Causes of death among people with myelomeningocele: A multi-institutional 47-year retrospective study.

PURPOSE: This study aimed to analyze organ system-based causes and non-organ system-based mechanisms of death (COD, MOD) in people with myelomeningocele (MMC), comparing urological to other COD. METHODS: A retrospective review was performed of 16 institutions in Canada/United States of non-random convenience sample of people with MMC (born > = 1972) using non-parametric statistics. RESULTS: Of 293 deaths (89% shunted hydrocephalus), 12% occurred in infancy, 35% in childhood, and 53% in adulthood (documented COD: 74%). For 261 shunted individuals, leading COD were neurological (21%) and pulmonary (17%), and leading MOD were infections (34%, including shunt infections: 4%) and non-infectious shunt malfunctions (14%). For 32 unshunted individuals, leading COD were pulmonary (34%) and cardiovascular (13%), and leading MOD were infections (38%) and non-infectious pulmonary (16%). COD and MOD varied by shunt status and age (p < = 0.04), not ambulation or birthyear (p > = 0.16). Urology-related deaths (urosepsis, renal failure, hematuria, bladder perforation/cancer: 10%) were more likely in females (p = 0.01), independent of age, shunt, or ambulatory status (p > = 0.40). COD/MOD were independent of bladder augmentation (p = >0.11). Unexplained deaths while asleep (4%) were independent of age, shunt status, and epilepsy (p >= 0.47). CONCLUSION: COD varied by shunt status. Leading MOD were infectious. Urology-related deaths (10%) were independent of shunt status; 26% of COD were unknown. Life-long multidisciplinary care and accurate mortality documentation are needed.

Application of the decisional regret scale (DRS) in men undergoing anti-incontinence procedures for stress urinary incontinence.

Background: Decisional regret (DR) has previously been applied to other urologic issues (hypospadias, prostate cancer). The present study seeks to evaluate certain patient factors that directly correlate to high DR scores in anti-incontinence procedures. Methods: Medical records for 119 patients undergoing anti-incontinence procedures between 2009 and 2020 were retrospectively reviewed. Forty-one patients were accessible for telephone follow-up and provided a Decisional Regret Scale (DRS) questionnaire. If patients had both a sling and an artificial urinary sphincter (AUS) placed, questionnaires were administered for each. DRS score was quantified in accordance with prior literature, with scores ranging 0-100. We subdivided patients based on demographics and surgical data, correlating this with DRS score. Results: In 41 patients, 46 procedures (13 slings, 33 AUS) were performed. Thirty-nine (95.1%) men underwent robotic-assisted laparoscopic prostatectomy, and 11 (26.8%) men reported prior pelvic radiation. Post-procedural continence, irrespective of procedure, yielded an average 2±1.56 pads per day (PPD). Mean DRS score across the cohort was 29.78. DRS score was subdivided into mild, moderate, and severe, with majority noting "none to mild" regret (63%), 15.2% reporting moderate and 21.7% severe. Predictors of higher regret included history of radiation (P=0.056), choice of anti-incontinence procedure (P=0.011), and need for surgical revision (P=0.00042). DR was unrelated to race, complete continence, and time to follow-up. Conclusions: DRS has recently been applied to anti-incontinence procedures for male stress incontinence; our study highlights novel findings not previously assessed. Majority of men had minimal regret with a subset that had significant regret-history of radiation, multiple revisions/explant, and those who elected for sling upfront. These results highlight the importance of patient selection and pre-operative counselling.

Stromal sarcoma of the prostate.

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.

Analysis of Factors Associated with Patient or Caregiver Regret following Surgery for Fecal Incontinence.

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 <0.001) and regret increased as age at surgery increased, particularly when patients were operated on at age 13 to 15 years (OR 2.6, 95% CI 1.0-6.4 for age 13 years; OR 2.9, 95% CI 1.1-7.8 for age 14 years; OR 3.1, 95% CI 1.1-8.8 for age 15 years). CONCLUSIONS: This is the first known study describing decisional regret following surgery for fecal incontinence. Surgical factors aimed at achieving continence may be effective in decreasing postoperative regret. The finding of increased regret in teenage patients compared to younger children should be shared with families since it may impact the age at which surgery is pursued.

Contemporary Trends in the Inpatient Management of Fournier's Gangrene: Predictors of Length of Stay and Mortality Based on Population-based Sample.

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.

Primary Yolk Sac Tumor of the Prostate: A Case Report and Review of the Literature.

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.

Melanotic Xp11 translocation renal cancer: report of a case with a unique intratumoral sarcoid-like reaction.

BACKGROUND: Melanotic Xp11 translocation renal cancer is a rare tumor belonging to the family of microphthalmia-associated 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.

Surgical management of urolithiasis in spinal cord injury patients.

Urolithiasis is a common condition in patients with spinal cord injury (SCI). Surgical management of stones in this population is more challenging and associated with lower clearance rates than the general population. The rate of complications - specifically infectious complications - is also high due to the chronic bacterial colonization. Shock wave lithotripsy (SWL) has a low clearance rate of 44-73 %. Percutaneous nephrolithotripsy is indicated for larger nephrolithiasis, but multiple procedures may be required to clear the stones. Ureteroscopy has been associated with low success rates because of difficulty in obtaining ureteral access. Historically, bladder stones were managed with open surgery or SWL. Recently, good results have been reported with the combination of endoscopic and laparoscopic techniques. Surgical management of urolithiasis in patients with SCI should be performed in high-volume centers in light of the technical challenges and higher rate of perioperative complications.

Chronic Cystitis Associated With Keratinizing Squamous Metaplasia Progressing to Squamous Papilloma.

We present a patient with extensive keratinizing squamous metaplasia that progressed to squamous papilloma over the course of follow-up. To our knowledge, this is the first published case of keratinizing squamous metaplasia with melanotic deposits of an unknown material with synchronous development of squamous papilloma.

Muscle-invasive bladder cancer in a young adult: a case report and a review of the literature.

The peak incidence of bladder cancer (BC) is in the sixth decade of life. Muscle-invasive bladder cancer (MIBC) in young adults is extremely rare. We report a case of MIBC in a 28-year-old smoking male patient. The patient presented with hematuria and flank pain for which he underwent a computerized tomography (CT) scan of the abdomen and pelvis with and without contrast. The CT scan showed a 6 cm mass on the left side of the trigone extending to the left urteric orifice and left hydronephrosis, but no lymphadenopathy was noted. The patient then underwent a left nephrostomy tube placement followed by trans-urethral resection of bladder tumor (TURBT). The tumor involved both ureteric orifices and extended to the prostatic urethra. Complete resection was not feasible. Pathology showed high-grade T1 urothelial carcinoma. CT scan of the chest showed no distant lung metastasis. The patient then elected to undergo radical cystectomy with ileal conduit urinary diversion. Final pathology revealed T2a N0 urothelial carcinoma of the bladder. Our aim is to present our experience and review the literature for the natural history and oncological and quality of life outcomes of urothelial carcinoma of the bladder in young patients.

Plasmacytoid urothelial carcinoma of the bladder metastatic to the stomach: a case report.

Introduction. Plasmacytoid urothelial carcinoma (PUC) of the bladder is a rare histological variant of urothelial carcinoma that was recently identified. Available data on this histological variant is limited. Case Report. We report the case of a 75-year-old man with presumed history of high-grade urothelial cancer of the bladder, treated with transurethral resection and Bacille Calmette-Guérin (BCG) in 2004. Six years after treatment of the bladder cancer, the patient underwent gastrectomy for an undifferentiated carcinoma of the stomach. On followup, patient developed right ureterohydronephrosis and peritoneal carcinomatosis. Biopsy of the bladder during stent placement revealed a plasmacytoid urothelial carcinoma of the bladder. Rereadings revealed that the initial bladder and gastric malignancies were also plasmacytoid carcinoma, indicating that, the patient had since 2004, a PUC of the bladder that spread to the stomach and peritoneal cavity. Conclusion. Plasmacytoid urothelial carcinoma of the bladder is an aggressive variant of urothelial carcinoma. Based on our case and the literature review, this tumor can be misdiagnosed because of its rarity, leading to treatment delays. Both the urologist and the pathologist need to have a high index of suspicion for PUC whenever they encounter unusual clinical and/or pathological findings.

Primary mixed neuroendocrine carcinoma of the bladder with large cell component: a case report and review of the literature.

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.

Multiple endocrine neoplasia 2a presenting with pheochromocytoma and pituitary macroadenoma.

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.

Primary Ewing's sarcoma of the kidney with caval involvement: a case report.

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.

Primary carcinoid tumor of the ileal efferent limb of an ileovesicostomy: a case report.

We report on the evaluation and management of a 47-year-old white male found to have primary carcinoid tumor of the ileal segment of his diverting ileovesicostomy thirty-five months after initial creation. Subsequent to presentation with intermittent gross hematuria, CT urogram highlights an 8 mm enhancing lesion near the enterovesical junction of urinary diversion. Office cystoscopy confirms presence of a lesion that was later endoscopically resected and found to be a well-differentiated carcinoid tumor. Evaluation with serum markers, direct visualization utilizing endoscopy, and imaging was without finding of alternate primary or metastatic lesions. The patient ultimately had the proximal ileal portion of his ileovesicostomy excised and the distal portion converted into an ileal conduit. After briefly discussing the carcinoid tumor and the carcinoid syndrome it may cause, we review the literature on the incidence of carcinoid tumors in a population requiring the use of intestine in the urinary tract.

In vivo trans-rectal ultrasound-coupled optical tomography of a transmissible venereal tumor model in the canine pelvic canal.

In vivo trans-rectal near-infrared (NIR) optical tomography was performed concurrently with, albeit reconstructed without spatial a prior of, trans-rectal ultrasound (US) on transmissible venereal tumor (TVT) developed as a model in the canine pelvic canal. Studies were taken longitudinally at prior to, 14 days after, and 35 days after the TVT injection. As the tumor grew, the nodules became increasingly hyperabsorptive and moderately hyperscattering on NIR. The regions of strong NIR contrast, especially on absorption images, correlated well with those of US hypoechoic masses indicative of tumors. Combining the information of trans-rectal NIR and US detected the tumor more accurately than did the US alone at 14 days postinjection.

Abnormal expression of differentiation related proteins and proteoglycan core proteins in the urothelium of patients with interstitial cystitis.

PURPOSE: 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. MATERIALS AND METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Radiographic changes following excisional tapering and reimplantation of megaureters in childhood: long-term outcome in 46 renal units.

PURPOSE: 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. MATERIALS AND METHODS: 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. RESULTS: 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. CONCLUSIONS: Despite functional improvement on postoperative radiographic imaging, it is not uncommon to see persistent hydroureteronephrosis following excisional tapering and reimplantation of megaureters in childhood.

Screening and identification of differentially expressed transcripts in circulating cells of prostate cancer patients using suppression subtractive hybridization.

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.

Abnormal expression of molecular markers for bladder impermeability and differentiation in the urothelium of patients with interstitial cystitis.

PURPOSE: 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. MATERIALS AND METHODS: 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. RESULTS: 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, p <0.0001) 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. CONCLUSIONS: 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.