No difference in oncological outcomes and perioperative complications between patients with ESRD with unilateral and bilateral UTUC receiving radical nephroureterectomy.

Patients with end stage renal disease (ESRD) are at high risk of developing upper tract urothelial carcinoma (UTUC). Due to high recurrence rate of UTUC in contralateral kidney and ureter, and high risk of complications related to surgery and anesthesia, whether it's necessary to remove both kineys and ureters at one time remains in debate. We utilized Taiwanese UTUC Registry Database to valuate the difference of oncological outcomes and perioperative complications between patients with ESRD with unilateral and bilateral UTUC receiving surgical resection. Patients with ESRD and UTUC were divided into three groups, unilateral UTUC, previous history of unilateral UTUC with metachronous contralateral UTUC, and concurrent bilatetral UTUC. Oncological outcomes, perioperative complications, and length of hospital stays were investiaged. We found that there is no diffence of oncological outcomes including overall survival, cancer specific survival, disease free survival and bladder recurrence free survival between these three groups. Complication rate and length of hospital stay are similar. Adverse oncological features such as advanced tumor stage, lymph node involvement, lymphovascular invasion, and positive surgical margin would negatively affect oncological outcomes.

Right-sided ureteral hemangiosarcoma in a paraplegic dog.

BACKGROUND: This study aims to describe a rare case of primary ureteral hemangiosarcoma, in which surgical intervention preserved the kidney and ureter after tumor removal. CASE PRESENTATION: A 13-year-old, neutered male dog, weighing 14 kg, mixed-breed, presented with apathy, anorexia, acute-onset vomiting, and abdominal discomfort during the physical examination. Ultrasonography and pyelography revealed a right-sided dilation of the renal pelvis and ureter due to complete obstruction in the middle third of the ureter. A mass obstructing the lumen of the right ureter was completely resected, and ureteral suturing was performed, preserving the integrity of the involved structures. Histopathology confirmed primary ureteral hemangiosarcoma. Due to the local and non-invasive nature of the mass, chemotherapy was not initiated. The patient's survival was approximately two years, and normal renal function was preserved throughout this period. CONCLUSIONS: Considering this type of tumor in the differential diagnosis of upper urinary tract obstructive disorders. Furthermore, the preservation of the ureter and kidney is a suitable therapeutic option after surgical resection of non-invasive tumors.

Retrograde Ureteral Stents Versus Percutaneous Nephrostomy in the Management of Malignant Ureteral Obstruction: A Systematic Review and Meta-Analysis.

OBJECTIVE: To evaluate outcomes in cancer patients with ureteral obstruction by comparison of retrograde stenting and percutaneous nephrostomy techniques. METHODS: Systematic review of all studies up to October 2023. Studies were identified from all major databases including MEDLINE, Cochrane, and EMBASE. All comparative studies between retrograde stenting and percutaneous nephrostomy were searched; studies with paediatric populations were excluded. Primary outcomes were procedure and intervention failure rates; secondary outcomes were infection, blockage, displacement, and unplanned exchange rates along with procedure time and length of stay. RESULTS: Eighteen studies with 1228 patients contributed to the summative outcome. Percutaneous nephrostomy was statistically superior to retrograde stenting for procedure failure rate (P <.00001) and intervention failure rate (P =.0004). Retrograde stenting was statistically superior to percutaneous nephrostomy for displacement rates (P = .003), procedure time (P <.00001), and length of stay (P <.00001). Retrograde stenting showed no difference to percutaneous nephrostomy for infection rates (P = .94), blockage rates (P = .93), unplanned exchange rates (P = .48), CONCLUSION: There is no absolute superiority for retrograde stenting or percutaneous nephrostomy for malignant ureteral obstruction. Both techniques have their advantages and disadvantages, with some comparable outcomes; patients are key when selecting the best technique. Larger studies are required to assess the outcomes of both techniques.

Perioperative renal function change and oncological outcomes of radical nephroureterectomy in patients with upper tract urothelial carcinoma: A multicenter retrospective study.

BACKGROUND: The effect of radical nephroureterectomy (RNUx) on postoperative renal function in patients diagnosed with upper tract urothelial carcinoma (UTUC) has not been thoroughly explored. METHODS: We conducted a retrospective analysis including 785 patients who underwent RNUx for UTUC. We assessed the preoperative and postoperative estimated glomerular filtration rates (eGFRs) and factors related to the decline in eGFR. Additionally, we examined the effect of comorbidities (diabetes or hypertension) on the postoperative eGFR at 1 year. Cox proportional hazard models were employed to investigate the clinical effect of RNUx on oncological outcomes, including non-urothelial tract recurrence-free survival (NUTRFS), cancer-specific survival (CSS), and overall survival (OS). RESULTS: The median preoperative and postoperative eGFR levels were 54.7 and 40.6 ml/min/1.73 m2 respectively. The proportions of patients with preoperative and postoperative eGFR ≥60 mL/min/1.73 m2 were 35.9% and 5.1%, respectively. The median decline in the eGFR after surgery was 26.8%. Patients with preoperative eGFR <60 ml/min/1.73 m2 demonstrated significantly lower odds of a postoperative decline in eGFR of 25% or more. The effect of comorbidities on postoperative eGFR at 1 year was significant (P = 0.048). The 3-year NUTRFS, CSS, and OS rates were 72.9%, 85.2%, and 81.5%, respectively. Preoperative chronic kidney disease was an independent factor associated with inferior NUTRFS, CSS, and OS. CONCLUSION: Different degrees of impairment of renal function occur among UTUC patients. Only 5.1% of patients retain a postoperative eGFR ≥60 ml/min/1.73 m2. Preoperative renal impairment was linked to reduced odds of postoperative eGFR decrease and associated with survival. In addition, the presence of comorbidities had a significant effect on the decline in eGFR. These findings emphasize the importance of developing evidence-based perioperative treatment strategies for UTUC patients with impaired renal function.

The Impact of Venous Thromboembolism on Upper Tract Urothelial Carcinomas Undergoing Open or Minimally Invasive Radical Nephroureterectomy in the USA: Perioperative Outcomes and Health Care Costs from Insurance Claims Data.

BACKGROUND AND OBJECTIVE: Venous thromboembolism (VTE) is a significant predictor of worse postoperative morbidity in cancer surgeries. No data have been available for patients with preoperative VTE and upper tract urothelial carcinoma (UTUC) undergoing radical nephroureterectomy (RNU). Our aim was to assess the impact of a preoperative VTE diagnosis on perioperative outcomes in the RNU context. METHODS: Patients aged 18 yr or older with a UTUC diagnosis undergoing RNU were identified in the Merative Marketscan Research deidentified databases between 2007 and 2021. Multivariable logistic regression adjusted by relevant perioperative confounders was used to investigate the association between a diagnosis of VTE prior to RNU and 90-d complication rates, postoperative VTE, rehospitalization, and total costs. A sensitivity analysis on VTE severity (pulmonary embolism [PE] and/or deep venous thrombosis [DVT]) was examined. KEY FINDINGS AND LIMITATIONS: Within the investigated cohort of 6922 patients, history of any VTE preceding RNU was reported in 568 (8.21%) cases, including DVT (n = 290, 51.06%), PE (n = 169, 29.75%), and superficial VTE (n = 109, 19.19%). The history of VTE before RNU was predictive of higher rates of complications, the most prevalent being respiratory complications (odds ratio [OR]: 1.78, 95% confidence interval [CI]: 1.43-2.22). Preoperative VTE was found to be associated with an increased risk of VTE following RNU (OR: 14.3, 95% CI: 11.48-17.82), higher rehospitalization rates (OR: 1.26, 95% CI 1.01-1.56) other than home discharge status (OR: 1.44, 95% CI: 1.18-1.77), and higher costs (OR 1.42, 95% CI: 1.20-1.68). Limitations include the retrospective nature and the use of an insurance database that relies on accurate coding and does not include information such as pathologic staging. CONCLUSIONS AND CLINICAL IMPLICATIONS: The presented findings will contribute to the counseling process for patients. These patients may benefit from enhanced pre/postoperative anticoagulation. More research is needed before the following results can be used in the clinical setting. PATIENT SUMMARY: Patients aged 18 yr or older with an upper tract urothelial carcinoma (UTUC) diagnosis undergoing radical nephroureterectomy (RNU) were identified in the Merative Marketscan Research deidentified databases between 2007 and 2021. Multivariable logistic regression adjusted by relevant perioperative confounders was used to investigate the association between a diagnosis of venous thromboembolism (VTE) prior to RNU and 90-d complication rates, postoperative VTE, rehospitalization, and total costs. A sensitivity analysis on VTE severity (pulmonary embolism and/or deep venous thrombosis) was examined. The presented findings will contribute to the counseling of patients with UTUC and preoperative VTE.

Analysis of the Therapeutic Efficacy of Laparoscopic Treatment for Fibroepithelial Polyps of the Ureter in Children.

Objective: The aim of this study is to assess the efficacy and safety of laparoscopic surgery in the treatment of pediatric ureteral fibroepithelial polyp (FEP) patients. Our hypothesis is that laparoscopic surgery can effectively treat FEPs while minimizing patient discomfort and complications. Our research aims to evaluate the clinical outcomes of the surgery, including postoperative symptom relief, improvement in kidney function, and risk of postoperative complications. Methods: The clinical records of 34 patients who underwent ureteral polyp surgery at the Department of Urology at Anhui Provincial Children's Hospital between May 2014 and February 2023 were retrospectively analyzed. All patients underwent laparoscopic surgery. Among the 34 pediatric patients, there were 31 males and 3 females, with 2 on the right side and 32 on the left side. Of these cases, 24 polyps were located at the ureteropelvic junction, while seven were found in the upper segment of the ureter and three in its middle segment. Patients' ages ranged from 4 years and 3 months to 15 years, with a median age of 8 years and 6 months. All children presented with varying degrees of hydronephrosis, and preoperative clinical symptoms included ipsilateral flank or abdominal pain, hematuria, and other discomfort. Preoperative examinations mainly comprised ultrasound, intravenous pyelography, CT, or magnetic resonance urography imaging studies, as well as diuretic renography. All pediatric patients underwent laparoscopic excision of the polyp segment of the ureter, followed by renal pelvis ureteroplasty or ureter-to-ureter anastomosis. Results: All patients underwent surgery without conversion to open surgery. The surgical duration ranged from 72 to 313 minutes, with an average of 179.5 minutes. The average intraoperative blood loss was 14 mL. Postoperatively, one patient experienced leakage at the anastomotic site; however, no other significant complications occurred during or after the procedure. Postoperative histopathology confirmed the presence of FEPs in the ureter for all cases. All patients experienced a favorable postoperative recovery, with hospitalization periods ranging from 3 to 16 days and an average stay of 8.6 days. A Double-J stent was inserted in all patients for a duration of 1 to 2 months after surgery, and upon removal, follow-up color Doppler ultrasound revealed reduced hydronephrosis within 1 to 3 months. Follow-up examinations were conducted at intervals ranging from 3 to 108 months postsurgery, with an average follow-up time of 42.2 months, during which no recurrence of ureteral polyps or symptoms such as pain and hematuria was observed. Conclusions: The findings of this study demonstrate that laparoscopic excision of the polyp segment of the ureter, renal pelvis ureteroplasty, and ureter-to-ureter anastomosis represent safe and effective treatment modalities for pediatric FEPs in the ureters. This technique offers several advantages, including minimal invasiveness, rapid recovery, and definitive therapeutic efficacy, which effectively alleviate clinical symptoms and improve hydronephrosis.

The influence of the initial clinical presentation of upper tract urothelial carcinoma on histopathological tumor features.

PURPOSE: To investigate the influence of the initial clinical presentation (symptomatic vs. asymptomatic) on histopathological tumor features in patients with upper tract urothelial carcinoma (UTUC). METHODS: We conducted a single-center, cross-sectional, and retrospective study that enrolled 72 adults with primary UTUC who underwent radical nephroureterectomy at our institution over a period of 4 years (April 2019-April 2023). RESULTS: Symptomatic patients exhibited significantly higher frequencies of high-grade UTUC (73.6% vs. 36.8%, p = 0.006), ≥ T2 stage UTUC (60.4% vs. 26.3%, p = 0.007), and larger tumor sizes (median 5 vs. 4 cm, p = 0.015) compared to asymptomatic patients. Multiple regression analyses demonstrated significant associations between symptomatic presentation and the presence of high-grade UTUC (OR 6.35, 95% CI 1.81-22.27, p = 0.004), ≥ T2 stage UTUC (OR 5.98, 95% CI 1.62-22.08, p = 0.007), and larger tumor size (B 3.14, 95% CI 0.62-5.66, p = 0.015). A subset of patients with hematuria was separately analyzed to assess the influence of hematuria severity (gross vs. microscopic) on UTUC characteristics. Patients with gross hematuria exhibited significantly higher frequencies of high-grade UTUC (72.9% vs. 33.3%, p = 0.048) and ≥ T2 stage UTUC (58.3% vs. 22.2%, p = 0.001). Multiple regression analyses showed significant associations between gross hematuria and the presence of high-grade UTUC (OR 6.34, 95% CI 1.15-34.95, p = 0.034) and ≥ T2 stage UTUC (OR 6.54, 95% CI 1.11-38.93, p = 0.039). CONCLUSION: Initial symptomatic presentation was independently associated with adverse histopathological UTUC characteristics, potentially attributed to earlier detection of UTUC in asymptomatic patients, before the onset of symptoms.

Promising Response of Paclitaxel in Metastatic Transitional Cell Carcinoma of Distal Ureter Complicated with Hydroureteronephrosis: A Case Report.

Ureteric carcinoma is the rarest of all urothelial malignancies, and little attention has been given to it. Palliation in these groups of patients is a dilemma in the clinics. Use of chemotherapeutic agents in ureteric carcinoma is a double edged sword, as these patients had already impaired renal function due to post-renal failure and nephrotoxic nature of most of the chemotherapeutic agents can further deteriorate the renal function, making the management approach, a relatively visionary task. Here, we present a case of a 77-year-old female with metastatic ureteric carcinoma locally complicated with hydroureteronephrosis, coming to us with gross haematuria, lower abdominal pain along with cough. Apart from age factor of the lady, presence of hydroureteronephrosis and pulmonary metastases was another challenge for us. Paclitaxel remains the mainstay of our treatment. Keywords: carcinoma; case reports; metastasis; paclitaxel.

Chondrosarcoma of Ureter in an Elderly Patient: A Case Report.

Chondrosarcoma is a rare type of cancer that can affect the upper urinary tract. Because of its rarity, the clinical presentation of chondrosarcoma can be similar to other urinary tract conditions, such as renal colic, hematuria, and urothelial carcinoma. The primary treatment for chondrosarcoma is the surgical removal of the tumor, and radiation or chemotherapy may be used for advanced cases. However, because of the limited number of patients with this condition, there are no established guidelines for chemotherapy, and the outcomes are unclear. In this case, we present a 71-year-old female patient who was diagnosed with ureteral chondrosarcoma. She presented with abdominal pain and hydronephrosis, and a tumor was found beneath a small stone. The patient underwent nephroureterectomy and received oral fluorouracil chemotherapy due to the advanced stage of the disease. Fortunately, the patient survived, and at the 7 months post-operative follow-up there was no evidence of recurrence. In conclusion, the chondrosarcoma of the upper urinary tract is a rare condition that can be difficult to diagnose due to its similarity to other urinary tract conditions. Treatment typically involves the surgical removal of the tumor, with radiation or chemotherapy reserved for advanced cases. However, because of the limited number of patients, there are no established guidelines for chemotherapy, and the outcomes of treatment are unclear.

Gross hematuria as the presentation of ureteral paraganglioma: a case report and literature review.

BACKGROUND: Paraganglioma of genitourinary tract is uncommon, and origin from ureter is even rarer. We aim to present a case of paraganglioma from ureter in a 48-year-old female patient, who presented with gross hematuria. CASE PRESENTATION: We present a 48-year-old female who complained of gross hematuria for one week. A left ureteral tumor was found by image study. However, hypertension was unexpectedly recorded during diagnostic ureteroscopy survey. Due to persisted gross hematuria and bladder tamponade, she underwent left nephroureterectomy with bladder cuff resection. Blood pressure surged again when the tumor was surgically approached. Ureteral paraganglioma was confirmed according to pathological report. After the surgery, the patient recovered well, and no more gross hematuria was noted. She is now under regular follow-up at our outpatient clinic. CONCLUSION: Ureteral paraganglioma should be kept in mind not only when blood pressure fluctuates during operation, but also before we manipulate the ureteral tumor when gross hematuria is the only sign. Whenever the presumption of paraganglioma is raised, laboratory evaluation and anatomical or even functional imaging should be considered. The concomitant anesthesia consultation before the surgery should not be deferred, either.

A rare cause of hydronephrosis: Leiomyoma of the ureter and a literature review.

INTRODUCTION: Leiomyomas of the genitourinary tract are rare and their manifestation in the ureter is even rarer. To our knowledge, only 14 cases of leiomyoma of the ureter have been reported worldwide since 1955, therefore this case will be 15th. CASE PRESENTATION: We present a rare case of primary leiomyoma of the right ureter. Ureteroscopy did not show any abnormal findings in the ureteral mucosa. The primary leiomyoma was resected with distal ureterectomy and partial cystectomy that was followed with ureteroneocystostomy due to extraluminal mass that caused hydronephrosis and back pain. CONCLUSION: Although rare, we believe that leiomyoma should be considered in the differential diagnoses of well-circumscribed ureteral masses and kidney-sparing surgery should be performed.

Robotic distal ureterectomy and re-implant for obstructing metastatic melanoma - surgical considerations in the palliative setting.

Non-urothelial malignant ureteral obstruction (MUO) causes hydronephrosis, renal damage and infectious sequelae. The overall condition, symptoms, and plans for systemic therapy inform urologic intervention. In well-selected cases, there is a role for definitive reconstruction. We describe a robotic-assisted distal ureterectomy and reimplant for definitive repair of obstructive metastatic melanoma.

Tranexamic acid-induced ureteric clot obstruction in a patient with urothelial cell carcinoma resulting in upper urinary tract perforation.

This is the first report of upper urinary tract (UUT) perforation secondary to tranexamic acid (TXA) induced ureteric clot obstruction. A 77-year-old woman was referred to the urology department with intermittent, painless visible haematuria from a lesion in the right upper calyx, suspicious of urothelial cell carcinoma. She did not have any flank pain or blood clots in her urine. Preoperatively, her haemoglobin level dropped from 113 g/L to 95 g/L and was prescribed oral TXA by her general practitioner, which led to extensive clot formation within the right kidney and ureter resulting in pain and ureteric obstruction. At ureteroscopy, a large ureteric blood clot was extracted and perforation close to the tumour with resultant urinary extravasation was noted. The patient subsequently underwent a successful nephroureterectomy, but risk of potential perforation-related complications such as tumour seeding, sepsis and urinoma formation could have been avoided. TXA in haematuria from the UUT should be strongly discouraged due to the risk of ureteric clot obstruction and UUT perforation.

Bilateral obstructive megaureter with right lower ureteric urothelial carcinoma: a very rare coalition.

Upper tract urothelial cancers account for 5% of all urothelial cancers. Among these, more than 90% of tumours are located in the renal pelvis, while ureteric tumours are extremely rare. The distal ureter is the most common location, although multifocal implants may also occur. Megaureter is a common cause of obstructive uropathy in neonates and children. Nevertheless, it may be unnoticed, if asymptomatic. Megaureter may be obstructing or refluxing. Long-standing urinary stasis and recurrent urinary tract infection in megaureter may cause chronic irritation of the ureteric mucosa leading to dysplasia and malignancy. We report a 55-year-old man diagnosed with bilateral obstructive megaureter with right lower ureteric urothelial cancer and review the current literature.

Bladder and upper urinary tract cancers as first and second primary cancers.

BACKGROUND: Previous population-based studies on second primary cancers (SPCs) in urothelial cancers have focused on known risk factors in bladder cancer patients without data on other urothelial sites of the renal pelvis or ureter. AIMS: To estimate sex-specific risks for any SPCs after urothelial cancers, and in reverse order, for urothelial cancers as SPCs after any cancer. Such two-way analysis may help interpret the results. METHODS: We employed standardized incidence ratios (SIRs) to estimate bidirectional relative risks of subsequent cancer associated with urothelial cancers. Patient data were obtained from the Swedish Cancer Registry from years 1990 through 2015. RESULTS: We identified 46 234 urinary bladder cancers (75% male), 940 ureteral cancers (60% male), and 2410 renal pelvic cancers (57% male). After male bladder cancer, SIRs significantly increased for 9 SPCs, most for ureteral (SIR 41.9) and renal pelvic (17.2) cancers. In the reversed order (bladder cancer as SPC), 10 individual FPCs were associated with an increased risk; highest associations were noted after renal pelvic (21.0) and ureteral (20.9) cancers. After female bladder cancer, SIRs of four SPCs were significantly increased, most for ureteral (87.8) and pelvic (35.7) cancers. Female bladder, ureteral, and pelvic cancers associated are with endometrial cancer. CONCLUSIONS: The risks of recurrent urothelial cancers were very high, and, at most sites, female risks were twice over the male risks. Risks persisted often to follow-up periods of >5 years, motivating an extended patient follow-up. Lynch syndrome-related cancers were associated with particularly female urothelial cancers, calling for clinical vigilance.

The IDENTIFY study: the investigation and detection of urological neoplasia in patients referred with suspected urinary tract cancer - a multicentre observational study.

OBJECTIVE: To evaluate the contemporary prevalence of urinary tract cancer (bladder cancer, upper tract urothelial cancer [UTUC] and renal cancer) in patients referred to secondary care with haematuria, adjusted for established patient risk markers and geographical variation. PATIENTS AND METHODS: This was an international multicentre prospective observational study. We included patients aged ≥16 years, referred to secondary care with suspected urinary tract cancer. Patients with a known or previous urological malignancy were excluded. We estimated the prevalence of bladder cancer, UTUC, renal cancer and prostate cancer; stratified by age, type of haematuria, sex, and smoking. We used a multivariable mixed-effects logistic regression to adjust cancer prevalence for age, type of haematuria, sex, smoking, hospitals, and countries. RESULTS: Of the 11 059 patients assessed for eligibility, 10 896 were included from 110 hospitals across 26 countries. The overall adjusted cancer prevalence (n = 2257) was 28.2% (95% confidence interval [CI] 22.3-34.1), bladder cancer (n = 1951) 24.7% (95% CI 19.1-30.2), UTUC (n = 128) 1.14% (95% CI 0.77-1.52), renal cancer (n = 107) 1.05% (95% CI 0.80-1.29), and prostate cancer (n = 124) 1.75% (95% CI 1.32-2.18). The odds ratios for patient risk markers in the model for all cancers were: age 1.04 (95% CI 1.03-1.05; P < 0.001), visible haematuria 3.47 (95% CI 2.90-4.15; P < 0.001), male sex 1.30 (95% CI 1.14-1.50; P < 0.001), and smoking 2.70 (95% CI 2.30-3.18; P < 0.001). CONCLUSIONS: A better understanding of cancer prevalence across an international population is required to inform clinical guidelines. We are the first to report urinary tract cancer prevalence across an international population in patients referred to secondary care, adjusted for patient risk markers and geographical variation. Bladder cancer was the most prevalent disease. Visible haematuria was the strongest predictor for urinary tract cancer.

Large distal ureteric stone with high burden urothelial cancer of the entire ureter and renal pelvis: a dual pathology.

Upper-tract urothelial cancer comprises only 3% of all urothelial cancers. Risk factors include tobacco smoking, recurrent urinary infection, urolithiasis and analgesic abuse. Urolithiasis-induced chronic inflammation leads to urothelial proliferation and eventual malignant transformation. The most common association is reported with squamous cell cancer. A 54-year man under evaluation for right flank pain was diagnosed with a large distal ureteric stone and urothelial cancer of the entire right ureter and renal pelvis. The patient underwent right nephroureterectomy and stone retrieval, with urinary bladder cuff excision and pelvic lymph node dissection. On follow-up, the patient succumbed to disease recurrence with widespread metastasis. Urothelial cancer associated with stone disease is atypical. Long-standing inflammation causing metaplastic and dysplastic changes is a possible hypothesis. Careful assessment of the malignancy should be looked for in patients with long-standing obstruction due to stone disease.

Tertiary lymphoid tissue in early-stage IgG4-related tubulointerstitial nephritis incidentally detected with a tumor lesion of the ureteropelvic junction: a case report.

BACKGROUND: IgG4-related kidney disease causes renal impairment of unknown pathogenesis that may progress to kidney failure. Although ectopic germinal centers contribute to the pathogenesis of the head and neck lesions of IgG4-related disease, the presence of tertiary lymphoid tissue (TLT) containing germinal centers in IgG4-RKD has rarely been reported. CASE PRESENTATION: We report a 72-year-old Japanese man who had IgG4-related tubulointerstitial nephritis (TIN) with TLT formation incidentally detected in a resected kidney with mass lesion of IgG4-related ureteritis in the ureteropelvic junction. During follow-up for past surgical resection of a bladder tumor, renal dysfunction developed and a ureter mass was found in the right ureteropelvic junction, which was treated by nephroureterectomy after chemotherapy. Pathology revealed no malignancy but abundant IgG4-positive cell infiltration, obliterative phlebitis and storiform fibrosis, confirming the diagnosis of IgG4-related ureteritis. In the resected right kidney, lymphoplasmacytes infiltrated the interstitium with focal distribution in the renal subcapsule and around medium vessels without storiform fibrosis, suggesting the very early stage of IgG4-TIN. Lymphocyte aggregates were also detected at these sites and consisted of B, T, and follicular dendritic cells, indicating TLT formation. IgG4-positive cells infiltrated around TLTs. CONCLUSIONS: Our case suggests that TLT formation is related with the development of IgG4-TIN and our analysis of distribution of TLT have possibility to elucidate IgG4-TIN pathophysiology.