Synchronous extraskeletal Ewing's sarcoma/PNET and gallbladder carcinoma : a case report and literature review

Ewing’s sarcoma (ES) and primitive neuroectodermal tumour (PNET) are now considered to be the same tumour and usually occur in long bones. Extraskeletal Ewing’s sarcoma is an extremely rare neoplasm, accounting for 1% of soft tissue sarcomas, with most common location in the thorax. Gallbladder cancer (GBC) represents the most common type among the biliary tract cancers with a poor prognosis even among patients undergoing aggressive therapy. We present study of extraskeletal ES/PNET found in the hilus of the liver of an elderly, diagnosed one month prior with GBC woman. The patient underwent two cycles of chemotherapy SAIME/SAVAC for ES and thereafter was operated. During three-year follow-up no recurrence of ES/PNET has been reported. However, two years after chemotherapy the patient suffered a relapse of adenocarcinoma of the gallbladder and thus received palliative chemotherapy of gemcitabine and cisplatin. After 16 months of recurrence she died. To the best of our knowledge, this is the first case of ES/PNET located in the hilus of the liver and as a synchronous neoplasm

Einzelnukleotid-Variationen in Genen der DNA-Reparaturmechanismen bei Sézary Syndrom

Sézary Syndrom ist ein seltenes kutanes Lymphom, das durch eine Erythrodermie und leukämische Ausschwemmung von malignen T-Zellen gekennzeichnet ist. Die Pathogenese dieser Erkrankung bleibt trotz zahlreicher Studien unklar. Bis jetzt wurden keine spezifischen Mutationen beschrieben. Es wird vermutet, dass Genveränderungen von verschiedenen Mechanismen im Endeffekt zur malignen T-Zell-Transformation und damit Entwicklung der Erkrankung führen. Im Rahmen dieser Arbeit wurden Genome von 8 an Sézary Syndrom erkrankten Patienten auf Einzelnukleotid-Variationen (SNVs) bei 130 Genen, die vor allem an DNA-Reparaturmechanismen, des Weiteren Zellzyklus-Kontrolle und epigenetische Regulation beteiligt sind, untersucht. SNVs wurden bei 44 Genen (33,8%) gefunden. Die Mehrheit von diesen waren bekannte (62,8%), Missense- (97,7%) und a. e. heterozygote (86,3%) Variationen. Zwischen den Patienten bestand ein statistisch signifikanter Unterschied in Bezug auf bekannte/unbekannte sowie homozygote/heterozygote SNVs. Bei BRCA1, PRKDC, RTEL1 wurden jeweils 6 (maximale Anzahl) SNVs pro Gen festgestellt. Bei 6 von 8 (75%) Patienten wurden die Veränderungen bei BRCA1, CSB, EXO1, WRN und XRCC6BP1 gefunden. Am häufigsten waren Gene der homologen Rekombination (47%) betroffen. Ein statistisch signifikanter Unterschied in Bezug auf die Häufigkeit von betroffenen DNA-Reparaturwegen bestand allerdings nicht. Am häufigsten traten jedoch SNVs bei Genen, die in die Reparatur von DNA-Doppelstrangbrüchen involviert sind, auf. Dazu gehört außer der homologen Rekombination non-homologous und microhomology-mediated end joining (NHEJ, MMEJ). Diese Mechanismen spielen eine wesentliche Rolle bei der Aufrechterhaltung der genomischen Stabilität, deren Störung das charakteristische molekulare Merkmal des Sézary Syndroms darstellt. Eine genaue Differenzierung der Patienten anhand der genetischen Veränderungen und somit betroffenen Signalwegen kann zukünftig bei der Wahl der besten Therapie hilfreich sein.Sézary syndrome (SS) is a rare cutaneous lymphoma of the skin with erythroderma and blood involvement. Despite numerous studies the etiology of this disease remains unclear. Genetic alterations involving multiple pathways are suspected to play a role in the malignant tranformation of T-cells and the development of SS. In this study, genomes of 8 patients with SS were searched for single nucleotide variations (SNVs) in 130 genes, which in particular are involved in DNA repair mechanisms, control of the cell cycle and epigenetic modifications. SNVs were detected in 44 genes (33,8%); most of them were known (62,8%), missense (97,7%) and probably heterozygote (86,3%). There was a statistically significant difference between the patients corncering the number of unknown/known and homo-/heterozygote variations. A maximal number of SNVs (6) was found in BRCA1, PRKDC and RTEL1. Variations in BRCA1, CSB, EXO1, WRN and XRCC6BP1 were detected at 6 of 8 patients (75%). Genes of the homologous recombination were most often affected (47%). However there was no statistically relevant difference in the frequency of involved genes and the DNA repair pathways. The variations in the genes of the DNA double-strand repair play a major role in genome maintenance, disturbances of which are a typical feature of SS. Further research on the pathogenesis of this disease would facilitate development of novel therapeutic targets

A gas ejector for CO2 supercritical cycles

he CO2 ejectors are recently often used as the main expansion device in the modern refrigeration cycles. On the other hand, according to the newest literature the implementation the ejectors into supercritical CO2 power cycles increase its performance. The recent studies showed that in case of the power cycles the ejector pressure lift and mass entrainment ratio are relatively high. Therefore, the main scope of this paper is the investigation of the possibilities of designing the ejector for supercritical Brayton CO2 system. The CFD based computational tool was used to design the ejector for the considered cycle. The system analysis was used to define the ejector on design point. The results of that analysis showed that the required pressure lift and must be equal to 103 bar and mass entrainment ratio equal to 0.995, respectively. The CFD-based evaluation of the proposed ejector showed that these values are impossible to achieve. Therefore, the modifications of the crucial ejector dimensions was performed to increase its performance. Nevertheless, the maximum possible pressure lift for the proposed ejector was equal to 60 bar The analysis of the gathered results showed that the design of the ejector fulfilling the system requirements may be impossible to achieve. © 2018 Elsevier Ltd. All rights reserved.A gas ejector for CO2 supercritical cyclesacceptedVersio

Combustion performance evaluation of Posidonia oceanica using TGA and bubbling fluidized-bed combustor (batch reactor)

Combustion performance of emerging raw marine biomass called Posidonia oceanica (PO) was investigated using TGA apparatus and a bubbling fluidized-bed batch reactor. The kinetic mechanism and parameters of the combustion process were determined. The Flynn-Wall-Ozawa (FWO) method and data fitting method were analyzed. It was observed that a model based on consecutive processes: devolatilisation and char combustion for two fractions of PO (holocellulose and lignin), is the best model for the analyzed cases. Combustion performance was observed using a BFB reactor and the composition of flue gas after combustion was analyzed, and the conversion of NOx and SO2 was taken into account. The relatively low SO2 emission in the case of PO combustion can be attributed to the impact of the sulphur self-retention (SSR) process. The results were compared to the combustion of wood biomass and Turow lignite. The results showed the good combustion performance of PO