Solitary fibrous tumor of the breast: A case report.

INTRODUCTION: Solitary fibrous tumors (SFTs) are uncommon mesenchymal neoplasms that comprise <2 % of all soft tissue tumors. They are a diagnostically challenging group of neoplasms that can occur essentially anywhere. Molecular or genetic testing of soft tissue tumors will increasingly add to the foundation of distinctive histologic features, as accurate diagnosis is critical for appropriate treatment. CASE PRESENTATION: A 28-year-old woman was referred to our hospital for a left breast mass. Ultrasonography showed an oval hypoechoic mass with partially obscured boundaries. Surgical specimens revealed spindle tumor cells surrounding the mammary ducts and were immunoreactive for both CD34 and STAT6, suggesting SFTs. However, the infiltration of spindle tumor cells into the surrounding fat, and the storiform-like pattern made us consider dermatofibrosarcoma protuberans (DFSP) as a differential diagnosis. Lack of amplification of the COL1A1-PDGFB fusion gene, a characteristic feature of DFSP, led to our definitive diagnosis of breast SFT. DISCUSSION: The presence of STAT6 in tumor cell nuclei is a highly sensitive immunohistochemical marker for SFT. In our case, morphological features evoked the differential diagnosis of DFSP and we investigated the COL1A1-PDGFB fusion gene. The diagnostic process of reliably performing careful morphological examination and immunohistochemical marker test, and then obtain conviction by molecular cytogenetic technique is more and more important for soft tissue tumors. CONCLUSIONS: We report a quite uncommon case of breast SFT and excluded DFSP as a differential diagnosis. If it is difficult to distinguish between these diseases, molecular cytogenetic analysis would be required for accurate diagnosis.

Mutation of PTPN11 (Encoding SHP-2) Promotes MEK Activation and Malignant Progression in Neurofibromin-Deficient Cells in a Manner Sensitive to BRAP Mutation.

Germline mutations of NF1 cause neurofibromatosis type 1 (NF1) through the activation of the RAS signaling pathway, and some NF1 patients develop malignant peripheral nerve sheath tumors (MPNSTs). Here, we established subclones of the human NF1-MPNST cell line sNF96.2 that manifest increased tumorigenic activity and increased phosphorylation of the protein kinases MEK and Akt relative to the parental cells. Genomic DNA sequencing identified 14 additional heterozygous mutations within the coding regions of 13 cancer- and other disease-related genes in these subclones. One of these genes, PTPN11, encodes SHP-2, and the forced expression of the identified G503V mutant of SHP-2 increased both tumorigenic activity and MEK phosphorylation in parental sNF96.2 cells, suggesting that the combination of PTPN11 and NF1 mutations induces the pathological activation of the RAS pathway. These effects of SHP-2 (G503V) were inhibited by the coexpression of the G370A mutant of BRAP, which was also detected in the highly malignant subclones, and this inhibition was accompanied by the calpain-dependent cleavage of SHP-2 (G503V). The cleavage of SHP-2 (G503V) and suppression of MEK phosphorylation mediated by BRAP (G370A) were not detected in NF1-intact (HeLa) cells. Tumor promotion by SHP-2 (G503V) and its suppression by BRAP (G370A) may serve as a basis for the development of new treatment strategies for NF1.

Tranilast inhibits the expression of genes related to epithelial-mesenchymal transition and angiogenesis in neurofibromin-deficient cells.

Neurofibromatosis type 1 (NF1) is caused by germline mutations in the NF1 gene and is characterized by café au lait spots and benign tumours known as neurofibromas. NF1 encodes the tumour suppressor protein neurofibromin, which negatively regulates the small GTPase Ras, with the constitutive activation of Ras signalling resulting from NF1 mutations being thought to underlie neurofibroma development. We previously showed that knockdown of neurofibromin triggers epithelial-mesenchymal transition (EMT) signalling and that such signalling is activated in NF1-associated neurofibromas. With the use of a cell-based drug screening assay, we have now identified the antiallergy drug tranilast (N-(3,4-dimethoxycinnamoyl) anthranilic acid) as an inhibitor of EMT and found that it attenuated the expression of mesenchymal markers and angiogenesis-related genes in NF1-mutated sNF96.2 cells and in neurofibroma cells from NF1 patients. Tranilast also suppressed the proliferation of neurofibromin-deficient cells in vitro more effectively than it did that of intact cells. In addition, tranilast inhibited sNF96.2 cell migration and proliferation in vivo. Knockdown of type III collagen (COL3A1) also suppressed the proliferation of neurofibroma cells, whereas expression of COL3A1 and SOX2 was increased in tranilast-resistant cells, suggesting that COL3A1 and the transcription factor SOX2 might contribute to the development of tranilast resistance.

Ultraviolet imager on Venus orbiter Akatsuki and its initial results.

The ultraviolet imager (UVI) has been developed for the Akatsuki spacecraft (Venus Climate Orbiter mission). The UVI takes ultraviolet (UV) images of the solar radiation reflected by the Venusian clouds with narrow bandpass filters centered at the 283 and 365 nm wavelengths. There are absorption bands of SO2 and unknown absorbers in these wavelength regions. The UV images provide the spatial distribution of SO2 and the unknown absorber around cloud top altitudes. The images also allow us to understand the cloud top morphologies and haze properties. Nominal sequential images with 2-h intervals are used to understand the dynamics of the Venusian atmosphere by estimating the wind vectors at the cloud top altitude, as well as the mass transportation of UV absorbers. The UVI is equipped with off-axial catadioptric optics, two bandpass filters, a diffuser installed in a filter wheel moving with a step motor, and a high sensitivity charge-coupled device with UV coating. The UVI images have spatial resolutions ranging from 200 m to 86 km at sub-spacecraft points. The UVI has been kept in good condition during the extended interplanetary cruise by carefully designed operations that have maintained its temperature maintenance and avoided solar radiation damage. The images have signal-to-noise ratios of over 100 after onboard desmear processing.

Venus looks different from day to night across wavelengths: morphology from Akatsuki multispectral images.

Since insertion into orbit on December 7, 2015, the Akatsuki orbiter has returned global images of Venus from its four imaging cameras at eleven discrete wavelengths from ultraviolet (283 and 365 nm) and near infrared (0.9-2.3 µm), to the thermal infrared (8-12 µm) from a near-equatorial orbit. The Venus Express and Pioneer Venus Orbiter missions have also monitored the planet for long periods but from polar or near-polar orbits. The wavelength coverage and views of the planet also differ for all three missions. In reflected light, the images reveal features seen near the cloud tops (~ 70 km altitude), whereas in the near-infrared images of the nightside, features seen are at mid- to lower cloud levels (~ 48-60 km altitude). The dayside cloud cover imaged at the ultraviolet wavelengths shows morphologies similar to what was observed from Mariner 10, Pioneer Venus, Galileo, Venus Express and MESSENGER. The daytime images at 0.9 and 2.02 µm also reveal some interesting features which bear similarity to the ultraviolet images. The nighttime images at 1.74, 2.26 and 2.32 µm and at 8-12 µm reveal features not seen before and show new details of the nightside including narrow wavy ribbons, curved string-like features, long-scale waves, long dark streaks, isolated bright spots, sharp boundaries and even mesoscale vortices. Some features previously seen such as circum-equatorial belts (CEBs) and occasional areal brightenings at ultraviolet (seen in Venus Express observations) of the cloud cover at ultraviolet wavelengths have not been observed thus far. Evidence for the hemispheric vortex organization of the global circulation can be seen at all wavelengths on the day- and nightsides. Akatsuki images reveal new and puzzling morphology of the complex nightside cloud cover. The cloud morphologies provide some clues to the processes occurring in the atmosphere and are thus, a key diagnostic tool when quantitative dynamical analysis is not feasible due to insufficient information.

Initiation of a lightning search using the lightning and airglow camera onboard the Venus orbiter Akatsuki.

The existence of lightning discharges in the Venus atmosphere has been controversial for more than 30 years, with many positive and negative reports published. The lightning and airglow camera (LAC) onboard the Venus orbiter, Akatsuki, was designed to observe the light curve of possible flashes at a sufficiently high sampling rate to discriminate lightning from other sources and can thereby perform a more definitive search for optical emissions. Akatsuki arrived at Venus during December 2016, 5 years following its launch. The initial operations of LAC through November 2016 have included a progressive increase in the high voltage applied to the avalanche photodiode detector. LAC began lightning survey observations in December 2016. It was confirmed that the operational high voltage was achieved and that the triggering system functions correctly. LAC lightning search observations are planned to continue for several years.

[Perioperative Rehabilitation for Patients with Esophageal Cancer].

Transthoracic esophagectomy for patients with esophageal cancer is one of the most invasive of all gastrointestinal surgery. Postoperative management and surgical techniques are improved. However, many patients are more elderly or suffering from comorbid diseases or in malnutrition status. In order to prevent for postoperative complications after esophagectomy, especially for respiratory complication and swallowing dysfunction, adequate rehabilitation approaches are necessary depending on the various conditions.

[Ultrasound-guided rectus sheath block for upper abdominal surgery].

Upper abdominal surgery leads to severe postoperative pain. Insufficient postoperative analgesia accompanies a high incidence of complications. Therefore, postoperative analgesia is very important. The epidural analgesia has many advantages. However it has a high risk of epidural hematoma in anticoagulated patients. Rectus sheath block provided safer and more reliable analgesia in recent years, by the development of ultrasound tools. We experienced two cases of the rectus sheath block in upper abdominal surgery under ultrasound guidance. Ultrasound guided rectus sheath block can reduce the risk of peritoneal puncture, bleeding, and other complications. Rectus sheath block is very effective to reduce postoperative pain in upper abdominal surgery as an alternative method to epidural anesthesia in anticoagulated patients.