed worldwide despite the benefits of EM, such as renal function maintenance, no hemodialysis requirement, and treatment cost reduction. This might be explained by the association of EM with a high risk of local recurrence and progression. Furthermore, the need for rigorous patient selection and close surveillance following EM may be relevant. Nevertheless, recent developments in diagnostic modalities, pathological evaluation, surgical devices and techniques, and intracavitary regimens have been reported, which may contribute to improved risk stratification and treatments with superior oncological outcomes. In this review, considering recent advances in endourology and oncology, we propose novel treatment strategies for optimal EM., (© 2023 The Japanese Urological Association.)

Context: The European Association of Urology (EAU) guidelines panel on upper urinary tract urothelial carcinoma (UTUC) has updated the guidelines to aid clinicians in evidence-based management of UTUC., Objective: To provide an overview of the EAU guidelines on UTUC as an aid to clinicians., Evidence Acquisition: The recommendations provided in these guidelines are based on a review of the literature via a systematic search of the PubMed, Ovid, EMBASE, and Cochrane databases. Data were searched using the following keywords: urinary tract cancer, urothelial carcinomas, renal pelvis, ureter, bladder cancer, chemotherapy, ureteroscopy, nephroureterectomy, neoplasm, (neo)adjuvant treatment, instillation, recurrence, risk factors, metastatic, immunotherapy, and survival. The results were assessed by a panel of experts., Evidence Synthesis: Even though data are accruing, for many areas there is still insufficient high-level evidence to provide strong recommendations. Patient stratification on the basis of histology and clinical examination (including imaging) and assessment of patients at risk of Lynch syndrome will aid management. Kidney-sparing management should be offered as a primary treatment option to patients with low-risk UTUC and two functional kidneys. In particular, for patients with high-risk or metastatic UTUC, new treatment options have become available. In high-risk UTUC, platinum-based chemotherapy after radical nephroureterectomy, and adjuvant nivolumab for unfit or patients who decline chemotherapy, are options. For metastatic disease, gemcitabine/carboplatin chemotherapy is recommended as first-line treatment for cisplatin-ineligible patients. Patients with PD-1/PD-L1-positive tumours should be offered a checkpoint inhibitor (pembrolizumab or atezolizumab)., Conclusions: These guidelines contain information on the management of individual patients according to the current best evidence. Urologists should take into account the specific clinical characteristics of each patient when determining the optimal treatment regimen according to the risk stratification of these tumours., Patient Summary: Cancer of the upper urinary tract is rare, but because 60% of these tumours are invasive at diagnosis, timely and appropriate diagnosis is most important. A number of known risk factors exist., (Copyright © 2023 European Association of Urology. Published by Elsevier B.V. All rights reserved.)

Introduction: The intraoperative anatomical findings (IOAF) of all ureteropelvic junction obstruction (UPJO) cases are not identical. Moreover, there is also controversy in the literature regarding histopathological (HP) findings in cases of UPJO. In the present study, we evaluated different IOAF and assessed their association with specific HP parameters., Materials and Methods: This was a cross-sectional study set-up, which was carried out in a tertiary care centre. Children with UPJO who underwent surgery between 2017 and 2020 were enrolled. The following IOAF were noted: Type of pelvis (extrarenal or intrarenal), insertion of the ureter (high or normal), presence of lower pole crossing vessel (CV), negotiation of UPJ segment with double J stent (3 Fr) and length of internal narrowing (LIN) at UPJ. The resected segment of UPJ was assessed at three levels (pelvis, UPJ and ureter) for various HP parameters including fibrosis, oedema, inflammation and smooth muscle hypertrophy (SMH)., Results: Thirty-nine children were included in the study with a mean age of 31 months. The summary statistics of IOAF were intrarenal pelvis in 5 cases, high insertion of the ureter (HIU) in 9, CV in 6, negotiable UPJ in 23, and 16 cases showed LIN >1 cm. All cases showed SMH at the pelvis region and SMH with fibrosis at the UPJ region. At the pelvis region, there was an association between (1) HIU with oedema and chronic inflammation (CIF), (2) CV with CIF and (3) LIN with CIF and SMH. At the UPJ region, there was an association between (1) CV and negotiable UPJ with less fibrosis and (2) LIN with SMH. At the ureteric end, CV showed an association with less fibrosis and more CIF., Conclusion: All UPJO cases have some common HP findings. Although, some particular IOAF, i.e., presence of CV, negotiable UPJ, HIU and LIN showed association with specific HP parameters., Competing Interests: None

Significance Statement: Congenital obstructive uropathy (COU) is a prevalent human developmental defect with highly heterogeneous clinical presentations and outcomes. Genetics may refine diagnosis, prognosis, and treatment, but the genomic architecture of COU is largely unknown. Comprehensive genomic screening study of 733 cases with three distinct COU subphenotypes revealed disease etiology in 10.0% of them. We detected no significant differences in the overall diagnostic yield among COU subphenotypes, with characteristic variable expressivity of several mutant genes. Our findings therefore may legitimize a genetic first diagnostic approach for COU, especially when burdening clinical and imaging characterization is not complete or available., Background: Congenital obstructive uropathy (COU) is a common cause of developmental defects of the urinary tract, with heterogeneous clinical presentation and outcome. Genetic analysis has the potential to elucidate the underlying diagnosis and help risk stratification., Methods: We performed a comprehensive genomic screen of 733 independent COU cases, which consisted of individuals with ureteropelvic junction obstruction ( n =321), ureterovesical junction obstruction/congenital megaureter ( n =178), and COU not otherwise specified (COU-NOS; n =234)., Results: We identified pathogenic single nucleotide variants (SNVs) in 53 (7.2%) cases and genomic disorders (GDs) in 23 (3.1%) cases. We detected no significant differences in the overall diagnostic yield between COU sub-phenotypes, and pathogenic SNVs in several genes were associated to any of the three categories. Hence, although COU may appear phenotypically heterogeneous, COU phenotypes are likely to share common molecular bases. On the other hand, mutations in TNXB were more often identified in COU-NOS cases, demonstrating the diagnostic challenge in discriminating COU from hydronephrosis secondary to vesicoureteral reflux, particularly when diagnostic imaging is incomplete. Pathogenic SNVs in only six genes were found in more than one individual, supporting high genetic heterogeneity. Finally, convergence between data on SNVs and GDs suggest MYH11 as a dosage-sensitive gene possibly correlating with severity of COU., Conclusions: We established a genomic diagnosis in 10.0% of COU individuals. The findings underscore the urgent need to identify novel genetic susceptibility factors to COU to better define the natural history of the remaining 90% of cases without a molecular diagnosis., (Copyright © 2023 by the American Society of Nephrology.)

In children, ureteropelvic junction obstruction (UPJO) is mostly caused by intrinsic factors (IUPJO) such as abnormal amounts of muscle and collagen deposition; extrinsic UPJO are rare and often due to crossing vessels (CVs). What is not clear is whether there is also intrinsic UPJ pathology in patients with CV. The aim of our study was to compare the histology of the two types of obstruction and to determine whether these histologic features are distinguishable enough to enable to identify the cause of obstruction based on histologic appearance alone. We retrospectively reviewed pathology reports of 38 children with UPJO that underwent surgery in our hospital from 2008 to 2022. The intrinsic and extrinsic groups consisted of 18 and 20 patients, respectively. After ematoxylin-eosin and Gomori's trichrome staining the specimens were scored for fibrosis and muscular hypertrophy in histhopatology, and CD117 antibody were used to detect interstitial Cajal-like cells. In our study, histological analyses revealed no differences between the CV and IUPJO specimens in terms of presence and degree of fibrosis and muscular hypertrophy; likewise, for presence of interstitial Cajal-like cells.

We present a rare case of an extragonadal retroperitoneal yolk sac tumour in an adult male, who presented with severe abdominal pain to his local hospital. Imaging revealed a large retroperitoneal soft tissue mass with no evidence of metastases. Initial biopsy demonstrated poorly differentiated carcinoma, favoured to be renal cell carcinoma. The patient underwent surgical resection following re-presentation with severe abdominal pain and significant interval enlargement of the mass. Laparotomy revealed a renal tumour that had ruptured through the left mesocolon into the peritoneal cavity. Postoperative histopathological examination revealed a yolk sac tumour involving the kidney, perinephric fat, renal sinus fat, renal hilar lymph node and colonic mesentery. Immunohistochemical staining for alpha-fetoprotein and glypican 3 was positive in the tumour cells without evidence of other germ cell elements, confirming the diagnosis of a pure yolk sac tumour. To our knowledge, this is an extremely rare case of a primary pure yolk sac tumour arising from the kidney in an adult., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Ureteropelvic junction obstruction (UPJO) is a congenital or acquired functionally significant impairment of urinary transport from the renal pelvis to the ureter. Congenital UPJO typically results from intrinsic disease such as the presence of an aperistaltic segment of the ureter, aberrant vessels or kidney abnormalities. Rare conditions can sometimes mimic an UPJO. We present a case of an 86-year-old woman with a UPJO diagnosed on CT. The patient was counseled on treatment options and elected to undergo a left uretherorenoscopy (URS) plus left laparoscopic pyeloplasty. The definitive histopathologic diagnosis was perinephric myxoid pseudotumor of fat, an extremely rare neoplasm, mass-forming. To the best of our knowledge, this is the first known case of a pseudotumor of fat causing UPJO. 6-month follow-up showed neither recurrence nor residual UPJO. We describe a rare presentation of extrinsic perinephric myxoid pseudotumor of fat causing UPJ obstruction. In elderly patients with no history of malignancy, UPJ obstruction can occur because of atypical masses., (© 2022. The Author(s) under exclusive licence to The Japan Society of Nephrology.)

Purpose: Horseshoe kidney (HSK) is the most common renal fusion anomaly, occurring in 0.25% of the population (1). It presents technical obstacles to pyeloplasty for ureteropelvic junction obstruction (UPJO) despite robotic assistance (2, 3). KangDuo-Surgical-Robot-01 (KD-SR-01), an emerging robotic platform in China, has yielded satisfactory outcomes in pyeloplasty (4, 5). We first describe our modified technique of robotic bilateral pyeloplasty for UPJO in HSK using KD-SR-01 system in the Lithotomy Trendelenburg position., Materials and Methods: A 36-year-old man with HSK and bilateral UPJO suffered right flank pain due to renal calculi (Figure-1). Repeated double-J stent insertion and ureteroscopy lithotripsy did not relieve his symptoms. A robot-assisted modified bilateral dismembered V-shaped flap pyeloplasty was performed using KD-SR-01 system in the Lithotomy Trendelenburg position., Results: Total operative time was 298 minutes with 50 ml estimated blood loss. There was no conversion to laparoscopic or open surgery. A follow-up of 14 months showed relieving symptoms and stable renal function. Cine magnetic resonance urography and computed tomography urography revealed improved hydronephrosis and good drainage. No intraoperative or postoperative complications occurred., Conclusions: It is technically feasible to perform a KD-SR-01-assisted modified bilateral dismembered V-shaped flap pyeloplasty in the Lithotomy Trendelenburg position for HSK. This procedure achieves managing UPJO on both sides without redocking the system and provides a wider operative field. In addition, it may be associated with better ergonomics, better cosmetic outcomes, and less possibility of postoperative bowel adhesion. However, further investigation is still warranted to confirm its safety, efficacy, and advantages over traditional procedures., Competing Interests: None declared., (Copyright® by the International Brazilian Journal of Urology.)

Lymphovascular invasion (LVI) predicts poor survival in patients with pathologically localized or locally advanced upper urinary tract urothelial carcinoma (UT-UC). However, LVI is associated with high tumor grade, tumor necrosis, advanced tumor stage, tumor location, concomitant carcinoma in situ, lymph node metastasis, and sessile tumor architecture. These factors might interfere with the analysis of the impact of LVI on oncological prognosis. To address this, this study aimed to clarify the relationship between LVI and patient prognosis in UT-UC using propensity score weighting. Data were collected from 789 patients with UT-UC treated with radical nephroureterectomy without chemotherapy. We evaluated the significance of LVI in predicting metastasis-free survival (MFS), cancer-specific survival (CSS), and overall survival (OS) using propensity score weighting. All weighted baseline characteristics included in the propensity score model were balanced between the LVI (+) and LVI (-) groups. The MFS, CSS, and OS were all significantly poorer in the LVI (+) group. For patients without LVI, the 5-year MFS, CSS, and OS rates were 65.3%, 73.1%, and 67.3%, respectively, whereas the corresponding rates were 50.2%, 63.8 %, and 54.6%, respectively, for patients with LVI. (all P < .001). For patients without LVI, the 10-year MFS, CSS, and OS rates were 61.5%, 69.6%, and 59.2%, respectively, whereas those for patients with LVI were 44.5%, 57.0%, and 42.7%, respectively (all P < .001). LVI is an important pathological feature that predicts metastasis development and worse survival outcome after radical surgery in UT-UC patients., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background/aim: There are few reports confirming the relationship between the therapeutic effects of adjuvant systemic chemotherapy and intravesical recurrence after radical nephroureterectomy for upper urinary tract urothelial carcinoma. We aimed to evaluate the benefits of adjuvant systemic chemotherapy on intravesical recurrence after radical nephroureterectomy for upper urinary tract urothelial carcinoma., Patients and Methods: We retrospectively reviewed the medical records of 133 patients with pathological T stage ≥3 upper urinary tract urothelial carcinoma or lymph node metastasis who underwent radical nephroureterectomy between January 2010 and September 2020 at our hospital and other satellite hospitals. In total, 60 patients received adjuvant systemic chemotherapy, and 73 did not. The Student's t-test and chi-square (χ 2 ) test were used to compare between-group differences. The log-rank test was utilized to compare differences in intravesical recurrence between patients with or without adjuvant systemic chemotherapy. Cox proportional hazards regression analysis was performed to identify the predictive factors of intravesical recurrence., Results: The median follow-up period was 25 months. Forty (30.1%) patients presented with intravesical recurrence. The 1-year intravesical recurrence-free survival rates of patients with and without adjuvant systemic chemotherapy were 86.0% and 70.2%, respectively (p=0.046). Multivariate analysis showed that adjuvant systemic chemotherapy was significantly associated with a lower risk of intravesical recurrence (p=0.032)., Conclusion: Patients with pathological T stage ≥3 upper urinary tract urothelial carcinoma or lymph node metastasis can have a satisfactory intravesical recurrence-free survival rate with adjuvant systemic chemotherapy., (Copyright © 2023 International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Nested subtype of urothelial carcinoma (NVUC) of the upper urinary tract is rare. While this subtype has an aggressive behavior in the urinary bladder, little is known about this subtype in the renal pelvis and ureter. In addition, due to NVUC's bland morphology and resemblance to von Brunn nests proliferation, which can be florid in the renal pelvis and ureter, establishing a diagnosis of NVUC in these sites can be difficult, especially on small biopsies. Nineteen cases of NVUC of the renal pelvis and the ureter were identified. One was in-house and 18 cases were sent to the genitourinary pathology consult service. Four cases were excluded due to difficulty in obtaining the slides from other institutions. In all, 8/15 (53.33%) cases showed large nested morphology, 4/15 (26.66%) cases showed mixed large and small nested morphology, and only 3/15 (20%) cases showed predominantly small nested morphology. In all cases, the invasive component was pure nested subtype, and tumors were all unifocal. In 8/15 cases, there was the presence of an overlying low-grade papillary urothelial carcinoma. An associated desmoplastic stromal reaction was none/minimal in 12/15 (80%) cases, with a prominent reaction in the remaining 3/15 (20%) cases. Pathologic stages for the resections were pT1 (n=1), pT2 (n=3), pT3 (n=8), and pT4 (n=1). There was only 1 case with a lymph node metastasis at the time of resection who had pT4 disease. Follow-up was available in 10/15 cases, 7/10 cases showed no recurrences or metastases, and the remaining 3/10 cases later developed urothelial carcinoma of the bladder. In 5/15 cases, follow-up could not be obtained. Similar to NVUC in the urinary bladder, NVUC in the upper tract typically lacks an associated desmoplastic reaction and has by definition bland cytology. In contrast to NVUC of the urinary bladder, NVUC in the upper urothelial tract is: (1) more frequently associated with an overlying papillary urothelial carcinoma; (2) more commonly has a large nested pattern; and (3) appears to behave less aggressively., Competing Interests: Conflicts of Interest and Source of Funding: The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Introduction: Diagnosing real urinary obstruction and surgical decision making for the operative correction of urinary obstruction, are still problematic in congenital hydronephrosis (CH). Compliance of the renal pelvis is one of the important defense mechanisms of renal parenchyma against urinary obstruction. We observed early loss of function in some cases of CH with low and moderate anteroposterior diameter of the renal pelvis (APDRP)., Objective: To evaluate structural properties of the renal pelvic tissue of patients with CH and the relation of these structural properties with renal function and Anteroposterior diameter of the renal pelvis., Study Design: Ureteropelvic junction (UPJ) excised during UPJ obstruction operations from 2013 to 2019 were evaluated histopathologically. The patients were divided into the two groups according to initial renal function, i.e. group with initial preoperative differential renal function (DRF) less than 35% and group with initial DRF greater than 35%. The percentage of collagen to whole tissue area were analyzed using image processing program. The relationship between DRF and tissue collagen ratio, SFU and APDRP was evaluated., Results: There were 5 patients in the DRF <35% group and 16 patients in the DRF >35% group. However, APDRP's of the DRF <35% group were also significantly lower than the DRF >35% group. The collagen distribution in the muscle layer was more prominent in the DRF <35% group. Proportionally, percentage of collagen stained surface was significantly higher in DRF <35% group., Discussion: There are numerous histopathological studies evaluating the cause of UPJ obstruction. Besides these studies that are oriented to etiology, there are many studies comparing the histopathological changes at UPJ with surgical outcome and prognosis. In the present study, we found that renal pelvis collagen ratio was significantly increased in patients with lower APDRP and with severe renal function loss. This increase in the collagen content in the renal pelvis have been shown to affect the compliance negatively and decrease APDRP, which leads to a faster loss of renal function. Thus, pelvic structural changes accompanying UPJ obstruction may aggravate urinary obstructive process., Conclusion: Increased renal pelvis collagen ratio negatively affects the expansion of the renal pelvis, which is one of the protective mechanisms of the renal parenchyma, and may be one of the triggering mechanisms of early loss of renal function., Competing Interests: Conflict of interest None., (Copyright © 2022 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Rationale: Mucinous adenocarcinoma of the renal pelvis is extremely rare in malignancy of the renal pelvis, and <100 cases have been reported so far. However, horseshoe kidneys appear to be more prone to this mucinous gland metaplasia. Due to the lack of a specific diagnostic basis, it is difficult to confirm the diagnosis before surgery, which is easy to cause misdiagnosis., Patient Concerns: We report a 64-year-old male patient who found a cystic mass in the left kidney during physical examination. CT examination showed a horseshoe kidney with a cystic mass 9.5 × 8.0 cm in front of the left kidney, lacking obvious diagnostic features., Diagnosis: It was misdiagnosed as cystic teratoma before the operation, and was diagnosed as mucinous adenocarcinoma of the renal pelvis through pathological examination after the operation., Interventions: Resection of the tumor by nephron-sparing surgery and postoperative chemotherapy., Outcomes: No tumor recurrence was found at 6 years of follow-up. After 7 years, the patient had multiple metastases in the abdominal wall and peritoneum, and no tumor recurrence was found in the urinary system. The patient received chemotherapy again and survived well., Lessons: The prognosis of nephron-sparing tumor resection for MRAP is not significantly different from that of radical nephroureterectomy + bladder cuff excision. Because it can reduce the risk that patients can not tolerate follow-up chemotherapy due to abnormal renal function after surgery, which may be more beneficial in patients with kidney abnormalities or chronic disease., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: Sarcomatoid carcinoma is a rare, high-grade malignancy with epithelial and mesenchymal components. It may be a good candidate for immunotherapy because it is associated with overexpression of programmed cell death ligand 1. Sarcomatoid urothelial carcinoma (UC) of the upper urinary tract is extremely rare. Here we report the first case of sarcomatoid UC of the renal pelvis that responded to immunotherapy., Case Presentation: A 79-year-old man was referred to our hospital complaining of various symptoms, including anorexia and abdominal pain. A computed tomography scan revealed a right atrial tumor, a 9 cm left renal mass with a renal vein tumor thrombus, para-aortic lymphadenopathy, and multiple small lung nodules. The patient underwent resection of the right atrial tumor. Pathological analysis of the tumor did not lead to an accurate diagnosis even after several rounds of immunohistochemistry. He underwent a needle biopsy of the left kidney and was initially diagnosed with collecting duct carcinoma, a rare subtype of renal cell carcinoma (RCC). Following the initial diagnosis, immunotherapy with nivolumab and ipilimumab commenced. Thereafter, almost all lesions, including the left renal tumor, were reduced in size. However, he underwent a left nephrectomy approximately a year after beginning immunotherapy due to repeated left renal bleeding. Histological examination of the nephrectomy specimen revealed two forms of cancer-sarcomatoid UC and conventional high-grade UC. Two months after surgery, the patient was found to have new lung metastases. He underwent chemotherapy with gemcitabine and cisplatin, followed by immunotherapy with pembrolizumab. However, both treatments were ineffective. The patient died of cancer 19 months after his first admission., Conclusions: The presented case of sarcomatoid UC of the renal pelvis that partially responded to immunotherapy suggests that immunotherapy can be a promising treatment for sarcomatoid UC., (© 2023. The Author(s).)

Although renal pelvic and ureteral urothelial carcinoma share similarities in their origins, disparities on a genetic and clinical level make them divergent entities. Clinical information from the Surveillance, Epidemiology, and End Results (SEER) database was used to validate the characteristics and molecular subtypes using single-center data, which were compared between the two types of muscle-invasive tumors. Simultaneously, to expand the sample size for further verification, we explored a deep learning algorithm to correctly classify molecular subtypes from H&E histology slides. We suggested that the renal pelvic group might have a proclivity towards luminal and the ureter towards basal and P53-like. Furthermore, we explore the heterogeneity of matrix and immune tumor microenvironment, and the ureteral group had more immune cell infiltration and higher stiffness. Collectively, these results showed that muscle-invasive upper tract urothelial carcinoma exist in distinct properties of clinical characteristics, molecular subtype, and tumor microenvironment., (© 2022 The Authors. Cancer Science published by John Wiley & Sons Australia, Ltd on behalf of Japanese Cancer Association.)

Background: With the advance in genome-wide analyses, genetic alternations have been found to play an important role in carcinogenesis and aggressiveness of UC. Through bioinformatic analysis of gene expression profiles of urinary bladder urothelial carcinoma (UBUC) from publicly available GEO dataset (GSE31684), Zinc finger and SCAN domain containing 4 (ZSCAN4) was identified as a significant downregulated gene in muscle-invasive bladder cancer when compared with non-muscle-invasive bladder cancer., Methods: The expression of ZSCAN4 was evaluated by immunohistochemistry in 340 upper urinary tract urothelial carcinomas (UTUCs) and 295 UBUCs. The expression profiles of ZSCAN4 and potential signaling pathways were analyzed bioinformatically., Results: In UTUC, low expression of ZSCAN4 was significantly associated with advanced primary pT stage (P = 0.011), increased nodal metastasis (P = 0.002) and increased vascular invasion (P = 0.019). In UBUC, low expression of ZSCAN4 was significantly correlated with advanced primary pT stage (P < 0.001), increased nodal metastasis (P = 0.001), high histological grade (P = 0.003) and increased vascular invasion (P = 0.003). In survival analysis, low expression of ZSCAN4 acted as an independent negative prognostic factor for disease-specific survival and metastasis-free survival both in UTUC and UBUC. Gene ontology analysis showed that ZSCAN4 mRNA and its co-downregulated genes are associated with the mitotic cell cycle., Conclusions: Low expression of ZSCAN4 predicted worse outcome in urothelial carcinoma and might have potential regulatory role in cell mitosis., (© 2023. The Author(s).)