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Alexander Combes,1 Simon McQueen,2 Catalina Alejandra Palma,1 David Benz,2 Scott Leslie,1,3– 5 Paul Sved,1,5 John Boulas,1,5 Arthur Vasilaras,1,5 Chris Rogan,2,5 Ilias Drivas,2 David Robert Eisinger,1 Richard Waugh2 1Department of Urology, Royal Prince Alfred Hospital, Camperdown, NSW, 2050, Australia; 2Department of Radiology, Royal Prince Alfred Hospital, Camperdown, NSW, 2050, Australia; 3RPA Institute of Academic Surgery, Sydney, NSW, Australia; 4Faculty of Medicine, University of Sydney, Sydney, NSW, Australia; 5Department of Urology, Chris O’Brien Lifehouse, Sydney, NSW, AustraliaCorrespondence: Alexander Combes, Department of Urology, Royal Prince Alfred Hospital, 50 Missenden Road, Camperdown, NSW, 2050, Australia, Tel +612 9515 7773, Fax +612 9515 7774, Email combes_08@hotmail.comPurpose: Renal angiomyolipoma (AML) is the most common benign renal tumor. Whilst generally asymptomatic, they can cause life-threatening bleeding. Selective angioembolization (SAE) may be used to treat large symptomatic and asymptomatic AMLs. We aimed to evaluate the efficacy of SAE for symptomatic and asymptomatic renal AMLs and determine characteristics that predict spontaneous bleeding.Patients and Methods: Data were retrospectively collected from a prospectively maintained database from July 2011 to April 2022. Patients were included if AML was > 4cm and they underwent subsequent SAE. Follow-up imaging was analyzed to calculate mean reduction in AML size. Clinical notes were reviewed to analyze lesion characteristics including vascularity, fat content and presence of aneurysm as well as post-procedural complications.Results: 26 patients with 30 AMLs were identified. Interval of follow-up imaging ranged from 1 to 60 months. 25 AMLs were embolized electively with 5 emergency embolizations performed for bleeding. Mean reduction in AML volume was 41% at 3 months (p=0.013) and 63% at 12 months (p=0.007). All 5 bleeding AMLs had a rich vascularity with 60% also having either aneurysms or a low fat content. Complications included post-embolic syndrome (n=9), segmental renal parenchyma devascularization (n=3), acute bleeding requiring re-embolization (n=2), nephrectomy for ongoing bleeding (n=1) and delayed bleeding managed conservatively (n=1). No deterioration in renal function was observed.Conclusion: SAE is an effective procedure for managing symptomatic and asymptomatic renal AML, with minimal significant complications. AML vascularity, fat content and aneurysms may be useful characteristics to assess future risk of bleeding in patients with renal AML.Keywords: selective angioembolization, aneurysm, fat content, tuberosclerosis complex

Background Total cystectomy and subsequent reconstruction of the urinary tract may be required for primary malignancy of the bladder, or in the context of multi-visceral resection for more advanced pelvic tumors. Complications following urinary diversion are a major source of morbidity, particularly in pelvic exenteration (PE) patients. Methods All patients who underwent radical cystectomy alone or during PE at a single tertiary referral centre between 2008 and 2014 were reviewed. Postoperative urological complications were collected and compared between groups. Results Two hundred and thirty-one patients underwent en bloc cystectomy (98 cystectomy alone, 133 as part of a PE). Postoperative urological complications occurred in 33% of the cystectomy alone group and 59% of the PE group (P

Objectives To assess the postoperative complication rate and overall survival when bovine pericardium is used as graft material for inferior vena cava (IVC) reconstructions in patients with renal cell carcinoma (RCC). The ideal graft material is yet to be established, with synthetic grafts widely studied and used in the current literature. Methods We performed a retrospective cohort analysis of consecutive patients who underwent IVC reconstructions as part of resection for RCC, using bovine pericardium as either a patch repair or tubular interposition graft. Results A total of 15 patients underwent resection with IVC reconstruction between 2010 and 2018. Nine patients had tubular interposition grafts and 6 had patch repairs. Three patients had Clavien-Dindo grade 3 or higher short-term complications. There was no difference in Comprehensive Complications Index between those who had interposition grafts and patch repairs. Two patients had a long-term graft-associated thrombus requiring temporary anticoagulation. Overall survival was 46.5 months (95% confidence interval [CI] 36.9-56.1). There were no perioperative deaths. All long-term deaths were due to disease progression. Conclusion Reconstruction of the IVC with a bovine pericardium graft is safe in experienced centers. Bovine pericardium could be considered as the material of choice, given its safety in the immediate postoperative period, ease of use, and patency without routine long-term anticoagulation. Advanced surgical management leads to good overall survival in this cohort with high tumor burden.

A custom CGH microarray that covers the SOX9 regulatory region. Log2 ratio scatterplot showing individual data points. Blue box highlights copy number gain with 3' breakpoint region magnified.

Summary 46,XX disorders of sexual development (DSDs) occur rarely and result from disruptions of the genetic pathways underlying gonadal development and differentiation. We present a case of a young phenotypic male with 46,XX SRY-negative ovotesticular DSD resulting from a duplication upstream of SOX9 presenting with a painful testicular mass resulting from ovulation into an ovotestis. We present a literature review of ovulation in phenotypic men and discuss the role of SRY and SOX9 in testicular development, including the role of SOX9 upstream enhancer region duplication in female-to-male sex reversal. Learning points: In mammals, the early gonad is bipotent and can differentiate into either a testis or an ovary. SRY is the master switch in testis determination, responsible for differentiation of the bipotent gonad into testis. SRY activates SOX9 gene, SOX9 as a transcription factor is the second major gene involved in male sex determination. SOX9 drives the proliferation of Sertoli cells and activates AMH/MIS repressing the ovary. SOX9 is sufficient to induce testis formation and can substitute for SRY function. Assessing karyotype and then determination of the presence or absence of Mullerian structures are necessary serial investigations in any case of DSD, except for mixed gonadal dysgenesis identified by karyotype alone. Treatment is ideal in a multidisciplinary setting with considerations to genetic (implications to family and reproductive recurrence risk), psychological aspects (sensitive individualized counseling including patient gender identity and preference), endocrinological (hormone replacement), surgical (cosmetic, prophylactic gonadectomy) fertility preservation and reproductive opportunities and metabolic health (cardiovascular and bones).

46,XX disorders of sexual development (DSDs) occur rarely and result from disruptions of the genetic pathways underlying gonadal development and differentiation. We present a case of a young phenotypic male with 46,XX SRY-negative ovotesticular DSD resulting from a duplication upstream of SOX9 presenting with a painful testicular mass resulting from ovulation into an ovotestis. We present a literature review of ovulation in phenotypic men and discuss the role of SRY and SOX9 in testicular development, including the role of SOX9 upstream enhancer region duplication in female-to-male sex reversal. Learning points: In mammals, the early gonad is bipotent and can differentiate into either a testis or an ovary. SRY is the master switch in testis determination, responsible for differentiation of the bipotent gonad into testis. SRY activates SOX9 gene, SOX9 as a transcription factor is the second major gene involved in male sex determination. SOX9 drives the proliferation of Sertoli cells and activates AMH/MIS repressing the ovary. SOX9 is sufficient to induce testis formation and can substitute for SRY function. Assessing karyotype and then determination of the presence or absence of Mullerian structures are necessary serial investigations in any case of DSD, except for mixed gonadal dysgenesis identified by karyotype alone. Treatment is ideal in a multidisciplinary setting with considerations to genetic (implications to family and reproductive recurrence risk), psychological aspects (sensitive individualized counseling including patient gender identity and preference), endocrinological (hormone replacement), surgical (cosmetic, prophylactic gonadectomy) fertility preservation and reproductive opportunities and metabolic health (cardiovascular and bones).

Background Urine leak following pelvic exenteration for locally advanced pelvic malignancy is a major complication leading to increased mortality, morbidity and length of stay. We reviewed our experience and developed a diagnostic and management algorithm for urine leaks in this patient population. Methods Consecutive patients who underwent en bloc cystectomy and conduit formation as part of pelvic exenteration at a single quaternary referral centre from 1995 to 2012 were reviewed. Patients with urine leak were identified. Medical records were reviewed to extract data on diagnosis and management and a suggested clinical algorithm was developed. Results Of 325 exenterations, there were 102 conduits, of which 15 patients (15%) developed a conduit related urine leak. Most (14/15) patients were symptomatic. Diagnosis was made by drain creatinine studies (12/15) and/or imaging (15/15). Management comprised of conservative management, radiologic urinary diversion, early surgical revision and late surgical revision in 3, 11, 2 and 1 patients respectively. Important lessons from our 17 year experience include a high index of suspicion in a patient who is persistently septic despite appropriate treatment, the importance of regular drain creatinine studies, CT (computer tomography) with delayed images (CT intravenous pyelogram) when performing a CT for investigation of sepsis and early aggressive management with radiologic urinary diversion to facilitate early healing. Conclusion Urine leak after pelvic exenteration is a complex problem. Conservative management usually fails and early diagnosis and intervention is the key. It is hoped that our algorithms will facilitate diagnosis and subsequent management of this group of patients.