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OBJECTIVE To describe and compare differences in perception of independence, urinary continence, and quality of life in an adult spina bifida (SB) population. METHODS We collected data on adult neurogenic bladder patients which included demographics, relevant procedures, and quality of life (QoL) questionnaires. QoL and functional outcomes were assessed using spinal cord independence measure (SCIM) and SF-8 health questionnaire. International consultation of incontinence questionnaire (ICIQ) was used to assess incontinence. Comparisons were drawn between patients who underwent surgical reconstruction and those who did not. Student t-tests were used for comparisons and a P-value

Primary spindle cell sarcoma of the prostate is exceedingly rare and accounts for 0.1% of all prostatic cancers. Typically, the disease course is rapid and portends a short and dismal prognosis. We report a case of prostatic stromal sarcoma (PSS) which likely lay dormant for several years after a transurethral resection of the prostate. This case is unique in that this cancer did not display the rapid growth of PSS in prior reports. Our patient received a cystoprostatectomy and an ileal conduit. This article describes a rare presentation of a rare tumor and a brief review of the literature.

Background Adolescents with neurogenic bladder are a vulnerable population that severely lacks consistent transitional care from pediatric to adult urology settings. Aims Our practice determined that 100 patients with spina bifida and other neurogenic bladder conditions were not appropriately transferred to the adult setting once reaching adulthood. Methods We initiated a transitional program to establish a dedicated and formal process for adolescent patients to transition to adult urology. The REACH clinic implements a formalized staging framework to facilitate migration of adolescents and young adults to the adult health setting. A social worker was incorporated to act as a patient advocate, behavioral health consultant, and resource specialist. Results To date 45 patients have been enrolled in the transition program. We have identified and categorized according to the appropriate stage. The REACH clinic has appropriately outlined the goals and mission of the program and resources utilized are financially practical and feasible by conducting a monthly combined clinic. The program has been instrumental in improving tracking and monitoring of these patients through their transition period. Conclusion Through the efforts of the pediatric and adult urology teams, the REACH program is a dedicated framework that provides structure for transition of the adolescent patient. The addition of a social worker has resulted in enriched rapport and will likely result in improved compliance. This program allows for surveillance and evaluation of patient outcome indicators in this patient population. We believe that early introduction and frequent encounters with the adult urologic team is crucial to successful transitions. Neurourol. Urodynam. © 2016 Wiley Periodicals, Inc.

Patients with congenital urologic conditions require complex chronic care from birth to adulthood. Early in life, these patients may establish close relationships with their specialized pediatric providers and commonly undergo complex reconstructive procedures. When these patients approach adulthood, they are faced with an informal transition to an adult urologist and may become lost to follow-up. Goals of care during this transition period must be identified and addressed by these patients’ future providers. The concept of transition care is now emerging in urology. We propose that the most effective model for successful transfer of care involves joint involvement from pediatric and adult providers in the same setting.

PURPOSE: Malone antegrade continence enema has been a successful and widely used procedure for achieving fecal continence in children. We present data on the previously uninvestigated issue of patient and caregiver regret following surgery for intractable constipation and fecal incontinence. MATERIALS AND METHODS: We reviewed all patients undergoing antegrade continence enema or cecostomy creation at a single institution between 2006 and 2016. Patients and caregivers were assessed for decisional regret using the Decisional Regret Scale. Results were correlated with demographics, surgical outcomes and complications. RESULTS: A total of 81 responses (49 caregivers and 32 patients) were obtained. Mean followup was 49 months. Decisional regret was noted in 43 subjects (53%), including mild regret in 38 (47%) and moderate to severe regret in 5 (6%). No statistical difference in regret was noted based on gender, complications or performance of concomitant procedures. On regression analysis incontinence was strongly associated with decisional regret (OR 4.4, 95% CI 1.1–18.1, p

Objective To describe clinical management of Fournier's gangrene and to characterize predictive factors associated with inpatient mortality and extended hospital stay. Materials and Methods The National Inpatient Sample was queried from 2004 to 2012 based on the International Classification of Diseases, Ninth Revision, Clinical Modification procedural and diagnosis codes. Patients admitted for Fournier's gangrene who underwent debridement were selected. Multivariate logistic regression analysis was performed to identify predictors of inpatient mortality and extended hospital stay. Results A total of 9249 patients were identified for a weighted estimate of 43,146 cases. Inpatient mortality was 4.7%. The median length of stay was 9 days (interquartile range 5-17 days). The most common procedure in addition to debridement was a complex closure (8.82%), followed by suprapubic tube placement (5.70%) and fecal diversion (4.68%). Increasing age, yearly hospital volume >10 cases, and Medicaid insurance status were associated with increased risk of mortality. Increasing age, teaching hospital status, increasing number of comorbidities, and Medicaid as a payor were predictive of increased hospital stay. Suprapubic tube placement (odds ratio [OR] 2.8 [95% confidence interval {CI} 1.92-4.07], P ≤ .001), fecal diversion (OR 11.1 [95% CI 6.20-19.7], P ≤ .001), and complex wound closure (OR 4.89 [95% CI 3.97-6.89], P ≤ .001) were also predictive of increased length of stay. Conclusion Identifiable patient and hospital characteristics are predictive of both mortality and length of stay in the management of Fournier's gangrene. Overall inpatient mortality appears lower than what has been reported in the majority of prior reports. The strongest predictor for increased length of stay is the need for complex wound closure, and urinary or fecal diversion.

Adolescents with neurogenic bladder are a vulnerable population that severely lacks consistent transitional care from pediatric to adult urology settings.Our practice determined that 100 patients with spina bifida and other neurogenic bladder conditions were not appropriately transferred to the adult setting once reaching adulthood.We initiated a transitional program to establish a dedicated and formal process for adolescent patients to transition to adult urology. The REACH clinic implements a formalized staging framework to facilitate migration of adolescents and young adults to the adult health setting. A social worker was incorporated to act as a patient advocate, behavioral health consultant, and resource specialist.To date 45 patients have been enrolled in the transition program. We have identified and categorized according to the appropriate stage. The REACH clinic has appropriately outlined the goals and mission of the program and resources utilized are financially practical and feasible by conducting a monthly combined clinic. The program has been instrumental in improving tracking and monitoring of these patients through their transition period.Through the efforts of the pediatric and adult urology teams, the REACH program is a dedicated framework that provides structure for transition of the adolescent patient. The addition of a social worker has resulted in enriched rapport and will likely result in improved compliance. This program allows for surveillance and evaluation of patient outcome indicators in this patient population. We believe that early introduction and frequent encounters with the adult urologic team is crucial to successful transitions. Neurourol. Urodynam. 36:973-978, 2017. © 2016 Wiley Periodicals, Inc.

Patients with spinal neuropathy are at an increased risk for urolithiasis. Data on percutaneous nephrolithotomy (PCNL) in this population are limited. Our objective is to review our experience in managing stones with PCNL in patients with spinal neuropathy.Twenty-one patients with spinal neuropathy underwent PCNL at our institution between January 2005 and August 2011. Their medical records were reviewed retrospectively to collect data relating to stone characteristics, treatment outcomes, and complications.Forty-two PCNL were performed on 26 kidneys. Five patients had bilateral stones. They were 14 (66.7%) patients with spinal cord injury, 5 (23.8%) with spina bifida, and 2 (9.5%) with other neurologic abnormalities. There were 90.5% of patients with preoperative bacteriuria and 47.6% with severe scoliosis, making positioning for PCNL challenging. Complete staghorn stones occurred in 46.2% of kidneys, and 50% of stones were struvite. Only 53.8% of kidneys were stone free after the first PCNL. The success rate increased to 80.8% after the second and 88.5% after the third PCNL. Urosepsis developed in three (14.3%) patients, necessitating admission to the intensive care unit postoperatively. Six (28.6%) patients needed blood transfusion. One patient had a pneumothorax and another had a perforation of the collecting system.Based on our experience, PCNL in patients with spinal neuropathy had a stone clearance rate comparable with that of the general population. These patients, however, needed multiple PCNLs to be stone free and had a higher incidence of complications (especially infectious).

Endodermal sinus (yolk sac) tumors (ESTs) are rare neoplasms that most commonly arise in the ovaries or testis. Only six cases of prostatic ESTs have been reported in the literature. We report a case of prostatic EST, the first case in which the patient had a history of previous testis cancer. Treatment included cisplatin-based chemotherapy and radical prostatectomy. Previous cases of primary ESTs and use of cisplatin-based therapy as well as metastatic tumors to the prostate are discussed.

Multiple Endocrine Neoplasia type 2A (MEN-2a) is a rare disease associated with tumors of endocrine organs. Presentation most commonly is with medullary thyroid cancer and infrequently with other complaints. Pituitary adenoma has been seen coincidentally with this disease very rarely. Presented is a case of coincident MEN-2a with a symptomatic pituitary adenoma and an asymptomatic pheochromocytoma. A brief review is also provided.

This is the first tomography-presentation of the optical properties of a normal canine prostate, in vivo, in its native intact environment in the pelvic canal. The imaging was performed by trans-rectal near-infrared (NIR) optical tomography in steady-state measurement at 840 nm on three sagittal planes across the right lobe, middle-line, and left lobe, respectively, of the prostate gland. The NIR imaging planes were position-correlated with concurrently applied trans-rectal ultrasound, albeit there was no spatial prior employed in the NIR tomography reconstruction. The reconstructed peak absorption coefficients of the prostate on the three planes were 0.014, 0.012, and 0.014 mm-1. The peak reduced scattering coefficients were 5.28, 5.56, and 6.53 mm-1. The peak effective attenuation coefficients were 0.45, 0.43, and 0.50 mm-1. The absorption and effective attenuation coefficients were within the ranges predictable at 840 nm by literature values which clustered sparsely from 355 nm to 1064 nm, none of which were performed on a canine prostate with similar conditions. The effective attenuation coefficients of the gland were shown to be generally higher in the internal aspects than in the peripheral aspects, which is consistent with the previous findings that the urethral regions were statistically more attenuating than the capsular regions.

Expression of the proteoglycan core proteins biglycan, decorin, perlecan and syndecan-1, and differentiation related markers of keratins 18 and 20 were examined to determine the origins of the loss of the glycosaminoglycan layer and investigate more fully the altered differentiation of the urothelium in interstitial cystitis.Formalin fixed biopsies from 27 patients with interstitial cystitis and 5 controls were immunohistochemically labeled for the described proteins and scored using a modification of previous scoring for other markers. Inflammation was scored from hematoxylin and eosin stained slides. By combining previous with new data, cluster analysis showed the relationships among the markers and samples.Interstitial cystitis specimens clustered into 4 groups, ranging from most biomarkers abnormal to most biomarkers normal, but all clustered separately from normal controls. One group of interstitial cystitis specimens mainly showed aberrant expression of E-cadherin, which might represent an early abnormality. The biomarkers fell into 2 major groupings. One group consisted of chondroitin sulfate, perlecan, biglycan, decorin and the tight junction protein ZO-1. A second cluster consisted of uroplakin, the epithelial marker keratin 18 and 20, and the morphology of the layer. E-cadherin and syndecan-1 showed little relation to the other 2 clusters or to each other. Inflammation correlated moderately with syndecan-1 but to no other marker.Findings strongly suggest abnormal differentiation in the interstitial cystitis urothelium with a loss of barrier function markers and altered differentiation markers being independent and occurring independently of inflammation. Loss of the glycosaminoglycan layer was associated with a loss of biglycan and perlecan on the luminal layer.

Uropathogenic E. coli (UPEC) strains are described as extraintestinal pathogenic E. coli with preference for the urinary tract. Bottone et al2 recently described the first documentation of a hypermucoviscous phenotype of a UPEC strain that displays a "stringing" phenomenon analogous to those produced by Klebsiella pneumonia strains known to invade the liver. The occurrence of this hypermucoviscous phenotype of UPEC strains causing urinary tract infection has not been well established. Following, we present a case report of two separate renal isolates from a patient with recurrent renal abscesses yielding the aforementioned hypermucoviscous phenotype of UPEC strains.

Pelvic fractures are commonly associated with urethral disruption; however, urethral injury from the repair of these fractures is rarely reported. Thus, the management of these injuries has not been well defined. We report a case of urethral injury from orthopedic screw insertion presenting 3 months postoperatively and effectively managed with removal of the screw and temporary Foley catheterization.

The objective of this study was to evaluate if transrectal optical tomography implemented at three wavelength bands for spectral detection could monitor changes of the hemoglobin oxygen saturation (StO2) in addition to those of the total hemoglobin concentration ([HbT]) in lesions of a canine prostate, including an induced tumor modeling canine prostate cancer. Near-infrared (NIR) optical tomography was integrated with ultrasound (US) for transrectal imaging. Multi-spectral detection at 705 nm, 785 nm and 808 nm rendered measurements of [HbT] and StO2. Canine transmissible venereal tumor (TVT) cells were injected into the right lobe of a dog's prostate gland, which had a pre-existing cyst in the left lobe. Longitudinal assessments of the prostate were performed weekly over a 63-day duration by NIR imaging concurrent with grey-scale and Doppler US. Ultrasonography revealed a bi-lobular tumor-mass regressing from day-49 to day-63. At day-49 this tumor-mass developed a hypoxic core that became larger and more intense by day-56 and expanded further by day-63. The tumor-mass presented a strong hyper-[HbT] feature on day-56 that was inconsistent with US-visualized blood flow. Histology confirmed two necrotic TVT foci within this tumor-mass. The cyst appeared to have a large anoxic-like interior that was greater in size than its ultrasonographically delineated lesion, and a weak lesional elevation of [HbT]. On day-56, the cyst presented a strong hyper-[HbT] feature consistent with US-resolved blood flow. Histology revealed acute and chronic hemorrhage in the periphery of the cyst. The NIR imaging features of two other TVT nodules and a metastatic lymph node were evaluated retrospectively. Transrectal US-integrated spectral optical tomography seems to enable longitudinal monitoring of intra-lesional oxygenation dynamics in addition to the hemoglobin content of lesions in the canine prostate.

Primary large cell neuroendocrine carcinomas (NECs) of the bladder are rarely encountered, and only a few reports have been documented. Frequently, they are found to be admixed with other histologies. In this report, we describe such a tumor found in a 65-year-old man who underwent radical cystectomy, after initial transurethral resection discovered a small cell NEC pathology. We also reviewed the limited number of neuroendocrine tumors reported containing a large cell component. Given the paucity of these tumors and the resultant difficulty in developing generalized treatment protocols, we promote the use of gene expression models to tailor chemotherapeutic regimens for individual tumors.

Ewing sarcoma, also referred to as primitive neuroectodermal tumor (PNET), is a malignancy, histologically characterized by Homer-Wright rosettes and small round cells, that presents most commonly in bone or soft tissue in the pediatric and adolescent populations. We report the case of a patient that presents with intermittent gross hematuria, abdominal mass and elevated blood pressure. After surgical excision of the renal mass, the rare finding of a primary renal Ewing's sarcoma was discovered. After surgery the patient was additionally treated with chemotherapy. After presenting this case, we briefly discuss the unique case of isolated renal Ewing's sarcoma, including incidence and treatment.

Different optical spectral characteristics were observed in a necrotic transmissible venereal tumor (TVT) and a cystic lesion in the same canine prostate by triple-wavelength trans-rectal optical tomography under trans-rectal ultrasound (TRUS) guidance. The NIR imager acquiring at 705nm, 785nm and 808nm was used to quantify both the total hemoglobin concentration (HbT) and oxygen saturation (StO2) in the prostate. The TVT tumor in the canine prostate as a model of prostate cancer was induced in a 7-year old, 27 kg dog. A 2 mL suspension of 2.5x106 cells/mL of homogenized TVT cells recovered from an in vivo subcutaneously propagated TVT tumor in an NOD/SCID mouse were injected in the cranial aspect of the right lobe of the canine prostate. The left lobe of the prostate had a cystic lesion present before TVT inoculation. After the TVT homogenate injection, the prostate was monitored weekly over a 9-week period, using trans-rectal NIR and TRUS in grey-scale and Doppler. A TVT mass within the right lobe developed a necrotic center during the later stages of this study, as the mass presented with substantially increased [HbT] in the periphery, with an area of reduced StO2 less than the area of the mass itself shown on ultrasonography. Conversely, the cystic lesion presented with slightly increased [HbT] in the periphery of the lesion shown on ultrasound with oxygen-reduction inside and in the periphery of the lesion. There was no detectable change of blood flow on Doppler US in the periphery of the cystic lesion. The slightly increased [HbT] in the periphery of the cystic lesion was correlated with intra-lesional hemorrhage upon histopathologic examination.

Needle-based core-biopsy to locate prostate cancer relies heavily upon trans-rectal ultrasound (TRUS) imaging guidance. Ultrasonographic findings of classic hypoechoic peripheral zone lesions have a low specificity of ~28%, a low positive predictive value of ~29%, and an overall accuracy of ~43%, in prostate cancer diagnosis. The prevalence of isoechoic or nearly invisible prostate cancers on ultrasonography ranges from 25 to 42%. As a result, TRUS is useful and convenient to direct the needle trajectory following a systematic biopsy sampling template rather than to target only the potentially malignant lesion for focal-biopsy. To address this deficiency in the first-line of prostate cancer imaging, a trans-rectal ultrasound-coupled spectral tomography (TRUST) approach is being developed to non-invasively resolve the likely optical signatures of prostate malignancy. The approach has evolved from using one NIR wavelength to two NIR bands, and recently to three bands of NIR spectrum information. The concept has been evaluated on one normal canine prostate and three dogs with implanted prostate tumor developed as a model. The initial results implementing TRUST on the canine prostate tumor model includes: (1) quantifying substantially increased total hemoglobin concentration over the time-course of imaging in a rapidly growing prostate tumor; (2) confirming hypoxia in a prostatic cystic lesion; and (3) imaging hypoxic changes of a necrotic prostate tumor. Despite these interesting results, intensive technologic development is necessary for translating the approach to benefiting clinical practice, wherein the ultimate utility is not possibly to eliminate needle-biopsy but to perform focal-biopsy that is only necessary to confirm the cancer, as well as to monitor and predict treatment responses.

OBJECTIVES To evaluate whether trans-rectal spectral optical tomography of total hemoglobin concentration (HbT) can image longitudinal and lateral developments of a canine transmissible venereal tumor (TVT) in a canine prostate. METHODS A near-infrared (NIR) applicator was integrated with a trans-rectal ultrasound (TRUS) transducer to perform ultrasound (US)-coupled optical tomography of the canine prostate. Spectral detection at 785 and 830 nm enabled quantitation of HbT. Canine TVT cells were injected into the right lobe of a dog's prostate gland. Longitudinal imaging assessment of the post-injection prostate was performed by coupled US/NIR imaging over a 45-day duration. RESULTS By day 7, NIR indicated TVT infiltration in the noninjected left prostatic lobe with the gray-scale US indistinct. By day 31, both NIR and gray-scale US revealed more widespread TVT involvement in the left than in the right lobe, as well as an extensive TVT mass in the caudal aspect of the gland, of which the peak HbT increased 3-fold and the mass volume grew exponentially over the 45-day duration. Increased blood supply to the mass was also observed on Doppler US. CONCLUSIONS TRUS-coupled spectral optical tomography enhances assessment of the laterality and progression of prostate tumor compared with using gray-scale and Doppler TRUS.

Spectral trans-rectal ultrasound-coupled optical tomography at 785nm and 830nm has revealed non-invasively longitudinal elevation of total hemoglobin concentration associated with development of transmissible venereal tumors in canine prostate over a 6-week time-course.

A trans-rectal optical tomography applicator for imaging human prostate is being developed. The optical applicator that contains 9 source and 13 detector channels is coupled to a bi-plane trans-rectal ultrasound probe with needle-biopsy assembly.

In vivo trans-rectal near-infrared (NIR) optical tomography is conducted on a tumor-bearing canine prostate with the assistance of trans-rectal ultrasound (TRUS). The canine prostate tumor model is made possible by a unique round cell neoplasm of dogs, transmissible venereal tumor (TVT) that can be transferred from dog to dog regardless of histocompatibility. A characterized TVT cell line was homogenized and passed twice in subcutaneous tissue of NOD/SCID mice. Following the second passage, the tumor was recovered, homogenized and then inoculated by ultrasound guidance into the prostate gland of a healthy dog. The dog was then imaged with a combined trans-rectal NIR and TRUS imager using an integrated trans-rectal NIR/US applicator. The image was taken by NIR and US modalities concurrently, both in sagittal view. The trans-rectal NIR imager is a continuous-wave system that illuminates 7 source channels sequentially by a fiber switch to deliver sufficient light power to the relatively more absorbing prostate tissue and samples 7 detection channels simultaneously by a gated intensified high-resolution CCD camera. This work tests the feasibility of detecting prostate tumor by trans-rectal NIR optical tomography and the benefit of augmenting TRUS with trans-rectal NIR imaging.

Despite the routine use of renal ultrasonography to document progressive improvement in hydronephrosis following ureteral tailoring and reimplantation of megaureters, there have been few reports characterizing the serial radiographic changes to be expected following this procedure. We evaluated the radiographic outcomes following surgical repair of megaureters at single institution, and assessed potential preoperative factors for predicting outcome.We conducted a retrospective analysis of all patients who underwent surgical correction of clinically significant megaureters at our center between 1996 and 2003. Demographic data, indications for surgery, and preoperative and postoperative radiographic imaging data were recorded.A total of 46 megaureters (39 patients) were tapered and reimplanted. Average patient age at surgery was 4.0 years (range 5 months to 19 years). Indications for surgery included recurrent or breakthrough urinary tract infections, decreased renal function and increased hydroureteronephrosis. Mean followup was 3.9 years (range 4 months to 7 years). Postoperative voiding studies showed vesicoureteral reflux in 3 reimplanted ureters (7%). There was no evidence of obstruction on postoperative nuclear renal scans in any patient. Renal ultrasonography revealed improvement or resolution of hydroureteronephrosis in 29 reimplanted units (63%). In general, male patients, those operated on at a younger age and those with a lower preoperative grade of hydronephrosis were most likely to demonstrate improvement or resolution of hydronephrosis. The best results were seen in ectopic megaureters, followed in decreasing order by refluxing megaureters, megaureters associated with ureteroceles and primary obstructive megaureters.Despite functional improvement on postoperative radiographic imaging, it is not uncommon to see persistent hydroureteronephrosis following excisional tapering and reimplantation of megaureters in childhood.

Background Tumor metastasis and changes in host immunosurveillance are important components in cancer development. Tumor cell invasion into the bloodstream is an essential step for systemic metastasis. Currently, the detection of tumor cells in the circulation is mainly dependent upon the utilization of known epithelial cell markers. However, expression of these molecules is not limited to cancer patients; healthy people also have a small number of epithelial cells in their circulation. Utilizing these markers to detect circulating tumor cells (CTCs) cannot adequately explain the mechanisms of tumor cell survival or their development of metastatic potential in peripheral blood. The immune system can also evolve along with the cancer, actually promoting or selecting the outgrowth of tumor variants. Unfortunately, both metastasis and immunosurveillance remain mysterious and are debatable because we have yet to define the molecules that participate in these processes. We are interested in identifying the existence of expressed genes, or mRNA species, that are specifically associated with circulating cells of cancer-bearing patients using prostate cancer (PCa) as a model. Results We established two comprehensive subtracted cDNA libraries using a molecular technique called suppression subtractive hybridization. This technique selectively amplifies transcripts that are specifically expressed in circulating cells of either PCa patients or healthy men. Following sequencing reaction, we showed that 17 out of 23 (73.9%) sequenced clones did not match any mRNAs in the GenBank database. This result suggests that genes associated with alterations in circulating cells of cancer-bearing patients are largely unknown. Semi-quantitative RT-PCR confirmed that two genes are up-regulated in circulating cells of PCa patients, whereas another two genes are down-regulated in the same patients. Conclusion The comprehensive gene expression analysis is capable of identifying differentially expressed genes in circulating cells of healthy men and PCa patients. We did not attempt to enrich specific cell types in this study because phenotypes of CTCs and subsets of leukocytes participating in immunosurveillance remain largely unknown. Continuous studies of these differentially expressed genes will eventually lead us to understand the mechanisms involved in tumor metastasis and immune modulation during cancer development.

Despite a lack of consensus concerning the etiology of interstitial cystitis (IC) the loss of impermeability and other abnormalities of the urothelium are features of the disease. In this study the distribution of proteins involved with epithelial adhesion, cellular differentiation and bladder impermeability in urothelial biopsies were explored by the immunohistochemical assessment of E-cadherin, ZO-1, uroplakin and chondroitin sulfate.Biopsies obtained from 27 patients with IC and 7 controls were immediately fixed in formalin, immunohistochemically labeled for the described proteins and scored for protein expression, morphology and differentiation.Only 3 IC samples appeared completely normal, while 24 of the 27 showed an abnormality in at least 1 marker and in 6 all 4 markers were abnormal. In patients vs controls findings were abnormal for uroplakin in 13 of 27 vs 1 of 7 (p = 0.085), for E-cadherin (over expressed) in 18 of 27 vs 0 of 7 (p = 0.0021), for ZO-1 in 11 of 27 vs 0 of 7 (p = 0.046) and for chondroitin sulfate in 15 of 27 vs 0 of 7 (p = 0.0054). The morphology/polarity score significantly correlated with ZO-1 (Pearson r = 0.3935, p = 0.0423) and chondroitin sulfate (Pearson r = 0.7079, p0.0001) expression. Chondroitin sulfate and ZO-1 showed a high correlation with each other (Pearson r = 0.5587, p = 0.0025). Uroplakin and E-cadherin expression were independent of all other markers.The findings reported strongly suggest abnormal differentiation in the IC bladder. The disruption of ZO-1 is similar to that reported in feline IC. Elevated E-cadherin may represent an adaptation to increased bladder permeability.

A systematic review of the literature was performed to identify barriers, themes, or additional insight specific to the transitional care processes from a pediatric to an adult health care setting for patients with spina bifida.

Background: Melanotic Xp11 translocation renal cancer is a rare tumor belonging to the family of microphthalmiaassociated transcription factor (MiTF)/transcription factor E (TFE) neoplasms. This tumor family also includes alveolar soft part sarcoma, perivascular epithelioid cell neoplasms, Xp11 translocation renal cell carcinoma, and melanoma. To date, six confirmed melanotic Xp11 translocation cancers (five renal, one ovarian) have been reported in the literature. Case Report: Here, we report the clinical, histologic, immunohistochemical, and molecular features of a unique melanotic Xp11 translocation renal cancer arising in a 34-year-old African-American female. Histologically, the tumor was composed of epithelioid tumor cells arranged in a nested pattern. The cells had clear to eosinophilic granular cytoplasm, vesicular nuclear chromatin, and prominent nucleoli. Multifocal intracytoplasmic deposits of granular brown melanin pigment were identified and confirmed by Fontana-Masson stain. An unusual histologic feature, not previously reported in melanotic Xp11 translocation renal cancer, was a sarcoid-like granulomatous reaction consisting of tight epithelioid granulomas with lymphocytic cuffing, numerous giant cells, and calcifications. Nuclear transcription factor E3 expression was identified by immunohistochemistry and TFE3 rearrangement was confirmed by fluorescence in situ hybridization. Additional immunohistochemical findings included immunoreactivity for HMB45, cathepsin K, and progesterone receptor; negative staining was seen with actin, desmin, cytokeratins, epithelial membrane antigen, CD10, vimentin, and PAX-8. The patient is currently free of disease, two years following initial clinicoradiologic presentation and twenty-two months following partial nephrectomy without additional therapy. Conclusion: This report further expands the spectrum of morphologic and clinical findings previously described in melanotic Xp11 translocation renal cancer, a distinctive tumor showing overlapping features between Xp11 translocation renal cell carcinoma, melanoma, and perivascular epithelioid cell neoplasms. Virtual slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/ 7225796341180634