Tenosynovial giant cell tumor (TSGCT) is a rare neoplasm. Although surgical resection is the widely accepted primary treatment for TSGCT, recurrences are frequent, and patients’ joint function may be severely compromised. Previous studies reported that CSF1‐COL6A3 fusion genes were identified in approximately 30% of TSGCTs. The aim of our study was to comprehensively clarify the genomic abnormalities in TSGCTs. We performed whole exome sequencing in combination with target sequence validation on 34 TSGCT samples. RNA sequencing was also performed on 18 samples. RNA sequencing revealed fusion transcripts involving CSF1, including novel CSF1‐VCAM1, CSF1‐FN1 and CSF1‐CDH1 fusions, in 13/18 (72%) cases. These fusion genes were validated by chromogenic in situ hybridization. All CSF1 fusions resulted in the deletion of CSF1 exon 9, which was previously shown to be an important negative regulator of CSF1 expression. We also found that 12 (35%) of the 34 TSGCT samples harbored CBL missense mutations. All mutations were detected in exons 8 or 9, which encode the linker and RING finger domain. Among these mutations, C404Y, L380P and R420Q were recurrent. CBL‐mutated cases showed higher JAK2 expression than wild‐type CBL cases (p = 0.013). CSF1 fusion genes and CBL mutations were not mutually exclusive, and both alterations were detected in six of the 18 (33%) tumors. The frequent deletion of CSF1 exon 9 in the fusion transcripts suggested the importance of this event in the etiology of TSGCT. Our results may contribute to the development of new targeted therapies using JAK2 inhibitors for CBL‐mutated TSGCT.

No abstract

No abstract

A space vehicle which undergoes the atmospheric re-entry or a planetary entry needs the heat shield system to protect inner equipments against severe aerodynamic heating environments. Charring ablator is usually used for the heat shield system. In order to design the heat shield system, it is necessary to predict the thermal behavior under aerodynamic heating by ablation analysis. A computer code for charring ablation and thermal response analysis is newly developed for simulation of one-dimensional transient thermal behavior of charring ablation materials. The mathematical model for the charring ablation including basic equation and computational method of ablation analysis is briefly described. A new ultra light weight phenolic carbon ablator called LATS (Lightweight Ablator series for Transfer vehicle) was recently developed. Arc-heated tests of the LATS ablator were carried out and measured results of the temperature response and surface mass loss are compared with the simulation results of the ablation analysis program. The agreement between the results of simulation and measurement is found to be good. It is also found that the mathematical model used in the ablation code can be applied to the ablation analysis of the low density LATS ablator.

No abstract