Prognostic factors for urachal cancer: a bayesian model-averaging approach.

PURPOSE: This study was conducted to evaluate prognostic factors and cancer-specific survival (CSS) in a cohort of 41 patients with urachal carcinoma by use of a Bayesian model-averaging approach. MATERIALS AND METHODS: Our cohort included 41 patients with urachal carcinoma who underwent extended partial cystectomy, total cystectomy, transurethral resection, chemotherapy, or radiotherapy at a single institute. All patients were classified by both the Sheldon and the Mayo staging systems according to histopathologic reports and preoperative radiologic findings. Kaplan-Meier survival curves and Cox proportional-hazards regression models were carried out to investigate prognostic factors, and a Bayesian model-averaging approach was performed to confirm the significance of each variable by using posterior probabilities. RESULTS: The mean age of the patients was 49.88 ± 13.80 years and the male-to-female ratio was 24:17. The median follow-up was 5.42 years (interquartile range, 2.8-8.4 years). Five- and 10-year CSS rates were 55.9% and 43.4%, respectively. Lower Sheldon (p=0.004) and Mayo (p<0.001) stage, mucinous adenocarcinoma (p=0.005), and larger tumor size (p=0.023) were significant predictors of high survival probability on the basis of a log-rank test. By use of the Bayesian model-averaging approach, higher Mayo stage and larger tumor size were significant predictors of cancer-specific mortality in urachal carcinoma. CONCLUSIONS: The Mayo staging system might be more effective than the Sheldon staging system. In addition, the multivariate analyses suggested that tumor size may be a prognostic factor for urachal carcinoma.

Clinical experiences of incidental prostate cancer after transurethral resection of prostate (TURP) according to initial treatment: a study of a Korean high volume center.

PURPOSE: These are the clinical experiences of Korean incidental prostate cancer patients detected by transurethral resection of the prostate according to initial treatment: active surveillance (AS), radical prostatectomy (RP) and hormone therapy (HT). MATERIALS AND METHODS: We retrospectively reviewed the records of 156 incidental prostate cancer patients between 2001 and 2012. The clinicopathologic outcomes were reviewed and follow-up results were obtained. RESULTS: Among 156 patients, 97 (62.2%) had T1a and 59 (37.8%) had T1b. Forty-six (29.5%) received AS, 67 (42.9%) underwent RP, 34 (21.8%) received HT, 4 (2.6%) received radiotherapy, and 5 (3.2%) chose watchful waiting. Of 46 patients on AS, prostate-specific antigen (PSA) progression occurred in 12 (26.1%) patients. Among them, 3 patients refused treatment despite PSA progression. Five patients, who underwent RP as an intervention, all had organ-confined Gleason score ≤6 disease. In 67 patients who underwent RP, 50 (74.6%) patients had insignificant prostate cancer and 8 (11.9%) patients showed unfavorable features. During follow-up, biochemical recurrence occurred in 2 patients. Among 34 patients who received HT, 3 (8.8%) patients had PSA progression. Among 156 patients, 6 patients died due to other causes during follow-up. There were no patients who died due to prostate cancer. CONCLUSION: The clinical outcomes of incidental prostate cancer were satisfactory regardless of the initial treatment. However, according to recent researches and guidelines, immediate definite therapy should be avoided without a careful assessment. We also believe that improved clinical staging is needed for these patients.

The vascular surgeon's experience with adrenal venous sampling for the diagnosis of primary hyperaldosteronism.

BACKGROUND: Adrenal venous sampling (AVS) is used to distinguish between bilateral idiopathic hyperplasia and a functional adrenal tumor in patients with hyperaldosteronism. Successful sampling from both adrenal veins is necessary for lateralization and may require more than 1 procedure. AVS has traditionally been performed by interventional radiologists; however, our goal was to examine the outcomes when performed by a vascular surgeon. METHODS: All patients with a diagnosis of hyperaldosteronism were referred for AVS regardless of imaging findings. Cortisol and aldosterone levels were measured in blood samples from both adrenal veins. Postoperative analysis of intraoperative laboratory values before and after cosyntropin administration determined successful cannulation and sampling of each vein. RESULTS: Between 2007 and 2012, 53 patients underwent AVS by one vascular surgeon. The average age was 54 and 63% were men. Our success rate increased with experience, because during the earlier years (2007-2010) primary and secondary success rates were 58% and 68%, respectively compared with later years (2011-2012) when primary and secondary success rates were 82% and 95%, respectively (P<0.05). Results of AVS altered localization of disease compared with what had been anticipated based on preoperative imaging and thus influenced surgical decision making in 47% of cases. CONCLUSIONS: AVS is an important procedure in the work up of hyperaldosteronism to help identify and localize metabolically active tumors. It is an additional area in medicine where a vascular surgeon can lend expertise. Success with the procedure improves with experience and should be performed by high volume surgeons.

Effect of race and insurance status on outcomes after vascular access placement for hemodialysis.

BACKGROUND: Race and insurance status are seen as potential barriers to health care access and maintenance. Our goal was to see how these, as well as other patient and procedural characteristics, affected our populations' upper extremity vascular access outcomes. METHODS: We retrospectively reviewed 601 vascular access patients from 2004 through 2012 in our urban university hospital. We recorded patient demographics, insurance status, comorbidities, and complications. Primary outcomes were reintervention, long-term mortality, and transplantation. RESULTS: Median age was 62 ± 15.8 years, and 58% were male. Most operations were arteriovenous fistulas (66%). The majority of patients identified themselves as Hispanic (50%), followed by white (22%), and black (19%). Most patients had Medicare only (42%), 31% had private insurance, and 27% had Medicaid as their insurance. Black/African American patients were more likely to receive an arteriovenous graft (AVG) compared with white and Hispanic patients (44% vs. 28% and 33%, P < 0.05). White patients were significantly older (68) than Hispanics (61) or blacks (58). Freedom from reintervention at 5 years was 55% with previous tunneled catheter use predictive. Mortality at 5 years was 35% and predicted by age, AVG placement, white race, and not receiving a kidney transplant. Predictors of not receiving a transplant included older age, lower albumin, AVG placement, and coronary artery disease. CONCLUSIONS: There were no disparities with insurance status in long-term outcomes in our population. Race was not a factor for reintervention or transplantation; however, black/African American patients were more likely have an AVG placed, and white patients had a lower long-term survival after access placement.

Two-stage hybrid repair of a complex symptomatic celiac aneurysm.

We report the case of a novel 2-stage hybrid repair of a complex celiac artery aneurysm. The patient was a 42-year-old man with a proximal celiac artery aneurysm giving rise to distinct right and left hepatic arterial branches. Repair was performed using a staged approach. First, a bifurcated aortohepatic bypass was constructed to the common and left hepatic arteries. After recovering from surgery, he underwent percutaneous embolization of the aneurysm. Completion angiograms demonstrated flow into all celiac branches with successful thrombosis of the aneurysm. At 12-month follow-up, the patient had remained symptom-free with patent bypass grafts and complete aneurysm exclusion. We describe the treatment option we used, which involves repair of a complex celiac aneurysm using a 2-stage, open, endovascular approach.

Dynamic diffuse optical tomography imaging of peripheral arterial disease.

Peripheral arterial disease (PAD) is the narrowing of arteries due to plaque accumulation in the vascular walls. This leads to insufficient blood supply to the extremities and can ultimately cause cell death. Currently available methods are ineffective in diagnosing PAD in patients with calcified arteries, such as those with diabetes. In this paper we investigate the potential of dynamic diffuse optical tomography (DDOT) as an alternative way to assess PAD in the lower extremities. DDOT is a non-invasive, non-ionizing imaging modality that uses near-infrared light to create spatio-temporal maps of oxy- and deoxy-hemoglobin in tissue. We present three case studies in which we used DDOT to visualize vascular perfusion of a healthy volunteer, a PAD patient and a diabetic PAD patient with calcified arteries. These preliminary results show significant differences in DDOT time-traces and images between all three cases, underscoring the potential of DDOT as a new diagnostic tool.

Phosphatase-defective LEOPARD syndrome mutations in PTPN11 gene have gain-of-function effects during Drosophila development.

Missense mutations in the PTPN11 gene, which encodes the protein tyrosine phosphatase SHP-2, cause clinically similar but distinctive disorders, LEOPARD (LS) and Noonan (NS) syndromes. The LS is an autosomal dominant disorder with pleomorphic developmental abnormalities including lentigines, cardiac defects, short stature and deafness. Biochemical analyses indicated that LS alleles engender loss-of-function (LOF) effects, while NS mutations result in gain-of-function (GOF). These biochemical findings lead to an enigma that how PTPN11 mutations with opposite effects on function result in disorders that are so similar. To study the developmental effects of the commonest LS PTPN11 alleles (Y279C and T468M), we generated LS transgenic fruitflies using corkscrew (csw), the Drosophila orthologue of PTPN11. Ubiquitous expression of the LS csw mutant alleles resulted in ectopic wing veins and, for the Y279C allele, rough eyes with increased R7 photoreceptor numbers. These were GOF phenotypes mediated by increased RAS/MAPK signaling and requiring the LS mutant's residual phosphatase activity. Our findings provide the first evidence that LS mutant alleles have GOF developmental effects despite reduced phosphatase activity, providing a rationale for how PTPN11 mutations with GOF and LOF produce similar but distinctive syndromes.

Transgenic Drosophila models of Noonan syndrome causing PTPN11 gain-of-function mutations.

Mutations in the PTPN11 gene, which encodes the protein tyrosine phosphatase SHP-2, causes Noonan syndrome (NS), an autosomal dominant disorder with pleomorphic developmental abnormalities. Certain germline and somatic PTPN11 mutations cause leukemias. Mutations have gain-of-function (GOF) effects with the commonest NS allele, N308D, being weaker than the leukemia-causing mutations. To study the effects of disease-associated PTPN11 alleles, we generated transgenic fruitflies with GAL4-inducible expression of wild-type or mutant csw, the Drosophila orthologue of PTPN11. All three transgenic mutant CSWs rescued a hypomorphic csw allele's eye phenotype, documenting activity. Ubiquitous expression of two strong csw mutant alleles were lethal, but did not perturb development from some CSW-dependent receptor tyrosine kinase pathways. Ubiquitous expression of the weaker N308D allele caused ectopic wing veins, identical to the EGFR GOF phenotype. Epistatic analyses established that csw(N308D)'s ectopic wing vein phenotype required intact EGF ligand and receptor, and that this transgene interacted genetically with Notch, DPP and JAK/STAT signaling. Expression of the mutant csw transgenes increased RAS-MAP kinase activation, which was necessary but not sufficient for transducing their phenotypes. The findings from these fly models provided hypotheses testable in mammalian models, in which these signaling cassettes are largely conserved. In addition, these fly models can be used for sensitized screens to identify novel interacting genes as well as for high-throughput screening of therapeutic compounds for NS and PTPN11-related cancers.