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PURPOSE: We determined the oncologic and urological outcomes in patients with bladder/prostate rhabdomyosarcoma according to the type and timing of urinary tract surgery, with emphasis on the role of the Padua orthotopic ileal neobladder. MATERIALS AND METHODS: We retrospectively analyzed oncologic and urological outcomes of 11 consecutive patients treated at our institution between 1998 and 2012. RESULTS: Two patients underwent urethrectomy and placement of a heterotopic catheterizable ileal neobladder. The membranous urethra was preserved in 9 patients, 6 underwent primary Padua ileal neobladder at radical cystectomy, 2 underwent delayed Padua ileal neobladder and 1 underwent bilateral cutaneous ureterostomy. Four of these 9 patients experienced disease recurrence, including local recurrence in 2 despite negative intraoperative biopsies. Survivors undergoing heterotopic catheterizable ileal neobladder or primary Padua ileal neobladder learned to empty the bladder to completion without long-term upper tract deterioration. Both cases managed by delayed Padua ileal neobladder required clean intermittent catheterization eventually. Erections were reported in 5 of 6 surviving males. CONCLUSIONS: The Padua ileal neobladder allowed preservation of volitional urethral voiding in all survivors in whom it was placed at radical cystectomy. Nevertheless, local recurrence was noted in 2 of the 9 cases where the membranous urethra was preserved. By comparison, patients undergoing delayed Padua ileal neobladder after attaining disease-free status never achieved voiding per urethra. Therefore, a heterotopic reservoir might be a more reliable choice under these circumstances. Erectile function is preserved in the majority of cases.

We report short and long-term donor site outcomes after oral mucosa graft harvesting for urological reconstruction in a large series of patients including children, and identify possible risk factors for an untoward long-term outcome.A total of 78 patients were evaluated. Short-term outcomes included time to restore normal oral diet, perioral sensory defect/discomfort and jaw opening impairment occurring within 4 weeks of surgery. Long-term outcomes included donor site scarring, perioral sensory defect and jaw opening impairment occurring more than 1 year postoperatively. Long-term outcomes were assessed via a questionnaire administered to patients and on clinical examination by an oral surgeon. Outcomes were compared in children (younger than 12 years at surgery) and adults, and with regard to harvesting site, graft length, length of followup and other variables.Two-thirds of the patients returned to a normal oral diet within 3 days postoperatively (range 1 to 8). All patients complained of perioral sensory defect/discomfort postoperatively, and 26% had jaw opening impairment. After a median followup of 7.6 years (range 1 to 13.2) perioral sensory defect was the most common complication observed (28%) in cases formally evaluated by an oral surgeon. The sensory defect was seldom perceived by the patients and never required treatment. It was statistically more common in patients undergoing surgery as adults, whereas none of the other variables proved significant.Oral mucosa graft harvesting is safe irrespective of age. About a quarter of patients, more commonly adults, will have a long-term perioral sensory defect. However, the defect is never perceived as bothersome.

We review our experience with renal manifestations in pediatric patients with the tuberous sclerosis complex, and offer recommendations for urological counseling, followup and treatment of these patients.We reviewed clinical notes on 41 patients with the tuberous sclerosis complex followed at our institution from childhood. Patient data were gathered in a database focusing on renal involvement. The latter was assessed by periodic clinical evaluations and ultrasound. The risk of renal involvement was evaluated in relation to patient age, genotypic pattern and number of extrarenal manifestations.Overall, 15 patients (36.6%) had renal involvement. The latter increased with age and was more common in cases with TSC2 genotypic pattern or multiple extrarenal manifestations. Angiomyolipomas were the most common lesions (11 patients), followed by renal cysts (2) and polycystic kidney disease (2). Cystic lesions were the most common in patients younger than 16 years. Renal failure developed in the 2 patients with polycystic kidney disease by the 2nd decade of life. Overall, treatment was required in 2 cases of symptomatic angiomyolipoma. Both patients were female, and had multiple extrarenal manifestations and bilateral renal involvement. One patient underwent open surgery at age 21.3 years and 1 underwent radiological embolization at age 23.4 years.Pediatric patients with the tuberous sclerosis complex should undergo urological evaluation and followup. Although most of the lesions remain silent during childhood, the incidence of renal involvement increases with age. The need for treatment is highest in females with multiple extrarenal manifestations and bilateral renal involvement.