Syed Adeeb ul Hassan Rizvi

Primitive neuroectodermal tumor/Ewing's sarcoma in adult uro-oncology: A case series from a developing country

Peripheral primitive neuroectodermal tumor/Ewings sarcoma (PNET/EWS) is primarily a tumor of soft tissues and bones. Primary localization of PNET/EWS in genitourinary organs is rare. No data on this localization of PNET/EWS are available in literature from Pakistan. We searched our adult uro-oncology records from 1994 till date and identified all cases of adult genitourinary and adrenal PNET/EWS diagnosed on histology and immunohistochemistry. Their case records were reviewed to obtain data on demographics, presentation, pathologic features, management and outcome. Six cases were found; all were young and had aggressive disease at presentation. Four had renal PNET/EWS. One case each of prostate and adrenal PNET/EWS was seen. Surgery and chemotherapy formed the mainstay of management. Three patients (50%) died during treatment, two were lost to follow-up and one case with renal PNET/EWS showed good initial response to chemotherapy but was later on lost to follow-up. In conclusion, PNET/EWS should be considered in the differential diagnosis of genitourinary malignant tumors in young patients. These tumors are aggressive with poor outcome.

Open surgical management of pediatric urolithiasis: A developing country perspective.

Objectives To describe decision factors and outcome of open surgical procedures in the management of children with stone. Materials and Methods Between January 2004 and December 2008, 3969 surgical procedures were performed in 3053 children with stone disease. Procedures employed included minimally invasive techniques shockwave lithotripsy (SWL), percutaneous nephrolithotomy (PCNL), ureterorenoscopy (URS), perurethral cystolithotripsy (PUCL), percutaneous cystolithotripsy (PCCL), and open surgery. From sociomedical records demographics, clinical history, operative procedures, complications, and outcome were recorded for all patients. Results Of 3969 surgeries, 2794 (70%) were minimally invasive surgery (MIS) techniques to include SWL 19%, PCNL 16%, URS 18.9%, and PUCL+PCCL 16% and 1175 (30%) were open surgeries. The main factors necessitating open surgery were large stone burden 37%, anatomical abnormalities 16%, stones with renal failure 34%, gross hydronephrosis with thin cortex 58%, urinary tract infection (UTI) 25%, and failed MIS 18%. Nearly 50% of the surgeries were necessitated by economic constraints and long distance from center where one-time treatment was preferred by the patient. Stone-free rates by open surgeries were pyelolithotomy 91%, ureterolithotomy 100%, and cystolithotomy 100% with complication rate of upto 3%. Conclusions In developing countries, large stone burden, neglected stones with renal failure, paucity of urological facilities, residence of poor patients away from tertiary centers necessitate open surgical procedures as the therapy of choice in about 1/3rd of the patients. Open surgery provides comparable success rates to MIS although the burden and nature of disease is more complex. The scope of open surgery will remain much wide for a large population for considered time in developing countries.

Plasma cell‐rich acute rejections in living‐related kidney transplantation: a clinicopathological study of 50 cases

Acute rejections (ARs) with plasma cell‐rich infiltrates (PCARs) are associated with poor outcomes.

Renal transplant in a tuberous sclerosis patient with bilateral giant renal angiomyolipomas and concurrent renal carcinoma

Co-existence of angiomyolipoma (AML) and renal cell carcinoma (RCC) in the same tumor mass is very rare and only eight cases have been reported. We present a case of a young female with tuberous sclerosis complex (TSC) with bilateral huge renal AMLs. Both tumors were removed, one of which revealed co-incidental RCC. She was subsequently successfully transplanted a kidney from her brother and is maintaining normal graft function eight months post-transplant. No recurrence or metastases of RCC has been detected till the last follow-up.

Renal Transplantation in Patients with Lower Urinary Tract Dysfunction: A Single Center Experience from a Developing Country

Objectives: Historically, patients with lower urinary tract dysfunction (LUTD) were considered poor candidates for renal transplantation (RT). We aimed to review our experience with this procedure for its safety and efficacy. Methods: We reviewed the case records of patients with LUTD who underwent RT at our center. Graft and patient survival were analyzed. Results: Out of 2053 RTs, 26 (1.2%) patients had LUTD as the primary cause of end-stage renal disease (ESRD). All patients underwent cystourethroscopy prior to transplantation, had abnormal bladders and all underwent bladder augmentation. Only 16 (61.5%) patients had urodynamic (UDN) evaluation prior to transplantation. Pretransplantation augmentation cystoplasty (AC) was performed in 24 (92.3%) patients, and post-RT in two (7.7%). Mitrofanoff channel was made in 25 (96.1%) patients using appendix in 14 (56%) patients and native ureter in 11 (44%). Double-J (DJ) stents were placed in all patients peroperatively. All patients developed 156 episodes of urinary tract infections (UTIs), with an average of 6 UTIs/ patient. All patients except three are maintaining their graft function within acceptable limits. We observed 100% patient and graft survival rates in this series. Conclusions: In conclusion, RT combined with AC is a feasible option for patients with LUTD with good results in the medium term and should be explored in selected patients.

Renal Transplantation in Low Resource Settings

Summary In low income settings rates of renal replacement therapy (RRT) range from 50%) in living related and Tacrolimus + Mycophenolate Mofetil (>50%) in deceased donor transplants. Graft and patient survival at 1 and 5 years are 83%–98%, 59%–90%, 90%–98%, and 85%–96%, respectively. Causes of graft and patient loss are interstitial fibrosis and tubular atrophy (up to 71%) and infections (up to 80%), respectively. In low resource settings it is important, when possible, for the government sector to establish freely accessible renal transplantation for all who need it where community becomes a part of the care of ESRD patients.