Pediatric renal cell carcinoma: single institution 25-year case series and initial experience with partial nephrectomy.

PURPOSE: RCC represents less than 2% to 6% of pediatric renal tumors. Few reports of long-term outcomes exist. We sought to determine the presentation, treatment and outcome of patients at our institution. MATERIALS AND METHODS: We retrospectively reviewed the age, mode of presentation, mode of treatment, histological subtype, tumor grade, stage and survival of all patients with RCC from 1980 to 2005. RESULTS: A total of 15 patients were identified. Mean age at presentation was 7.9 years. Symptomatic presentations in nearly 75% of patients included gross hematuria, abdominal pain and polycythemia. The remaining 25% of cases were asymptomatic, and were identified by physical examination or incidentally on imaging. Surgical resection consisted of radical nephrectomy in 10 patients and partial nephrectomy in 5. Pathological analysis revealed papillary RCC in 8 patients and clear cell RCC in 7. Six patients had high stage disease. One patient with stage IV disease died 8 months postoperatively. The remaining 14 patients were alive at a mean followup of 4.9 years. All but 1 patient remain recurrence-free, including all of those who underwent nephron sparing surgery. CONCLUSIONS: To our knowledge this single institution series is the first to include children treated with partial nephrectomy. Pediatric patients with RCC tend to be older and more likely to present symptomatically compared to the typical patient with Wilms tumor. Hematuria and abdominal pain were the most common presentations, and papillary RCC was proportionately more common in this series. Our initial experience suggests that equivalent cure rates can be expected from a nephron sparing approach in appropriately selected cases.

Porcine bladder acellular matrix porosity: impact of hyaluronic acid and lyophilization.

Bladder acellular matrix (ACM) is being investigated as a urinary bladder replacement scaffold. We have demonstrated that ACM is porous and theorized that this contributes to ACM fibrosis and contracture over time in vivo and may preclude uptake and retention of molecules, which may aid cellular repopulation. We sought to determine if hyaluronic acid (HA) would decrease ACM porosity. Porcine ACM was lyophilized and rehydrated in HA (SIGMA) to form the hybrid HA-ACM construct. Three groups (n = 15/group: HA-ACM, ACM, and lyophilized/rehydrated ACM) were tested for porosity to a 10 cm column of distilled water, measuring the effluent hourly for 3 h. A porosity index was determined as the total effluent divided by time and area (cc/cm2 hr). Alcian blue staining and fluorophore-assisted carbohydrate electrophoresis qualitatively and quantitatively confirmed the uptake of HA. HA-ACM and lyophilized/rehydrated ACM were significantly less porous to water than untreated ACM [mean (+/-SE): 0.09 (+/-0.02), 0.74 (+/-0.4), and 9.8 (+/-1.6) cc/cm2 hr, respectively; Mann Whitney p < 0.0001 (HA) and p < 0.0001 (lyo)]. The difference between HA-ACM and lyophilized ACM was also statistically significant (p = 0.014). ACM hybridization with HA decreases ACM porosity, in part because of ACM lyophilization during the hybridization process. In future applications, HA may function as a carrier for smaller molecules such as growth factors, and as a bioactive molecule to improve wound healing and decrease fibrosis in tissue-engineered bladder constructs.

Simplified mons plasty: a new technique to improve cosmesis in females with the exstrophy-epispadias complex.

PURPOSE: Long-term data suggest that a significant number of women with the exstrophy-epispadias complex (EEC) report dissatisfaction with the cosmetic appearance of the genitalia. We developed a new technique of mons plasty in an attempt to improve the overall cosmetic appearance of the external genitalia in females with EEC. MATERIALS AND METHODS: Nine consecutive females with a mean age of 6.4 years (range 2 days to 12.2 years) previously diagnosed with EEC underwent simplified mons plasty concomitantly with various other urological reconstructive procedures. Briefly, a simplified mons plasty was performed by approximating the bifid hemiclitoris and subsequently incising the lateral sulci between the labia majora and minora, which allowed the superomedial rotation of the labia majora and underlying peripubic adipose tissue, thus, recreating the mons. RESULTS: There were no perioperative complications. With a mean followup of 6 months (range 4 to 12) all patients had improved cosmesis with respect to a more prominent mons, more appropriately situated labia and adequately recessed introitus. CONCLUSIONS: Simplified mons plasty improves cosmesis in females with external genital abnormalities secondary to EEC. Although our followup is admittedly short, we believe that this technique should be considered in the armamentarium of the reconstructive surgeon during the operative treatment of this complex group of patients.