Urothelial Cell Carcinoma of the Renal Pelvis Misdiagnosed as Ureteropelvic Junction Obstruction: A Case Report.
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ABSTRACT:
SUBMITTER: Choi JH
PROVIDER: S-EPMC8622892 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Urothelial carcinoma (UC) is a common type of carcinoma worldwide, and it is rare to find it metastasize to the brain. Antibody-drug conjugates (ADCs) are an emerging treatment for patients. Our department treated a patient with Urothelial carcinoma (UC) that metastazied to the brain with Disitamab Vedotin. Therefore, the aim of this article is to review and share this rare carcinoma case treated in our department, and to have a deep understanding of Antibody-drug conjugates (ADCs) so that we can improve treatment for Urothelial carcinoma (UC). Informed consent was granted by the patient to share the case information.
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Project description:Spontaneous rupture of the urinary collecting system with extravasation of the urine is a very rare condition. This situation is commonly associated with an obstructing urinary stone. Herein, we report a case of an 86-year-old patient who has admitted to the emergency service with left flank pain continuing for 7 days and pain has exacerbated in the past 24 h. The patient had nausea, vomiting, and tenderness on the left side of the abdomen and left flank region. The patient was diagnosed with an 8 mm left kidney stone a month ago, and hydration and oral analgesics were recommended to the patient. Spontaneous rupture of the renal pelvis and urinary extravasation were detected by contrast-enhanced computed tomography scan. Double-J ureteral stent was placed to control symptoms and eliminate extravasation. In this paper, diagnosis and treatment options for spontaneous renal pelvis rupture are discussed.
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Project description:Intrinsic ureteropelvic junction obstruction is the most common cause of congenital hydronephrosis, yet the underlying pathogenesis is undefined. Hedgehog proteins control morphogenesis by promoting GLI-dependent transcriptional activation and inhibiting the formation of the GLI3 transcriptional repressor. Hedgehog regulates differentiation and proliferation of ureteric smooth muscle progenitor cells during murine kidney-ureter development. Histopathologic findings of smooth muscle cell hypertrophy and stroma-like cells, consistently observed in obstructing tissue at the time of surgical correction, suggest that Hedgehog signaling is abnormally regulated during the genesis of congenital intrinsic ureteropelvic junction obstruction. Here, we demonstrate that constitutively active Hedgehog signaling in murine intermediate mesoderm-derived renal progenitors results in hydronephrosis and failure to develop a patent pelvic-ureteric junction. Tissue obstructing the ureteropelvic junction was marked as early as E13.5 by an ectopic population of cells expressing Ptch2, a Hedgehog signaling target. Constitutive expression of GLI3 repressor in Ptch1-deficient mice rescued ectopic Ptch2 expression and obstructive hydronephrosis. Whole transcriptome analysis of isolated Ptch2+ cells revealed coexpression of genes characteristic of stromal progenitor cells. Genetic lineage tracing indicated that stromal cells blocking the ureteropelvic junction were derived from intermediate mesoderm-derived renal progenitors and were distinct from the smooth muscle or epithelial lineages. Analysis of obstructive ureteric tissue resected from children with congenital intrinsic ureteropelvic junction obstruction revealed a molecular signature similar to that observed in Ptch1-deficient mice. Together, these results demonstrate a Hedgehog-dependent mechanism underlying mammalian intrinsic ureteropelvic junction obstruction.
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Project description:Prenatal hydronephrosis is a common condition that may spontaneously resolve after birth. However, this condition can result in renal damage and requires surgical correction in a number of cases. Preventing renal damage is paramount, but existing diagnostic technology is invasive, exposes infants to radiation, is costly, and is often indeterminate. A better understanding of the pathophysiology of renal obstruction as reflected in the urinary proteome may provide new insights into the disease that could potentially alter the clinical management of hydronephrosis. We performed a quantitative proteomics study of urine that was surgically obtained from eight clinically significant, unilaterally obstructed infants versus eight healthy controls, with the goal of identifying quantitatively varying proteins and the biological networks associated with them. Notably, urine was obtained from both the obstructed kidney and the bladder. Over 1100 proteins were identified, and a total of 76 quantitatively varying proteins were identified. Proteins involved in oxidative stress, inflammation, and renal disease pathways showed the most significant abundance differences. This study gives a deeper understanding of the critical proteomic changes associated with renal obstruction and represents the deepest proteomic profile of renal obstruction to date.
| S-EPMC4974339 | biostudies-literature
Project description:BackgroundUreteropelvic junction obstruction (UPJO) is one of the most common causes of hydronephrosis in children. This study explored the effects and the regulatory mechanisms of the circular RNA (circRNA) hsa_circRNA_0045861 (circRNA_0045861) in UPJO.MethodsRNA sequencing was used to identify the differentially expressed circRNAs in UPJO. The effects of circRNA_0045861 on renal cell apoptosis was investigated by flow cytometry and Western blot analysis. Furthermore, we used bioinformatics methods to predict the possible target genes of circRNA_0045861. Fluorescence in-situ hybridization and dual-luciferase reporter assays were performed to validate the target genes of circRNA_0045861. Finally, we evaluated the effects of circRNA_0045861 target gene miR-181d-5p on UPJO-induced renal fibrosis in vivo.ResultsRNA sequencing identified 63 upregulated and 64 downregulated circRNAs in UPJO patients. The expression of circRNA_0045861 was significantly elevated in kidney damage both in vivo and in vitro. Silencing circ_0045861 inhibited transforming growth factor (TGF)-β1-induced apoptosis in vitro in human kidney 2 (HK-2) cells. Furthermore, circ_0045861 was shown to directly interact with the microRNA miR-181d-5p and regulate the expression of sirtuin 1 (SIRT1), thereby promoting the progression of apoptosis and renal injury. In addition, overexpression of miR-181d-5p inhibited cell apoptosis and renal fibrosis in a mouse model through downregulating the SIRT1/p53 pathway.ConclusionsCirc_0045861 may be a novel candidate circRNA in the pathogenesis of UPJO by acting as a pro-apoptotic factor via the miR-181d-5p/SIRT1 axis.
| S-EPMC8576705 | biostudies-literature
Project description:BackgroundUrothelial carcinoma (UC) of the renal pelvis with renal vein and inferior vena cava (IVC) tumor thrombus (TT) was extremely rare. We aimed to explore the clinical and pathological characteristics, diagnosis and treatment of renal pelvis UC with renal vein and IVC TT.MethodsFrom March 2016 to January 2019, eight patients of renal pelvis UC with renal vein and IVC TT were diagnosed and underwent operation in our hospital. Clinical features, operative details, pathological outcomes, and prognosis data were reviewed and collected.ResultsThere were five males and three females (52-84 years old). Their main symptoms were flank pain and hematuria. According to the Mayo classification, the TT was 4 level-0 (1 left and 3 right), 2 level-I (right), and 2 level-II (right). Half the patients underwent retroperitoneal laparoscopic radical nephroureterectomy with thrombectomy, and the other underwent open procedures. The mean operative time was 298.9 minutes. Pathological outcomes revealed high-grade UC, with positive lymph nodes in 6 cases. Four patients received adjuvant chemotherapy, one target therapy and one adjuvant chemotherapy combined with immunotherapy after surgery. The mean follow-up time was 11.1 months. Three patients are alive, and two of them developed recurrence and lung metastasis.ConclusionsPreoperative differentiation between renal pelvis UC and renal cell carcinoma with venous TT was very important for the management. Radical nephroureterectomy with thrombectomy might be a reasonable method for renal pelvis UC with venous TT. The prognosis of such cases was poor even if adjuvant therapy was scheduled.
| S-EPMC8350241 | biostudies-literature
Project description:BackgroundThe current study was conducted to assess the impact of lymphovascular invasion on the survival of patients with urothelial carcinoma of the renal pelvis.MethodsPatients with urothelial carcinoma of the renal pelvis who underwent radical nephroureterectomy from 2010 through 2015 were identified in the National Cancer Data Base. Patients were characterized according to demographic and clinical factors, including pathologic tumor stage and lymphovascular invasion. Associations with overall survival were assessed through proportional hazards regression analysis.ResultsA total of 4177 patients were identified; 1576 had lymphovascular invasion. Patients with T3 disease and lymphovascular invasion had 5-year survival that was significantly worse than that of patients with T3 disease without lymphovascular invasion (34.7% vs 52.6; P < .001 by the log-rank test), and approached that of patients with T4 disease without lymphovascular invasion (34.7% vs 26.5%; P = .002). On multivariate analysis controlling for age, comorbidities, grade, lymph node status, surgical margin status, race, sex, and chemotherapy administration, patients with T3 disease and lymphovascular invasion also were found to have significantly worse survival compared with patients with T3 disease without lymphovascular invasion (hazard ratio, 1.7; 95% confidence interval, 1.4-1.91).ConclusionsLymphovascular invasion status is a key prognostic marker that can stratify the risk of patients with pT3 upper tract urothelial carcinoma further. Patients with this pathologic feature should be carefully considered for clinical trials exploring existing and novel therapies. Cancer 2018;124:2507-14. © 2018 American Cancer Society.
| S-EPMC8248269 | biostudies-literature
Project description:ImportanceExtracellular matrix proteins and enzymes involved in degradation have been found to be associated with tissue fibrosis and ureteropelvic junction obstruction (UPJO). In this study we developed a promising urinary biomarker model which can identify reduced renal function in UPJ obstruction patients. This can potentially serve as a non-invasive way to enhance surgical decision making for patients and urologists.ObjectiveWe sought to develop a predictive model to identify UPJO patients at risk for reduced renal function.DesignProspective cohort study.SettingPre-operative urine samples were collected in a prospectively enrolled UPJO biomarker registry at our institution. Urinary MMP-2, MMP-7, TIMP-2, and NGAL were measured as well as clinical characteristics including hydronephrosis grade, differential renal function, t1/2, and UPJO etiology.ParticipantsChildren who underwent pyeloplasty for UPJO.Main outcome measurementPrimary outcome was reduced renal function defined as MAG3 function <40%. Multivariable logistic regression was applied to identify the independent predictive biomarkers in the original Training cohort. Model validation and generalizability were evaluated in a new UPJO Testing cohort.ResultsWe included 71 patients with UPJO in the original training cohort and 39 in the validation cohort. Median age was 3.3 years (70% male). By univariate analysis, reduced renal function was associated with higher MMP-2 (p = 0.064), MMP-7 (p = 0.047), NGAL (p = 0.001), and lower TIMP-2 (p = 0.033). Combining MMP-7 with TIMP-2, the multivariable logistic regression model predicted reduced renal function with good performance (AUC = 0.830; 95% CI: 0.722-0.938). The independent testing dataset validated the results with good predictive performance (AUC = 0.738).Conclusions and relevanceCombination of urinary MMP-7 and TIMP-2 can identify reduced renal function in UPJO patients. With the high sensitivity cutoffs, patients can be categorized into high risk (aggressive management) versus lower risk (observation).
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Project description:Background:Collecting (Bellini) duct carcinoma (CDC) or Bellini duct carcinoma (BDC) is a rare subtype of kidney tumors, accounting for less than 3% and known to have the worst prognosis. It is known to have multiple clinical presentations; this is why it can be easily misdiagnosed. The aim of this article is to present a case of CDC that was initially misdiagnosed with urothelial papillary carcinoma (UPC) in a 41-year-old male. Case Presentation. Our patient presented with a left flank pain evolving for one month and one episode of gross macroscopic hematuria. Upon presentation, he had left costovertebral angle tenderness. Initial lab tests were normal. Computed tomography revealed a 5?cm solid mass of the left renal pelvis and multiple infracentimetric perihilar lymph nodes. Subsequently, the patient had left nephroureterectomy. Microscopic examination showed the presence of a high-grade urothelial papillary carcinoma of the renal pelvis' lumen. All four of the dissected lymph nodes showed disease metastasis. Three years after establishing the diagnosis, the patient presented again for chronic abdominal pain, with a recent history of weight loss. CT scan showed a left paraaortic mass infiltrating the left psoas muscle over a length of 12?cm. Immunohistochemical profiling of this mass confirmed the diagnosis of Bellini duct carcinoma, rejecting the initial diagnosis of UPC. Therefore, the patient required a cisplatin-gemcitabine-based chemotherapy regimen. Conclusion:BDC remains one of the rare aggressive subtypes of RCC, having a multitude of initial clinical presentations and an unfavorable prognosis. In this patient, CDC was masquerading as a transitional cell carcinoma that should always be kept in mind as a possible presentation. Corresponding early imaging and histopathology exams are primordial for a correct diagnosis and thus a better prognosis.
| S-EPMC7035507 | biostudies-literature
Project description:Expression profiling by arrays Urothelial carcinoma (UC) can arise at any location along the urothelial tract, including the urethra, bladder, ureter or renal pelvis. Although tumors arising in these various locations demonstrate similar morphology, it is unclear whether the gene expression profiles are similar in the upper tract (ureter and renal pelvis) or in the lower tract (bladder and urethra) carcinomas, especially given their different embryologic origins. As differences may facilitate potentially different screening and treatment modalities, we sought to examine the relationship between urothelial carcinoma of the renal pelvis (rUC) and urothelial carcinoma of the bladder (bUC). Fresh tumor tissue was collected from patients with bUC (n=10) and benign mucosa from the bladder (n=7) was collected from individuals undergoing resection for non-UC conditions for comparison. Gene expression profiles from these samples were determined using high-throughput Affymetrix gene expression microarray chips. Bioinformatic approaches were used to compare gene expression profiles of these samples and those of rUC (n= 14) and normal kidney (n=14) that were mostly used in our previous publication. Using unsupervised analytic approaches, rUC and bUC were indistinguishable. When supervised analytic approach was used, a very small number of potentially differentially expressed genes was identified; these differences were most likely to be limited to a single pathway - the chloride ion binding activity pathway -which was more frequently activated in rUC than in bUC. We found that the gene expression profiles of UCs from the upper and lower tract were extremely similar, suggesting that similar pathogenic mechanisms likely function in the development of these tumors. The differential expression of genes in the identified pathway may represent a potential new avenue for detection of upper tract tumors.
2010-12-31 | GSE24152 | GEO