Citations

ABSTRACT Objectives Albumin messenger RNA (mRNA) expression is a marker of hepatocellular differentiation. Most published data are from review of tissue microarrays, and albumin in situ hybridization (ISH) expression across several tumor types is incompletely characterized. Methods Sections from 221 tumors were evaluated for albumin mRNA. Immunohistochemistry was used to confirm diagnoses. Albumin ISH was performed according to manufacturer-provided instructions. Fifty-nine cases were evaluated with both commercial ISH assays. Results Albumin mRNA was detected in all hepatocellular carcinomas (HCCs) and 81% of intrahepatic cholangiocarcinomas. Lung (20%), gallbladder (39%), hepatoid pancreatic (n = 1 of 1) adenocarcinoma, breast invasive ductal carcinoma (18%), yolk sac tumor (25%), and acinar cell carcinoma (29%) showed expression. Both assays were concordant in 93% of cases. Conclusions Albumin ISH was expressed in all HCCs studied. It was also positive in intrahepatic cholangiocarcinoma and patchy positive in gallbladder adenocarcinoma and a subset of other neoplasms, which can be a potential pitfall.

Abstract OBJECTIVES Limited literature is available on the best management strategy for tricuspid valve (TV) disease in adults. We sought to review our long-term outcomes of TV surgery with regard to survival and reoperation. METHODS A retrospective analysis of all patients ≥18 years of age [n = 2541, aged 67 ± 13years, 1433 (56%) females] who underwent first-time TV surgery between January 1993 and December 2013 was done. There were 1735 patients who received TV repair and 806 patients underwent replacement. A gradient boosting machine model was used to derive a propensity score for predicting replacement using 27 preoperative characteristics. Four hundred and eighteen propensity-matched pairs of TV repair and replacement were identified. Cox proportional hazard regression was used on the matched subset to determine the effect of replacement. RESULTS Functional TV regurgitation was present in 54% (n = 1369). A bioprosthesis was used in 84% (n = 680) of replacements, while 54% (n = 934) of TV repairs were ring annuloplasties. Operative mortality was 8% (n = 212). Overall survival was 54%, 29% and 13% at 5, 10 and 15 years, respectively. After propensity score matching, replacement was significantly associated with increased mortality [hazard ratio (HR) 1.54, 95% confidence interval (CI) 1.18–2.00; P = 0.001]. The cumulative incidence of TV reoperation was similar between the 2 groups when accounting for the competing risk of death [Fine–Gray HR 1.56, 95% CI 0.9–2.8; P = 0.144]. CONCLUSIONS TV surgery is associated with poor outcomes due to multiple patient comorbidities. TV repair results in better survival compared to replacement in patients with similar comorbidities with no increased risk of getting a reoperation.

Background. Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) is a distinct form of glomerulonephritis that often recurs after kidney transplantation causing severe graft injury and often failure. Methods. We describe post transplant outcomes and response to therapy in 20 recipients with PGNMID. Evidence of PGNMID recurrence or lack thereof was determined by protocol and clinical biopsies. Results. Histologic recurrence (deposition of monoclonal immunoglobulin) occurred in 18 of 20 recipients (90%), a median of 7 (1 to 65) months post transplant. At diagnosis, recurrence was generally associated with mild or no clinical manifestations and often with mild glomerular morphologic changes by light microcopy. Four of the 18 patients with recurrence did not progress and were not treated. Another 4 patients with recurrences were treated with cyclophosphamide with or without plasmapheresis, and 2 of these grafts were lost from glomerulonephritis. Nine patients with recurrences were treated with anti-CD20 antibodies (rituximab) alone, resulting in improvements in estimated glomerular filtration rate (31.5 ± 16 versus 38.8 ± 13.3 mL/min/1.73 m 2 , P = 0.011) and proteinuria (1280 [117 to 3752] versus 168 [83 to 1613] mg/24 h, P = 0.012) although complete clinical remission was rare. One graft in this later group was lost from recurrence 141 months post transplant. Posttreatment biopsies demonstrated stable or improved glomerular histology in most cases. However, PGNMID did not resolve in any case. Four patients received rituximab 4 months pretransplant to prevent recurrence. However, 3 had mild recurrences. Conclusions. Rituximab treatment of early PGNMID recurrence is effective, resulting in reasonable, long-term graft survival. Whether pretransplant rituximab modifies the course of recurrence requires additional studies.

Background Aortic dilation and aortic valve disease are known long-term complication of tetralogy of Fallot (TOF), but the risk of aortic dissection and the indications for prophylactic aortic surgery are unknown in this population. The purpose of this study was to: (1) determine the prevalence of significant aortic valve disease and/or significant aortic aneurysm (AVD-AA); (2) determine the incidence of progressive aortic dilation and aortic dissection in patients with TOF. Methods Retrospective review of adults with repaired TOF, and no prior aortic valve/aorta surgery, who had ≥2 measurements of the thoracic aorta >12 months apart, 1990–2017. The aortic root and mid-ascending aorta were measured at the onset of QRS complex from leading edge to leading edge. Significant aortic valve disease was defined as the presence of ≥moderate aortic stenosis and/or ≥moderate aortic regurgitation. Significant aortic aneurysm was defined as aortic root or mid-ascending aorta dimension ≥50 mm. Progressive aortic dilation was defined as increase in aortic dimension ≥2 mm. Results Of the 453 consecutive patients (37±13 years, men 216 (49%)) in the study, aortic aneurysm was present in 312 (69%) based on normative data; progressive aortic dilation occurred in 40 (9%), and there was no case of aortic dissection. Significant AVD-AA occurred in 52 (12%) patients; and 15 of them (29%) underwent aortic surgery without any surgical mortality. Conclusions Although aortic aneurysm was common, progressive aortic dilation was uncommon and aortic dissection did not occur in our patients with TOF with significant aortic aneurysms who did not undergo aortic surgery. This has important clinical implication in deciding the frequency of imaging follow-up and timing of surgical intervention in this population.

Background Automated individualized risk prediction tools linked to electronic health records ( EHR s) are not available for management of patients with peripheral arterial disease. The goal of this study was to create a prognostic tool for patients with peripheral arterial disease using data elements automatically extracted from an EHR to enable real‐time and individualized risk prediction at the point of care. Methods and Results A previously validated phenotyping algorithm was deployed to an EHR linked to the Rochester Epidemiology Project to identify peripheral arterial disease cases from Olmsted County, MN, for the years 1998 to 2011. The study cohort was composed of 1676 patients: 593 patients died over 5‐year follow‐up. The c‐statistic for survival in the overall data set was 0.76 (95% confidence interval [CI], 0.74–0.78), and the c‐statistic across 10 cross‐validation data sets was 0.75 (95% CI, 0.73–0.77). Stratification of cases demonstrated increasing mortality risk by subgroup (low: hazard ratio, 0.35 [95% CI, 0.21–0.58]; intermediate‐high: hazard ratio, 2.98 [95% CI, 2.37–3.74]; high: hazard ratio, 8.44 [95% CI, 6.66–10.70], all P <0.0001 versus the reference subgroup). An equation for risk calculation was derived from Cox model parameters and β estimates. Big data infrastructure enabled deployment of the real‐time risk calculator to the point of care via the EHR . Conclusions This study demonstrates that electronic tools can be deployed to EHR s to create automated real‐time risk calculators to predict survival of patients with peripheral arterial disease. Moreover, the prognostic model developed may be translated to patient care as an automated and individualized real‐time risk calculator deployed at the point of care.

Key PointsDUSP22-rearranged ALCLs belong to a distinct subset of ALCLs lacking activated STAT3. DUSP22-rearranged ALCLs have a unique molecular signature characterized by DNA hypomethylation and an immunogenic phenotype.

Abstract Background Pneumatosis intestinalis has long been a marker of advanced bowel ischemia and prompts urgent laparotomy. In post-operative settings, the presence of pneumatosis represents a significant management dilemma. We share a case-series of post-esophagectomy patients with pneumatosis intestinalis and no corollary intra-abdominal pathologic findings on re-exploration. Methods January 2000 to December 2017, 1760 patients underwent Ivor-Lewis esophagectomy or gastrectomy with jejunostomy-tube placement. Charts were reviewed retrospectively to identify patients with pneumatosis intestinalis discovered in the post-operative period. Demographic data, operative details and postoperative course were reviewed including incidence and details of re-exploration. Results Eleven patients met inclusion criteria. Nine were male (81.8%) and mean age was 69 years. All patients had radiographic confirmation of pneumatosis intestinalis and in many cases portal venous gas (Figure 1). Clinical course was variable without discernable trends in vitals or laboratory values. Development of significant postoperative ileus along with delivery of enteral tube feeds through a jejunostomy tube preceded development of the pneumatosis in all patients. Nine patients were re-explored and none had evidence of bowel ischemia. Conclusion The finding of pneumatosis intestinalis in the post-operative setting can be alarming and pose a management dilemma. With the advent of improved and readily available imaging, there has been an increase in findings that have no corollary physical symptomatology. In this series of patients, despite dramatic radiographic findings, none had ischemic bowel. Pneumatosis intestinalis alone in patients who have undergone esophagectomy should not be considered an indication for emergency re-exploration. Disclosure All authors have declared no conflicts of interest.