Rare case of stromal predominant Wilm's tumour with rhabdomyoblastic differentiation in FNAC smears.

The FNAC smear and histopathology of stromal predominant Wilm's tumour with rhabdomyoblastic Differentiation along with immunostaining in a 7-year-old male. In this paper, the diagnostic potential of FNAC in identifying rare histological variants of paediatric renal tumours is highlighted.

An Unusual Case of Extramedullary Hematopoiesis in the Right Kidney.

Extramedullary hematopoiesis (EMH) is the formation of blood cells outside the bone marrow, typically occurring in response to chronic anemia or bone marrow dysfunction. While EMH is most commonly observed in the liver, spleen, and lymph nodes, its occurrence in the kidney is exceedingly rare. In this case report, we are presenting a case of a 49-year-old male diagnosed with polycythemia vera who had an incidental right renal mass, which was histo-pathologically proven as extramedullary hematopoiesis in the right kidney mimicking lymphoma. This case underscores the importance of considering EMH in the differential diagnosis of renal masses, especially in patients with a history of myeloproliferative disorders. Early recognition and appropriate management are crucial to avoid unnecessary interventions and manage the underlying hematological condition effectively. Accurate diagnosis through histopathological examination is crucial to avoid unnecessary surgical interventions.

Significance of Charlson co-morbidity index as predictive factor for post-op complications after radical nephrectomy.

The current study planned to explore the correlation between an elevated Charlson Comorbidity Index score and post-operative complications following radical nephrectomy in patients with renal cell carcinoma. A total of 70 patients aged 30-80 years undergoing radical nephrectomy were categorised into low Charlson Comorbidity Index score <4 group A and high score >4 group B. Post-operatively, complications were noted in 21(30%) patients, with higher grades more prevalent in the group B patients (relative risk: 1.96, p=0.004). The finding underscored the importance of considering comorbidities in assessing the risk of complications following radical nephrectomy.

Laparoscopic Retrograde Nephrectomy as a Troubleshooting Technique to Prevent Open Conversion: The Technique Description With a Review of 40 Cases.

Introduction Laparoscopic nephrectomies are safe, with low complication rates in skilled hands. However, traditional approaches may be unsuitable for conditions such as post-renal abscesses, long-standing urinomas, non-functioning kidneys post-pyeloplasty, pyelolithotomies, post-partial nephrectomy recurrences, tuberculous kidneys, pyelonephritis, and redo-renal surgeries. This study describes a modified retrograde nephrectomy technique and its outcomes in 40 cases. Methods We reviewed 40 cases where the retrograde nephrectomy technique was used. Surgeons opted for this method based on intraoperative findings and initial difficulties in accessing the lower pole area. Results Traditional dissection was challenging due to adhesions in the lower pole. The retrograde technique, starting from the renal hilum, allowed early ligation of renal arteries and veins, reducing bleeding risks and facilitating safer caudal dissection. Conclusions The retrograde nephrectomy technique offers a safer and more efficient alternative for complex nephrectomies. Early vascular control minimizes hemorrhage risk, making it a valuable method in challenging renal surgeries.

Renal Endometriosis Mimics Renal Cell Carcinoma in a Hypoplastic Kidney: A Case Report.

Renal endometriosis is a rare disorder of cases of urinary tract endometriosis. A 42-year-old woman presented at our outpatient department with an incidental painless mass on her left hypoplastic kidney revealed on an abdominal ultrasound. Abdominal and pelvic examinations revealed no abnormal findings. A computed tomography (CT) scan revealed an anterolateral slightly enhanced left renal mass that measured 1.2 cm in diameter. Furthermore, CT did not reveal any evidence of abdominal or thoracic metastasis. There are a few case reports in the literature of tumors in specimens from patients who underwent nephrectomy for hypoplastic kidneys, but discriminating between benign and malignant masses is difficult unless a nephrectomy is performed. Given the radiological findings and the impaired function of the hypoplastic kidney, laparoscopic radical nephrectomy was recommended. The procedure was performed under general anesthesia without intraoperative or postoperative complications. Microscopic examination revealed several findings consistent with a diagnosis of renal endometriosis. The patient had no symptoms at her last follow-up visit. This case highlights that renal endometriosis can mimic renal cell carcinoma and awareness of this entity should be raised, as it can be asymptomatic, especially when located in a hypoplastic kidney.

Cutting-Edge Strategies for Renal Tumour-like Lesions in Granulomatosis with Polyangiitis: A Systematic Review.

BACKGROUND: Granulomatosis with polyangiitis (GPA) is characterised by granulomatous inflammation and small-to-medium vessel necrotising vasculitis, mainly affecting respiratory tract and kidneys. Renal involvement presenting as tumour-like lesions poses diagnostic and treatment challenges. METHODS: Following the observation of a GPA patient presenting with multiple renal tumour-like lesions, we conducted a systematic literature review on MEDLINE/PubMed, EMBASE, and Cochrane databases. Data gathered from the literature were analysed to summarise the diagnostic approach, management, and outcome of renal GPA-related tumour-like lesions. RESULTS: a 49-year-old female presented with persistent constitutional symptoms and multiple bilateral renal lesions. Renal biopsy showed chronic interstitial inflammation with necrotising granulomas. Laboratory tests disclosed positive anti-proteinase 3 (PR3) anti-neutrophil cytoplasmic antibody (ANCA) leading to a final diagnosis of GPA. She was effectively treated with high-dose glucocorticoids and rituximab. Literature search yielded 41 articles, concerning 42 GPA patients with renal masses, presenting bilaterally in 23.8% of the cases. Positive PR3-ANCA was observed in 86.5% of the cases. Half of 42 patients showed kidney abnormalities. Treatment with glucocorticoids (83.3%) and immunosuppressive agents (80.9%) resulted in an overall good remission rate and favourable prognosis. CONCLUSIONS: GPA should be considered in the differential diagnoses of kidney tumour-like lesions. The diagnosis is challenging, and histological examination greatly contributes to the diagnostic work-up.

Off-clamp vs on-clamp robot-assisted partial nephrectomy: a systematic review and meta-analysis.

OBJECTIVE: To compare intra- and postoperative outcomes between off-clamp and on-clamp robot-assisted partial nephrectomy (RAPN), using data from randomised controlled trials (RCTs) or covariate-matched studies (propensity score-matched or matched-pair analysis). METHODS: A Preferred Reporting Items for Systematic Reviews and Meta-Analyses-compliant literature review was conducted on PubMed, EMBASE, Scopus and CENTRAL for relevant studies comparing off-clamp to on-clamp RAPN. Primary outcomes were estimated blood loss, postoperative percentage decrease in estimated glomerular filtration rate (eGFR), and margin positive rate. Secondary outcomes were operative time, postoperative eGFR, length of stay, all postoperative complications, major complications, and need for transfusion. Random-effects meta-analyses were performed to generate mean differences (MDs) or odds ratios (ORs). RESULTS: A total of 10 studies (2307 patients) were shortlisted for analysis. There was no significant difference in estimated operative blood loss between off-clamp and on-clamp RAPN (MD 21.9 mL, 95% confidence interval [CI] -0.9 to 44.7 mL; P = 0.06, I2 = 58%). Off-clamp RAPN yielded a smaller postoperative eGFR deterioration (MD 3.10%, 95% CI 1.05-5.16%; P = 0.008, I2 = 13%) and lower odds of margin positivity (OR 0.62, 95% CI 0.40-0.94; P = 0.03, I2 = 0%). No significant differences were found for all secondary outcomes. CONCLUSIONS: Off-clamp and on-clamp RAPN are similarly effective approaches for selected renal masses. Within the classic trifecta of PN outcomes, off-clamp RAPN yields similar rates of perioperative complications and may possibly offer better preservation of renal function and reduced margin-positive rates.

Post-operative flank irradiation using conformal versus highly conformal radiotherapy techniques for paediatric renal tumours: Results from the French registry PediaRT.

PURPOSE: Three-dimensional conformal RT (3D-RT) techniques are gold standard for post-operative flank radiotherapy (RT) in paediatric renal tumours. Recently, highly conformal RT (HC-RT) techniques have been implemented without comparative clinical data. The main objective of this multicentre study was to compare locoregional control (LRC) in children treated either with HC-RT or 3D-RT techniques. METHODS: Patients treated with post-operative flank RT for renal tumour registered in the national cohort PediaRT between March 2013 and September 2019 were included. Treatment and follow-up data, including toxicities and outcomes, were retrieved from the database. LRC was calculated, and dose reconstruction was performed in case of an event. RESULTS: Seventy-nine patients were included. Forty patients were treated with HC-RT and 39 with 3D-RT. Median follow-up was 4.5 years. Three patients had locoregional failure (LRF; 4%). HC-RT was not associated with a higher risk of LRF. Three-year LRC were 97.4% and 94.7% in the HC-RT and 3D-RT groups, respectively. The proportion of planning target volumes receiving 95% or more of the prescribed dose did not significantly differ between both groups (HC-RT 88%; 3D-RT 69%; p = .05). HC-RT was better achieving dose constraints, and a significant mean dose reduction was observed in the peritoneal cavity and pancreas associated with lower incidence of acute gastrointestinal toxicity. CONCLUSION: LRF after post-operative flank RT for renal tumours was rare and did not increase using HC-RT versus 3D-RT techniques. Dose to the pancreas and the peritoneal cavity, as well as acute toxicity, were reduced with HC-RT compared to 3D-RT.

Renal Tumours Of Non-Clear Cell Histology; 10 Years' Experience In A Specialized Centre.

BACKGROUND: Non-clear cell renal cell carcinomas are uncommon renal tumours with diverse histologically and genetically defined entities. Due to limited clinical outcomes data, no standardized management approach can be offered to these patients. This study aimed to analyse outcomes of non clear cell renal cell carcinoma after surgical resection of localized renal tumours in our population. METHODS: Patients with renal tumours who underwent partial or radical nephrectomy at the Department of Urology, from January 2010 to December 2019 were identified and evaluated, in terms of prevalence, presentation, recurrence, and survival outcome. RESULTS: Non-clear cell tumours were found in one-fourth of the total number of nephrectomies performed during this period for renal cell carcinoma (RCC). The mean age was 50.48±14.76 years (range 18-89 years) with 57% being of the male gender. The predominant types were chromophobe RCC, papillary RCC, and sarcomatoid RCC, in all non-clear cell renal tumours. Mean Recurrence Free Survival (RFS) for all tumours was 75.26±2.7 months. The projected 5 years RFS of papillary RCC, chromophobe RCC and sarcomatoid RCC were 94.2%, 84.3% and 62.5% respectively. CONCLUSIONS: RCC of non-clear-cell histology depicts excellent survival in patients with localized renal tumours. Furthermore, sarcomatoid RCC has worse recurrence free survival followed by chromophobe RCC and papillary RCC, in our population subset.

Comparative analyses define differences between BHD-associated renal tumour and sporadic chromophobe renal cell carcinoma.

BACKGROUND: Birt-Hogg-Dubé (BHD) syndrome, caused by germline alteration of folliculin (FLCN) gene, develops hybrid oncocytic/chromophobe tumour (HOCT) and chromophobe renal cell carcinoma (ChRCC), whereas sporadic ChRCC does not harbor FLCN alteration. To date, molecular characteristics of these similar histological types of tumours have been incompletely elucidated. METHODS: To elucidate renal tumourigenesis of BHD-associated renal tumours and sporadic renal tumours, we conducted whole genome sequencing (WGS) and RNA-sequencing (RNA-seq) of sixteen BHD-associated renal tumours from nine unrelated BHD patients, twenty-one sporadic ChRCCs and seven sporadic oncocytomas. We then compared somatic mutation profiles with FLCN variants and RNA expression profiles between BHD-associated renal tumours and sporadic renal tumours. FINDINGS: RNA-seq analysis revealed that BHD-associated renal tumours and sporadic renal tumours have totally different expression profiles. Sporadic ChRCCs were clustered into two distinct clusters characterized by L1CAM and FOXI1 expressions, molecular markers for renal tubule subclasses. Increased mitochondrial DNA (mtDNA) copy number with fewer variants was observed in BHD-associated renal tumours compared to sporadic ChRCCs. Cell-of-origin analysis using WGS data demonstrated that BHD-associated renal tumours and sporadic ChRCCs may arise from different cells of origin and second hit FLCN alterations may occur in early third decade of life in BHD patients. INTERPRETATION: These data further our understanding of renal tumourigenesis of these two different types of renal tumours with similar histology. FUNDING: This study was supported by JSPS KAKENHI Grants, RIKEN internal grant, and the Intramural Research Program of the National Institutes of Health (NIH), National Cancer Institute (NCI), Center for Cancer Research.