Ewing's sarcoma of the male external genitalia: a case report and review of the literature.

BACKGROUND: Ewing's sarcoma (ES) within the genitourinary tract are relatively unheard of and those within the external male genitalia are even rarer. To our knowledge, this is the first known case of primary ES within the paratesticular region in an adult. CASE PRESENTATION: We present a case of a 24-year-old man with a right sided testicular mass on examination that was initially characterized as an adenomatoid tumor on ultrasound. After the patient was lost to follow up over the course of 9 months, the testicular mass grew significantly and was excised with pathology revealing primary paratesticular Ewing's sarcoma. This rare case emphasizes the importance of elucidating between the broad differentials of paratesticular masses, including the rare presentation of primary ES and adds a review of the literature of ES in the external male genitalia. CONCLUSIONS: Rare differentials such as this case should be considered in patients with paratesticular masses. Further diagnostic and management algorithms for extraosseous Ewing Sarcoma, particularly in the adult population, are warranted.

Comparing Survival Outcomes and Costs Associated With Radical Cystectomy and Trimodal Therapy for Older Adults With Muscle-Invasive Bladder Cancer.

Importance: Radical cystectomy is the guidelines-recommended treatment of muscle-invasive bladder cancer, but a resurgence of trimodal therapy has occurred. Limited comparative data are available on outcomes and costs attributable to these 2 treatments. Objective: To compare the survival outcomes and costs between trimodal therapy and radical cystectomy in older adults with muscle-invasive bladder cancer. Design, Setting, and Participants: This population-based cohort study used data from the Surveillance, Epidemiology, and End Results-Medicare linked database. A total of 3200 older adults (aged ≥66 years) with clinical stage T2 to T4a bladder cancer diagnosed from January 1, 2002, to December 31, 2011, and with claims data available through December 31, 2013, were included in the analysis. Patients who received radical cystectomy underwent either only surgery or surgery in combination with radiotherapy or chemotherapy. Patients who received trimodal therapy underwent transurethral resection of the bladder followed by radiotherapy and chemotherapy. Propensity score matching by sociodemographic and clinical characteristics was used. Data analysis was performed from August 1, 2017, to March 11, 2018. Main Outcomes and Measures: Overall survival and cancer-specific survival were evaluated using the Cox proportional hazards regression model and the Fine and Gray competing risk model. All Medicare health care costs for inpatient, outpatient, and physician services within 30, 90, and 180 days of treatment were compared. The total amount spent nationwide was estimated, using 180-day medical costs between treatments, by the total number of new cases of muscle-invasive bladder cancer in the United States in 2011. Results: Of the 3200 patients who met the inclusion criteria, 2048 (64.0%) were men and 1152 (36.0%) were women, with a mean (SD) age of 75.8 (6.0) years. After propensity score matching, 687 patients (21.5%) underwent trimodal therapy and 687 patients (21.5%) underwent radical cystectomy. Patients who underwent trimodal therapy had significantly decreased overall survival (hazard ratio [HR], 1.49; 95% CI, 1.31-1.69) and cancer-specific survival (HR, 1.55; 95% CI, 1.32-1.83). No differences in costs at 30 days were observed between trimodal therapy ($15 233 in 2002 vs $18 743 in 2011) and radical cystectomy ($17 990 in 2002 vs $21 738 in 2011). However, median total costs were significantly higher with trimodal therapy than with radical cystectomy at 90 days ($80 174 vs $69 181; median difference, $8964; Hodges-Lehmann 95% CI, $3848-$14 079) and at 180 days ($179 891 vs $107 017; median difference, $63 771; Hodges-Lehmann 95% CI, $55 512-$72 029). Extrapolating these figures to the total US population revealed $335 million in excess spending for trimodal therapy compared with the less costly radical cystectomy ($492 million) for patients who received a muscle-invasive bladder cancer diagnosis in 2011. Conclusions and Relevance: Trimodal therapy was associated with significantly decreased overall survival and cancer-specific survival as well as $335 million in excess spending in 2011. These findings have important health policy implications regarding the appropriate use of high value-based care among older adults with invasive bladder cancer who are candidates for either radical cystectomy or trimodal therapy.

Atypical small acinar proliferation at index prostate biopsy: rethinking the re-biopsy paradigm.

PURPOSE: Guidelines for atypical small acinar proliferation (ASAP) diagnosed on prostate biopsy recommend repeat biopsy within 3-6 months after diagnosis. We sought to discern the rate of detecting clinically significant prostate cancer on repeat biopsy and predictors associated with progression. MATERIALS AND METHODS: We performed a retrospective chart review of patients who underwent prostate biopsy at our institution from January 1, 2008, to December 31, 2015. Gleason grade group (GGG) system and D'Amico stratification were used to report pathology and risk stratification, respectively. Logistic and linear regression analyses were performed. RESULTS: A total of 593 patients underwent transrectal ultrasound-guided prostate biopsy, of which 27 (4.6%) had the diagnosis of ASAP. Of these, 11 (41%) had a repeat biopsy. Median time from diagnosis to repeat biopsy was 147 days (IQR 83.5-247.0). Distribution across the GGG system on repeat biopsy was as follows: 7 (63.6%) benign, 3 (27.3%) GG1, and 1 (9.1%) GG2. ASAP was not associated with subsequent diagnosis of clinically significant prostate cancer (OR 0.46, 95% CI 0.064-3.247, P = 0.432). There was no association between ASAP and high cancer risk (ASAP: ß = - 0.12; P = 0.204). CONCLUSIONS: Patients diagnosed with ASAP managed according to guideline recommendations are more likely diagnosed with benign pathology and indolent prostate cancer on repeat biopsy. These findings support prior studies suggesting refinement of guidelines in regard to the appropriateness and timeliness of repeat biopsy among patients diagnosed with ASAP.

Primary Malignancy in a Supernumerary Testicle Presenting as a Large Pelvic Mass.

Supernumerary testis, also known as polyorchidism, is a condition characterized by the presence of more than two testes. Another condition of the testes is seminoma, a common cause of testicular germ cell tumor. A 35-year-old male was transferred to our hospital with a diagnosis of abdominal mass causing abdominal pain. On physical exam, he had a palpable undescended left testicle in the left inguinal canal, which was determined to be seminoma. The mass was surgically removed, and the patient underwent chemotherapy. The report discusses his workup, treatment, and outcome. This case illustrates an unusual presentation of supernumerary testis with the extra testis harboring a seminoma. When presented with a case of testicular cancer with no tumor noted in the palpable testes, malignancy in an extranumerary testicle should be considered in the differential.

Cancer and All-cause Mortality in Bladder Cancer Patients Undergoing Radical Cystectomy: Development and Validation of a Nomogram for Treatment Decision-making.

OBJECTIVE: To develop and validate a nomogram assessing cancer and all-cause mortality following radical cystectomy. Given concerns regarding the morbidity associated with surgery, there is a need for incorporation of cancer-specific and competing risks into patient counseling and recommendations. MATERIALS AND METHODS: A total of 5325 and 1257 diagnosed with clinical stage T2-T4a muscle-invasive bladder cancer from January 1, 2006 to December 31, 2011 from Surveillance, Epidemiology, and End Results-Medicare and Texas Cancer Registry-Medicare linked data, respectively. Cox proportional hazards models were used and a nomogram was developed to predict 3- and 5-year overall and cancer-specific survival with external validation. RESULTS: Patients who underwent radical cystectomy were mostly younger, male, married, non-Hispanic white and had fewer comorbidities than those who did not undergo radical cystectomy (P < .001). Married patients, in comparison with their unmarried counterparts, had both improved overall (hazard ratio 0.76; 95% confidence interval 0.70-0.83, P < .001) and cancer-specific (hazard ratio 0.76; 95% confidence interval 0.68-0.85, P < .001) survival. A nomogram developed using Surveillance, Epidemiology, and End Results-Medicare data, predicted 3- and 5-year overall and cancer-specific survival rates with concordance indices of 0.65 and 0.66 in the validated Texas Cancer Registry-Medicare cohort, respectively. CONCLUSION: Older, unmarried patients with increased comorbidities are less likely to undergo radical cystectomy. We developed and validated a generalizable instrument that has been converted into an online tool (Radical Cystectomy Survival Calculator), to provide a benefit-risk assessment for patients considering radical cystectomy.

Increased Utilization of Positron Emission Tomography/Computed Tomography (PET/CT) Imaging and Its Economic Impact for Patients Diagnosed With Bladder Cancer.

BACKGROUND: The purpose of this study was to examine temporal nationwide utilization patterns and predictors for use of positron emission tomography/computed tomography (PET/CT) in comparison with magnetic resonance imaging (MRI) and computed tomography (CT) among patients diagnosed with bladder cancer. MATERIALS AND METHODS: A total of 36,855 patients aged 66 years or older diagnosed with clinical stage TI-IV, N0M0 bladder cancer from 2004 to 2011 were analyzed. We used multivariable logistic regression analyses to discern factors associated with receipt of imaging within 12 months from diagnosis. The Cochran-Armitage test for trend was used to determine changes in the proportion of patients receiving imaging after cancer diagnosis. RESULTS: Independent of clinical stage, there was marked increase in use of PET/CT throughout the study period (2011 vs. 2004: odds ratio, 17.55; 95% confidence interval, 10.14-30.38; P < .001). Although use of CT imaging remained stable during the study period, there was significantly decreased utilization of MRI (odds ratio, 0.60; 95% confidence interval, 0.49-0.75; P < .001) in 2011 versus 2004. The mean incremental cost of PET/CT versus CT and MRI was $1040 and $612 (in 2016 dollars), respectively. Extrapolating these findings to the patients with bladder cancer in the United States results in excess spending of $11.6 million for PET/CT imaging. CONCLUSION: We identified rapid adoption of PET/CT imaging independent of clinical stage, resulting in excess national spending of $11.6 million for this imaging modality alone. Further value-based research discerning the clinical versus economic benefits of advanced imaging among patients with bladder cancer are needed.

Survival differences among patients with bladder cancer according to sex: Critical evaluation of radical cystectomy use and delay to treatment.

OBJECTIVE: Sex differences in bladder cancer survival are well known. However, the effect of type of treatment, timing to surgery when rendered, and survival outcomes according to sex have not been extensively examined. Given the relatively low incidence of bladder cancer in females, large multicenter and population-based studies are required to elucidate sex differences in survival. In this study, we sought to characterize the effect of use and timing of radical cystectomy (RC) according to sex and survival outcomes. METHODS: A total of 9,907 patients aged 66 years or older diagnosed with clinical stage II to IV N0M0 bladder cancer from January 1, 2001 to December 31, 2011 from Surveillance, Epidemiology, and End Results-Medicare data were analyzed. We used multivariable regression analyses to identify factors predicting the use and delay of RC. Cox proportional hazards models were used to analyze survival outcomes. RESULTS: Of the 9,907 patients diagnosed with bladder cancer, 3,256 (32.9%) were females. Women were significantly more likely to undergo RC across all stages compared to their male counterparts (stage II: relative risk [RR] = 1.48, 95% CI: 1.33-1.65, P<0.001; stage III: RR = 1.24, 95% CI: 1.13-1.37, P<0.001; and stage IV: RR = 1.33, 95% CI: 1.19-1.49, P<0.001). Moreover, there was no significant difference in delay to RC according to sex across all clinical stages. Using propensity score matching, women had worse overall (hazard ratio = 1.07; CI: 1.01-1.14; P = 0.024), and worse cancer-specific survival (hazard ratio = 1.26; CI: 1.17-1.36, P<0.001) than men. CONCLUSION: Sex differences persist with women who are significantly more likely to undergo RC independent of clinical stage. However, women have significantly worse survival than men. Delay from diagnosis to surgery did not account for this decreased survival among women.

Novel intronic germline FLCN gene mutation in a patient with multiple ipsilateral renal neoplasms.

Multiple renal tumors of diverse morphology are rare and typically seen in Birt-Hogg-Dubé syndrome. Birt-Hogg-Dubé syndrome is a rare inherited cancer syndrome caused by a germline mutation in the folliculin (FLCN) gene, but the genetic causes for histologic diversity of renal tumors in Birt-Hogg-Dubé syndrome have not been elucidated. We describe here a 64-year-old man with a novel germline mutation in the FLCN gene who presented with 3 phenotypically distinct renal tumors in the same kidney, which were histologically classified as oncocytoma (1.4 cm), oncocytic papillary carcinoma (0.5 cm), and clear cell renal carcinoma (0.8 cm). Genetic analysis of normal kidney tissue revealed a heterozygous germline FLCN mutation (intron 9, IVS9+6 C>T). Additional molecular genetic testing revealed somatic mutations and epigenetic events in genes typically associated with these specific histologic tumor types: oncocytoma harbored a second FLCN mutation (intron 12, IVS12+4 C>T), oncocytic papillary carcinoma harbored promoter methylation of FLCN, and a missense mutation in the MET gene (P246L), whereas clear cell carcinoma harbored inactivating VHL mutation (5-base pair deletion in exon 2) and VHL gene promoter methylation. In addition, chromosomal analysis of peripheral blood lymphocytes showed low level chromosome instability, not previously associated with germline mutations in the FLCN gene.

Ductal prostate cancer: contemporary management and outcomes.

OBJECTIVES: To evaluate contemporary management and outcomes of ductal prostate cancer (PCA). MATERIALS AND METHODS: We reviewed all patients with ductal PCA and at least 6 months of follow-up seen at UTMB from 1990 to 2005, which comprised 17 patients (mean age: 67.7 years, range 55-87). At time of diagnosis, 11 patients had localized disease (Group 1) and 6 patients had distant metastasis (Group 2). RESULTS: Treatment of Group 1 patients included radiation and endocrine treatment for at least 2 years (n = 7), radiation alone (n = 2), and radical surgery (n = 2). At a mean follow-up of 3.6 years (r = 1-12 years) 8 patients (67.7%) remained free of recurrence, 1 patient had biochemical recurrence alone, 1 patient had recurrence in the anterior urethra, and the other had progression with metastasis to the brain and subsequent death. In addition to metastasis to regional/distant lymph nodes and bone in Group 2, metastatic sites included brain (n = 1), peritoneum (n = 1), and lung (n = 1). Mean follow-up was 2.3 years (r = 8 months to 4 years). All patients received androgen deprivation. One patient had progression of disease despite lack of biochemical recurrence and is alive at 2.5 years. One patient died from other causes while the 4 remaining patients are in remission at last follow-up. CONCLUSIONS: Contemporary management of localized ductal PCA with radiation and endocrine therapy yields adequate disease-free survival. Metastatic sites include brain, lung, peritoneum, and anterior urethra, and most patients respond well to endocrine treatment.

Characteristics of urologists predict the use of androgen deprivation therapy for prostate cancer.

PURPOSE: We previously have reported wide variations among urologists in the use of androgen deprivation for prostate cancer. Using the Surveillance, Epidemiology, and End Results-Medicare linked database, we examined how individual urologist characteristics influenced the use of androgen deprivation therapy. METHODS: Participants included 82,375 men with prostate cancer who were diagnosed from January 1, 1992, through December 31, 2002, and the 2,080 urologists who provided care to them. Multilevel analyses were used to estimate the likelihood of androgen deprivation use within 6 months of diagnosis in the overall cohort, in a subgroup in which use would be of uncertain benefit (primary therapy for localized prostate cancer), and in a subgroup in which use would be evidence-based (adjuvant therapy with radiation for locally advanced disease). RESULTS: In the overall cohort of patients, a multilevel model adjusted for patient characteristics, tumor characteristics, and urologist characteristics (eg, board certification, academic affiliation, patient panel size, years since medical school graduation) showed that the likelihood of androgen deprivation use was significantly greater for patients who saw urologists without an academic affiliation. This pattern also was noted when the analysis was limited to settings in which androgen deprivation would have been of uncertain benefit. Odds ratios for use in that context were 1.66 (95% CI, 1.27 to 2.16) for urologists with no academic affiliation and 1.45 (95% CI, 1.13 to 1.85) for urologists with minor versus major academic affiliations. CONCLUSION: Use of androgen deprivation for prostate cancer varies by the characteristics of the urologist. Patients of non-academically affiliated urologists were significantly more likely to receive primary androgen deprivation therapy for localized prostate cancer, a setting in which the benefits are uncertain.

Retroperitoneal mucinous cystadenocarcinoma in a man: case report and review of the literature.

In the English medical literature, 27 cases of primary retroperitoneal mucinous cystadenocarcinoma have been published. We report the second case of a primary retroperitoneal mucinous cystadenocarcinoma in a man. The patient was an 83-year-old man, with a large 26 x 20 x 16-cm retroperitoneal cystic mass causing abdominal discomfort and cachexia, who underwent excision of the mass. Prior reports suggest that this type of tumor has an aggressive clinical course, and surgical excision is the treatment of choice. These rare tumors need to be included in the differential of retroperitoneal cystic tumors.

Isolated recurrence of ductal prostate cancer to anterior urethra.

We report the case of a 74-year-old man with ductal prostate cancer who had originally undergone radiotherapy but developed metastases to the anterior urethral mucosa. This is the fourth such case ever reported in English publications. We suspect the metastases developed from implantation after instrumentation, a common finding in the previously reported cases. Although no specific treatment regimen exists, we believe that local resection followed by androgen deprivation is the treatment of choice in these patients.

Increasing use of gonadotropin-releasing hormone agonists for the treatment of localized prostate carcinoma.

BACKGROUND: The role of androgen deprivation therapy in prostate carcinoma is controversial in earlier stages of disease. The authors examined the time trends and patterns of use for androgen deprivation in the form of gonadotropin-releasing hormone (GnRH) agonists or orchiectomy, in population-based tumor registries. METHODS: Data were obtained from the linked Surveillance, Epidemiology and End Results-Medicare database. A total of 100,274 men with prostate carcinoma diagnosed from 1991 through 1999 were selected. The main outcome was the proportion of men who received >/= 1 dose of a GnRH agonist in the first 6 months of diagnosis. This was plotted by year and stratified for age, grade, stage as well as primary versus adjuvant usage. Multiple logistic regression was used to examine predictors of GnRH agonist use in the subset of patients with localized cancer. RESULTS: There was a consistent increase in GnRH agonist use by year for all ages, stages, and grades. Even in men >/= 80 years with localized stage and low to moderate grade tumors, primary GnRH agonist use increased over the study period, from 3.7% in 1991 to 30.9% in 1999 (P < 0.001). The multivariable analysis showed that significant variability in GnRH agonist use existed among SEER geographic regions. CONCLUSIONS: The use of GnRH agonists for prostate carcinoma increased dramatically during the 1990s. This increase occurred across all stages and histologic grades of prostate carcinoma, and was greatest in patients >/= 80 years.

Arterialization of deep dorsal vein of penis for penile ischemia.

Penile ischemia, a rare complication of diabetic end-stage renal disease, is usually treated by penectomy once conservative measures fail. We present a patient with diabetes mellitus and end-stage renal disease with penile ischemia that was successfully treated with an arteriovenous interposition bypass graft between the common femoral artery and the deep dorsal vein of the penis. Retrograde flow into the corpus spongiosum resulted in immediate pain relief and healing of the ischemic lesions.

Renal and perirenal abscesses in patients with otherwise anatomically normal urinary tracts.

PURPOSE: We characterized evaluation, management and outcomes in a group of patients diagnosed with renal and perirenal abscesses who had otherwise anatomically normal urinary tracts. MATERIALS AND METHODS: We reviewed our experience with renal/perirenal abscesses at University of Texas Medical Branch from 1991 to 2002. Treatment was determined by physician preference in each individual. RESULTS: Of 70 patients with renal/perirenal abscesses 26 had otherwise anatomically normal urinary tracts, 24 (92%) had at least 1 possible contributory factor, such as diabetes mellitus, and only 38% had the correct diagnosis at initial presentation. The abscess was intranephric in 39% of cases, intranephric and perinephric in 19%, and perinephric only in 42%. Of the 26 patients 18 were treated with percutaneous drainage or aspiration of the abscess and 12 (66.7%) had positive cultures. Eight of the 12 patients (67%) with positive abscess cultures had the same organism in urine and/or blood. All 26 patients were treated with broad-spectrum intravenous antibiotics. In most patients abscess size influenced additional treatments, such as percutaneous needle aspiration or catheter drainage. None of the patients required open surgical drainage, nephrectomy or nephrostomy tube placement. At a mean followup of 10 months all patients had complete radiographic resolution of the abscess without further complications except 1 who had pyelonephritis and another who was found to have a poorly perfused kidney. CONCLUSIONS: With accurate diagnosis and minimally invasive therapy patients with renal and/or perirenal abscesses and otherwise anatomically normal urinary tracts have excellent functional and anatomical outcomes.

Testicular involvement in disseminated fungal infection by Pseudallescheria boydii.

We present an immunocompromised patient with a painless testicular mass. Usually, this mass is suspicious for testicular cancer; however, a fungal abscess from Pseudallescheria boydii was found. The patient eventually died from disseminating infection. This is the first such report of a testicular infection involving P. boydii. Early diagnosis and treatment are paramount because of the poor outcome with disseminated disease.

Genital and perineal burns in children: 10 years of experience at a major burn center.

PURPOSE: The aim of this report is to review a decade of experience in the management of perineal and genital burns at a major burn center. METHODS: Seventy-eight children who sustained perineal or genital burns admitted to the Shriners Hospitals for Children in Galveston are discussed. RESULTS: Genital and perineal burns occurred in the context of major burns and were rarely isolated. A total of 64.1% were caused by hot liquids (scalds), 29.5% were flame burns, 3.8% contact burns, and 2.6% electrical burns. A total of 61% of the burns could be treated conservatively with loose debridement, topical, and parenteral antibiotics with satisfactory outcomes. Foley catheterization did not increase the morbidity in these patients except in children less than 1 year of age. Testicular involvement was associated with the most severe burns. Child abuse was found in 46% and 48% of boys and girls, respectively, younger than 2 years that had sustained scald burns to the perineum and, or genitals. CONCLUSIONS: Most perineal and genital burns in children can be treated successfully with a conservative approach. Child abuse should be vigorously investigated.