Tracheal Chondrosarcoma-A Novel Presentation of Lynch Syndrome.

This case report describes a man in his 60s with no cancer history who presented with 2 weeks of dysphagia, dysphonia, and a left neck mass.

Treatment of ureteral anastomotic strictures with reimplantation and survival after cystectomy and urinary diversion.

OBJECTIVE: To examine whether long-term renal function and overall survival outcomes vary according to management approach for ureteral anastomotic stricture (UAS) after cystectomy and urinary diversion. METHODS: We conducted a retrospective cohort study of patients with benign UAS following cystectomy and urinary diversion using our institutional database. We compared time to stricture, renal function, rates of renal loss, and overall survival between patients undergoing ureteral reimplantation vs. those undergoing nonoperative management (nephrostomy tube or ureteral stent). A multivariable Cox proportional hazard model was used to determine whether reimplantation was independently associated with overall survival. RESULTS: We identified 87 UAS in 69 patients. Reimplantation was performed in 26 patients (37.7%), and 43 patients (62.3%) were managed nonoperatively. The interval between cystectomy and stricture diagnosis was similar in the reimplanted and nonoperative groups (3.06 vs. 4.34mo, P = 0.42). The differences between baseline and follow-up creatinine levels (+0.40 vs.+0.40mg/dl, P = 0.72) and estimated glomerular filtration rate (-25.0 vs.-18.9ml/min/1.73m2, P = 0.66) were similar between groups, as were rates of renal loss (34.6% vs. 39.5%, P = 0.68); however, mortality was significantly higher in the nonoperative group. After multivariable adjustment, overall survival remained significantly higher among UAS patients who underwent reimplantation (adjusted hazard ratio [aHR] for risk of death = 0.32, 95% CI: 0.13-0.80). CONCLUSION: Reimplantation was associated with improved overall survival but not with improved long-term renal functional outcomes compared with nonoperative management. Nonrenal complications of nonoperative UAS management may play an important role in reducing longevity.

Symptom burden and information needs in prostate cancer survivors: a case for tailored long-term survivorship care.

OBJECTIVES: To determine the relationship between long-term prostate cancer survivors' symptom burden and information needs. PATIENTS AND METHODS: We used population-based data from the Michigan Prostate Cancer Survivor Study (2499 men). We examined unadjusted differences in long-term information needs according to symptom burden and performed multivariable logistic regression to examine symptom burden and information needs adjusting for patient characteristics. RESULTS: High symptom burden was reported across all domains (sexual 44.4%, urinary 14.4%, vitality 12.7%, bowel 8.4%, emotional 7.6%) with over half of respondents (56%) reporting they needed more information. Top information needs involved recurrence, relationships, and long-term effects. Prostate cancer survivors with high symptom burden more often searched for information regardless of domain (P < 0.05). High sexual burden was associated with greater need for information about relationships [odds ratio (OR) 2.05, 95% confidence interval (CI) 1.54-2.72] and long-term effects (OR 1.60, 95% CI 1.23-2.07). High bowel burden was associated with greater information need for long-term effects (OR 2.28, 95% CI 1.43-3.63). CONCLUSIONS: Long-term prostate cancer survivors with high symptom burden need more supportive information. Tailoring information to these needs may be an efficient approach to support the growing population of long-term prostate cancer survivors.

Griscelli Syndrome Type 3: Two New Cases and Review of the Literature.

A 3-year-old Arab boy with a history of hypoplastic left heart syndrome was referred to the pediatric dermatology clinic at Sheba Medical Center for evaluation of hypomelanosis, manifested by fair skin pigmentation and silvery-grey hair, eyebrows, and eyelashes. The child had one older brother with similar hypopigmentation and another older brother who had died of congenital heart disease. The child had no history of neurologic deficits or immunodeficiency and no additional findings on clinical evaluation.

Segmental neurofibromatosis presenting with congenital excessive skin folds.

Segmental neurofibromatosis (SNF) is a rare type of neurofibromatosis (NF-1) resulting from post-zygotic somatic mutations in the neurofibromin gene that leads to mosaicism. Reported manifestations of SNF include neurofibromas, freckling, or café-au-lait spots limited to a single body region or limb. We present a 5-month-old male referred to our clinic for evaluation of congenital excessive skin folds on the back. A mildly erythematous, poorly demarcated soft plaque was noted, consisting of excessive skin folds. A cluster of light brown hyperpigmented macules was seen overlying the plaque. A punch biopsy of the plaque confirmed a diagnosis of neurofibroma. Further investigation ruled out other manifestations of NF-1. The early onset of our patient's neurofibroma and its gross appearance with redundant skin folds are all unusual features. To our knowledge, congenital excessive skin folds found in a single tumor have not been previously described in the literature as a manifestation of SNF. Clinicians should be educated about the possibility of congenital localized skin folds in association with SNF in order to identify the disease in infancy and monitor any changes in neurofibroma pathology.

Understanding hospital readmission intensity after radical cystectomy.

PURPOSE: Hospital readmissions after radical cystectomy vary with respect to intensity in terms of impact on patients and health care systems. Therefore, we conducted a population based study to examine factors associated with increasing readmission intensity after radical cystectomy for bladder cancer. MATERIALS AND METHODS: Using SEER (Surveillance, Epidemiology, and End Results)-Medicare data we identified 1,782 patients who underwent radical cystectomy from 2003 to 2009. We defined readmission intensity in terms of length of stay (days) divided into quartiles of less than 3 (lowest), 3 to 4, 5 to 7 and more than 7 (highest). We used logistic regression to examine factors associated with readmission intensity. RESULTS: More than half of the patients with the highest intensity readmissions were readmitted within the first week and 77% were readmitted within 2 weeks of discharge. Patients with the highest intensity readmissions were similar in age, gender, race, socioeconomic status, pathological stage, comorbidity, neoadjuvant chemotherapy use and urinary diversion type compared to patients with the lowest intensity readmissions. After multivariable adjustment, complications during the index cystectomy admission (p <0.001), readmission week (p=0.04), and the interaction between index length of stay and discharge to a skilled nursing facility (p=0.04) were associated with the highest readmission intensity. CONCLUSIONS: Readmission intensity differs widely after discharge following radical cystectomy. As postoperative efforts to minimize the readmission burden increase, a better understanding of the factors that contribute to the highest intensity readmissions will help direct limited resources (eg telephone calls, office visits) toward high yield areas.

Increased concentration of circulating angiogenesis and nitric oxide inhibitors induces endothelial to mesenchymal transition and myocardial fibrosis in patients with chronic kidney disease.

BACKGROUND: Sudden cardiovascular death is increased in chronic kidney disease (CKD). Experimental CKD models suggest that angiogenesis and nitric oxide (NO) inhibitors induce myocardial fibrosis and microvascular dropout thereby facilitating arrhythmogenesis. We undertook this study to characterize associations of CKD with human myocardial pathology, NO-related circulating angiogenesis inhibitors, and endothelial cell behavior. METHODS: We compared heart (n=54) and serum (n=162) samples from individuals with and without CKD, and assessed effects of serum on human coronary artery endothelial cells (HCAECs) in vitro. Left ventricular fibrosis and capillary density were quantified in post-mortem samples. Endothelial to mesenchymal transition (EndMT) was assessed by immunostaining of post-mortem samples and RNA expression in heart tissue obtained during cardiac surgery. Circulating asymmetric dimethylarginine (ADMA), endostatin (END), angiopoietin-2 (ANG), and thrombospondin-2 (TSP) were measured, and the effect of these factors and of subject serum on proliferation, apoptosis, and EndMT of HCAEC was analyzed. RESULTS: Cardiac fibrosis increased 12% and 77% in stage 3-4 CKD and ESRD and microvascular density decreased 12% and 16% vs. preserved renal function. EndMT-derived fibroblast proportion was 17% higher in stage 3-4 CKD and ESRD (P trend = 0.02). ADMA, ANG, TSP, and END concentrations increased in CKD. Both individual factors and CKD serum increased HCAEC apoptosis (P=0.02), decreased proliferation (P=0.03), and induced EndMT. CONCLUSIONS: CKD is associated with an increase in circulating angiogenesis and NO inhibitors, which impact proliferation and apoptosis of cardiac endothelial cells and promote EndMT, leading to cardiac fibrosis and capillary rarefaction. These processes may play key roles in CKD-associated CV disease.

Circulating endoglin concentration is not elevated in chronic kidney disease.

BACKGROUND: Soluble endoglin, a TGF-ß receptor, plays a key role in cardiovascular physiology. Whether circulating concentrations of soluble endoglin are elevated in CKD or underlie the high risk of cardiovascular death associated with chronic kidney disease (CKD) is unknown. METHODS: Individuals with and without CKD were recruited at a single center. Estimated glomerular filtration rate (eGFR) was estimated using the modified MDRD study equation and the serum creatinine at the time of recruitment, and patients were assigned to specific CKD stage according to usual guidelines. Serum endoglin concentration was measured by ELISA and univariate and multivariable regression was used to analyze the association between eGFR or CKD stage and the concentration of soluble endoglin. RESULTS: Serum endoglin was measured in 216 patients including 118 with stage 3 or higher CKD and 9 individuals with end stage renal disease (ESRD). Serum endoglin concentration did not vary significantly with CKD stage (increase of 0.16 ng/mL per 1 stage increase in CKD, P = 0.09) or eGFR (decrease -0.06 ng/mL per 10 mL/min/1.73 m(2) increase in GFR, P = 0.12), and was not higher in individuals with ESRD than in individuals with preserved renal function (4.2±1.1 and 4.3±1.2 ng/mL, respectively). Endoglin concentration was also not significantly associated with urinary albumin excretion. CONCLUSIONS: Renal function is not associated with the circulating concentration of soluble endoglin. Elevations in soluble endoglin concentration are unlikely to contribute to the progression of CKD or the predisposition of individuals with CKD to develop cardiovascular disease.