No abstract

Use of EPU stents provided a viable alternative, particularly in younger patients, to DJ stenting with comparable success and complications, while avoiding the need for an additional anesthetic.

ESWL use has come under scrutiny with a shift in focus to cost-effectiveness and healthcare outcomes. Fortunately, advances in lithotripter technology have spawned several generations of devices that strive to improve stone-free rates and decrease complications. Most of all, a focus on patient selection criteria has helped improve procedural success. Years of experience utilizing ESWL for stone treatment have helped urologists better optimize its use and minimize complications. Improvements in technique along with more stringent patient and stone selection have helped ESWL remain a mainstay in the treatment of stone disease.

Laparoscopic herniorrhaphy using a peritoneal leaflet closure technique is safe and effective when used in infants and children to close an indirect hernia (i.e. patent processus vaginalis). No intraoperative complications occurred in this cohort and success rate was 97%.

Kidney epithelial targeted mitochondrial transcription factor A deficiency results in progressive mitochondrial depletion associated with severe cystic disease.

Historically, T(6;11) renal cell carcinoma (RCC) has been associated with the pediatric and adolescent populations and documentation of this tumor in adults has been rare. However, the frequency of translocation renal cell carcinoma (TRCC) may be widely underestimated in the adult population due to an inadequate immunohistochemical workup or misdiagnosis from similar gross and histological findings to other RCC. A subset of MiT family translocation carcinomas, t(6:11) (p21;q12) translocation tumors cause an alpha-TFEB gene fusion. Morphologically, this neoplasm tends to mimic the various types of RCC's, including clear cell, papillary, and even epitheloid angiomyolipomas. Adult cases of TRCC have shown to behave more aggressively than their indolent pediatric counterpart, but due to the limited number of reported cases the true nature of these tumors has yet to be determined. The aim of this review is to bring an awareness of translocation RCC to better understand its diagnoses, treatment and prognosis, and, in turn, to allow for new cases to further highlight the behavior of this rare variant.

Background: Staghorn calculi are well-established risk factors for recurrent urinary tract infections (UTIs) and subsequent renal deterioration. Less commonly, long-term urothelial irritation from a calculus may also pose a risk of malignant transformation.Case Presentation: A 77-year-old male with multiple medical comorbidities presented with a chronic right renal pelvic staghorn calculus and findings concerning for emphysematous pyelonephritis. He was subsequently taken to the operating room for a planned laparoscopic right nephrectomy. Final pathology analysis revealed sarcomatoid squamous cell carcinoma (SCC) of the renal pelvis with superimposed pyelonephritis and renal abscesses. Preoperative imaging was not suggestive of malignancy.Conclusion: Although SCC of the urothelium can be caused by chronic irritation, its presentation is usually isolated to the lower urinary tract and is rarely encountered in the renal pelvis. Our patient's presentation with sarcomatoid SCC is an even rarer entity. Chronic staghorn calculi must be considered as a potential risk factor for the development of both UTI and malignant urothelial transformation.

Beckwith-Wiedemann Syndrome (BWS) is an overgrowth disorder with various congenital anomalies. Although the most classic constellation includes macrosomia, macroglossia, and omphalocele, nephrourological findings are commonly associated with BWS. Clinical presentation is highly variable because of its complex molecular heterogeneity, which involves changes in DNA methylation and disruption of growth regulatory genes. We report 3 pediatric patients, ages 13 months to 3 years old, who presented with clinical features consistent with BWS. A variety of nephrourological abnormalities were also noted, including posterior urethral valves, hydroureteronephrosis, and undescended testes. Genetic testing for all 3 patients revealed duplication of the region chromosome 11p15.5.