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In rare cases, giant cell tumor of bone (GCTB) can undergo primary or secondary malignant transformation to malignant giant cell tumor of bone (MGCTB), but the details of the molecular alterations are still unclear. The present study aimed to elucidate the clinicopathologic and molecular features of MGCTBs based on immunohistochemistry, fluorescence in situ hybridization (FISH) and next generation sequencing (NGS) of nine MGCTBs (five primary and four secondary). Seven (78%) of 9 MGCTBs were immunohistochemically positive for H3.3 G34W. In two (22%) patients, although GCTB components were focally or diffusely positive for H3.3 G34W, their malignant components were entirely negative for H3.3 G34W, which was associated with heterozygous loss of H3F3A by FISH. NGS on four MGCTBs revealed pathogenic mutations in TP53 (n = 3), EZH2 (n = 1) and several other genes. Immunohistochemical analysis of the nine MGCTBs confirmed the p53 nuclear accumulation (n = 5) and loss of H3K27me3 expression (n = 3) and showed that they were mutually exclusive. In addition, four (80%) of five cases of pleomorphic or epithelioid cell-predominant MGCTBs were positive for p53, while three (75%) of four cases of spindle cell-predominant MGCTBs were negative for trimethylation at lysine 27 of histone 3 (H3K27me3). The results suggested that p53 alteration and dysfunction of histone methylation as evidenced by H3K27me3 loss may play an important role in the malignant progression of GCTB, and might contribute to the phenotype-genotype correlation in MGCTB. The combined histologic, immunohistochemical and molecular information may be helpful in part for the diagnosis of challenging cases.

Synovial sarcoma (SS) is a malignant soft tissue neoplasm harboring SS18-SSX fusion gene and is histologically characterized by spindle cells and epithelial components. Some investigations have demonstrated that desmoplastic reaction (DR) is an independent prognostic factor of cancers. However, it remains unknown whether DR is of predictive value for the prognosis of synovial sarcoma patients. Here, we reviewed the clinical and histological findings of 88 patients with SS. We defined DR as hyalinized collagenous structures and classified the degree of DR as follows: none, mild, moderate, and severe. Overall, 23 SS cases (24%) showed moderate or severe DR histologically. Statistically, the cases with moderate or severe degree of DR showed poorer prognosis than those with no or mild DR (local recurrence: P = 0.0059, distant metastasis: P = 0.0002, tumor death: P = 0.0382). The findings of the study suggest that the DR of synovial sarcoma could be an important prognostic factor.

We describe a Case of a 74-year-old Japanese man with poorly differentiated carcinoma of the anterior mediastinum. The patient underwent anterior mediastinal tumor resection through median sternotomy. The tumor, 7.0 × 5.0 cm, had invaded surrounding tissues (pericardium, right lung, right and left brachiocephalic veins, and superior vena cava). Complete resection of the tumor was not performed. One month after the operation, the patient developed multiple pulmonary metastases, right pleural dissemination, and carcinomatous pleurisy. He was treated with lenvatinib, a novel multi-kinase inhibitor, to which the metastasis responded favorably. This case reports for the first time the clinical usefulness of lenvatinib for poorly differentiated carcinoma of the anterior mediastinum. Management of side effects by several methods, including suspending use of medication on weekends (called a weekends-off strategy), is another strong argument to continue lenvatinib administration.

New concept of a passive-safety simple fast reactor “METAL-KAMADO” with metallic fuels is presented, which has same concept as a passive-safety thermal reactor “KAMADO”. A fuel element of the “METAL-KAMADO” consists of metallic fuel (U–10%Zr) and cooling holes of He gas flow. These fuel elements are located in a reactor water pool of atmospheric pressure (0.1 MPa) and low temperature ( Preliminary neutronic calculations of the “METAL-KAMADO” show possibility of high burn-up of more than 120 GWd/t with 10% enriched U–Zr fuel. Reactivity coefficients of the core are also discussed.

In order to clarify the fragmentation mechanism of a metallic alloy (U–Pu–Zr) fuel on liquid phase formed by metallurgical reactions (liquefaction temperature = 650 °C), which is important in evaluating the sequence of core disruptive accidents for metallic fuel fast reactors, a series of experiments was carried out using molten aluminum (melting point = 660 °C) and sodium mainly under the condition that the boiling of sodium does not occur. When the instantaneous contact interface temperature ( T i ) between molten aluminum drop and sodium is lower than the boiling point of sodium ( T c,bp ), the molten aluminum drop can be fragmented and the mass median diameter ( D m ) of aluminum fragments becomes small with increasing T i . When T i is roughly equivalent to or higher than T c,bp , the fragmentation of aluminum drop is promoted by thermal interaction caused by the boiling of sodium on the surface of the drop. Furthermore, even under the condition that the boiling of sodium does not occur and the solid crust is formed on the surface of the drop, it is confirmed from an analytical evaluation that the thermal fragmentation of molten aluminum drop with solid crust has a potential to be caused by the transient pressurization within the melt confined by the crust. These results indicate the possibility that the metallic alloy fuel on liquid phase formed by the metallurgical reactions can be fragmented without occurring the boiling of sodium on the surface of the melt.

New concept of a passive-safety reactor “KAMADO” has a negligible possibility of core melting and flexibility of total reactor power. The reactor core of KAMADO consists of fuel elements of graphite blocks, which have UO2 fuel rods and cooling water holes. These fuel elements are located in a reactor water pool of atmospheric pressure (1 atm) and low temperature (< 60°C). In case of LOCA, decay heat from fuel rods is removed by conduction heat transfer to the reactor water pool. Since the cooling water does not contact a fuel rod directly, core design has much flexibility without considering dry-out limitation and Minimum Critical Power Ratio (MCPR). Additionally an effective use of spent fuel is expected.

CRIEPI and Toshiba Corp. have been exploring to realize a small-sized nuclear reactor for the needs of dispersed energy source and multi-purpose reactor. A conceptual design of 4S ( S uper- S afe, S mall and S imple) reactor is proposed to meet the following design requirements: (1) All temperature feedback reactivity coefficients including whole core sodium void reactivity are negative; (2) The core integrity is secured against all anticipated transient without reactor scram; (3) No emergency power nor active mitigating system is required; (4) The reactivity core lifetime is more than 10 years. The 4S reactor is a metallic fueled sodium cooled fast reactor. A target of an electrical output is 10–50 MW. A remarkable feature of 4S is that its reactivity is not controlled by neutron absorber rods but by neutron reflectors to cope with a long core lifetime and a negative coolant void reactivity. This study includes a design consideration of 4S. Design discussions are mainly focused on various core designs to meet above requirements. A tall core active height is adopted to gain long core lifetime. An averaged fuel burn-up is tried to be increased up to 100 GWd/ton from a point of economic view. The reference 4S designs are 10 MWe (30 years core lifetime) and 50 MWe (10 years core lifetime).

Effects of mitigation and suppression of vapor explosion have been investigated by adding a surfactant, polymer, and neutral salt into the water droplet impinging onto a molten alloy pool surface. This configuration was selected to attain good reproducibility and visibility, because premixing events prior to the triggering restrained. In addition, an adequate amount of a surfactant can be adsorbed at the vapor/liquid interface compared to that in the case of conventional configurations such as a molten alloy droplet injecting into a water pool. Dilute anionic and nonionic surfactant aqueous solutions did not affect the triggering conditions or the pressure pulse generated by vapor explosion, even if the surfactant was added up to a density 25 times higher than the critical micelle concentration. Polymeric additives, which increase the viscosity of a fluid, have little suppression effect on vapor explosion. Spontaneous vapor explosion was, however, suppressed by a 200 wppm polyethylene glycol (molecular weight of 4×10 6 ) solution, since deposition of the solute due to cloudy-point phenomenon may stabilize the vapor film and prevent the solution from mixing finely. In order to exert this effect, molecular weight should be heavier so that a cloudy-point temperature is below the boiling point at the tested system pressure. Additionally, this threshold concentration became denser as the impingement velocity increased. Thus, a denser concentration and heavier molecular weight should be used to suppress vapor explosion when the vapor film may be subjected to large inertia and/or external force. When a neutral salt was added, the initial molten alloy temperature range where vapor explosion occurred shifted to a higher temperature and became wider. Vapor explosion was observed on the molten zinc surface, which does not explode spontaneously for water.

The near-shore migration and settlement phases of Japanese and American flounders of the genus Paralichthys are compared and discussed relative to differences in coastal environments. Field sampling was conducted in Wakasa Bay, Kyoto Prefecture, Japan, where the shelf is narrow, estuarine habitat limited and tidal range slight, and in Onslow Bay, North Carolina, USA, where the shelf is broad, estuarine habitat extensive and tide relatively strong. Distribution of larvae and juveniles suggests Japanese flounder spawn in close proximity to nursery grounds relative to flounder in Onslow Bay. Sampling of planktonic and benthic flounder just seaward of nursery grounds resulted in capture of a wide range of developmental stages in Wakasa Bay (early planktonic to juvenile), but was limited to metamorphosing larvae in Onslow Bay. Vertical distribution of larvae during the night also differed between the Bays. At night most larvae in Wakasa Bay remained near the bottom regardless of tidal stage. In contrast, larvae in Onslow Bay exhibited selective tidal stream transport. Laboratory experiments conducted to examine the behaviour of recently captured wild and laboratory-reared larvae indicated that wild flounder from Onslow Bay had an endogenous rhythm of activity that corresponded to the tide at the time of capture. In contrast, wild flounder from Wakasa Bay and laboratory-reared larvae showed no distinct activity pattern. These results suggest that physical characteristics of the environment influence the early life history patterns of flounders by modifying behaviour of migrating larvae.

The total Nusselt number increases with decreases in: (i) the conduction-radiation ratio contribution and (ii) the number of intermediate plates.