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Extraskeletal Ewing's sarcoma is an unusual primary tumor of the deep soft tissues. Clinically, skin lesions are usually fastgrowing and aggressive, with a risk of metastasis, and are more common in the paravertebral region and lower limbs. The ultimate diagnosis is made by anatomopathological study. This report describes the case of a 21-year-old patient with a tumor on her right flank that had been present for the past 2 months. She underwent surgery in an emergency department and progressively became more ill. [ABSTRACT FROM AUTHOR]

AIM: Although anti-vascular endothelial growth factor (VEGF) receptor tyrosine kinase inhibitors (RTKIs) have been tested in patients with neuroendocrine tumours (NETs) over the last two decades, no study to date has benchmarked efficacy and toxicity of these drugs in this patient population. METHODS: All phase II and phase III studies of anti-VEGF RTKIs in patients with NETs, published between January 1, 2000 andJuly 31, 2021, across major trial databases, were searched in August 2021 for relevant studies. The primary objectives of the meta-analysis were to compare objective response rate (ORR) and progression-free survival (PFS) between patients with pancreatic NETs (pNETs) and extra-pancreatic NETs (epNETs), and the incidence rate ratio (IRR) of adverse events between patients receiving anti-VEGF RTKIs and control. RESULTS: 1611 patients were available for the meta-analysis; 1194 received anti-VEGF RTKIs. ORR in pNETs was 18% (95% confidence interval (CI) 13-25%), while ORR in epNETs was 8% (95% CI 5-12%); test for differences between pNETs and epNETs (x12 = 8.38, p

Pediatric central nervous system (CNS) tumors represent the most common cause of cancer-related death in children aged 0-14 years. They differ from their adult counterparts, showing extensive clinical and molecular heterogeneity as well as a challenging histopathological spectrum that often impairs accurate diagnosis. Here, we use DNA methylation-based CNS tumor classification in combination with copy number, RNA-seq, and ChIP-seq analysis to characterize a newly identified CNS tumor type. In addition, we report histology, patient characteristics, and survival data in this tumor type. We describe a biologically distinct pediatric CNS tumor type (n = 31 cases) that is characterized by focal high-level amplification and resultant overexpression of either PLAGL1 or PLAGL2, and an absence of recurrent genetic alterations characteristic of other pediatric CNS tumor types. Both genes act as transcription factors for a regulatory subset of imprinted genes (IGs), components of the Wnt/β-Catenin pathway, and the potential drug targets RET and CYP2W1, which are also specifically overexpressed in this tumor type. A derived PLAGL-specific gene expression signature indicates dysregulation of imprinting control and differentiation/development. These tumors occurred throughout the neuroaxis including the cerebral hemispheres, cerebellum, and brainstem, and were predominantly composed of primitive embryonal-like cells lacking robust expression of markers of glial or neuronal differentiation (e.g., GFAP, OLIG2, and synaptophysin). Tumors with PLAGL1 amplification were typically diagnosed during adolescence (median age 10.5 years), whereas those with PLAGL2 amplification were diagnosed during early childhood (median age 2 years). The 10-year overall survival was 66% for PLAGL1-amplified tumors, 25% for PLAGL2-amplified tumors, 18% for male patients, and 82% for female patients. In summary, we describe a new type of biologically distinct CNS tumor characterized by PLAGL1/2 amplification that occurs predominantly in infants and toddlers (PLAGL2) or adolescents (PLAGL1) which we consider best classified as a CNS embryonal tumor and which is associated with intermediate survival. The cell of origin and optimal treatment strategies remain to be defined.

This was a two-stage phase II trial of a mTORC1/2 inhibitor (mTORC: mammalian target of rapamycin complex) Sapanisertib (TAK228) in patients with rapalog-resistant pancreatic neuroendocrine tumors (PNETs) (NCT02893930). Approved rapalogs such as everolimus inhibit mTORC1 and have limited clinical activity, possibly due to compensatory feedback loops. Sapanisertib addresses the potential for incomplete inhibition of the mTOR pathway through targeting of both mTORC1 and mTORC2, and thus to reverse resistance to earlier rapamycin analogues. In stage 1, patients received sapanisertib 3 mg by mouth once daily on a continuous dosing schedule in 28-day cycle. This trial adopted a two-stage design with the primary objective of evaluating objective tumor response. The first stage would recruit 13 patients in order to accrue 12 eligible and treated patients. If among the 12 eligible patients at least 1 patient had an objective response to therapy, the study would move to the second stage of accrual where 25 eligible and treated patients would be enrolled. This study activated on February 1, 2017, the required pre-determined number of patients (n = 13) had entered by November 5, 2018 for the first stage response evaluation. The accrual of this trial was formally terminated on December 27, 2019 as no response had been observed after the first stage accrual. Treatment-related grade 3 adverse events were reported in eight (61%) patients with hyperglycemia being the most frequent, in three patients (23%). Other toxicities noted in the trial included fatigue, rash diarrhea, nausea, and vomiting. The median PFS was 5.19 months (95% CI [3.84, 9.30]) and the median OS was 20.44 months (95% CI [5.65, 22.54]). Due to the lack of responses in Stage 1 of the study, the study did not proceed to stage 2. Thus the potential to reverse resistance was not evident.

Primitive neuroectodermal tumors (PNETs) are rare and aggressive members of the small round cell carcinoma family. Generally, PNETs are classified into two main groups: PNETs of the central nervous system and PNETs of the peripheral nervous system. Herein, we report the therapy response assessment of a rare case of isolated cardiac PNET using 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) imaging. Given that physiological cardiac FDG uptake is typically observed, assessing FDG avid lesions in the myocardium presents a challenge for [ABSTRACT FROM AUTHOR]

Methylation profiling has radically transformed our understanding of tumors previously called central nervous system primitive neuro-ectodermal tumors (CNS-PNET). While this marks a momentous step toward defining key differences, reclassification has thrown treatment into disarray. To shed light on response to therapy and guide clinical decision-making, we report outcomes and molecular features of children with CNS-PNETs from two multi-center risk-adapted studies (SJMB03 for patients ≥ 3 years; SJYC07 for patients

PurposeEwing sarcoma (ES) is a primitive sarcoma defined by EWSR1-ETS fusions as the primary driver alteration. To better define the landscape of cooperating secondary genetic alterations in ES, we analyzed clinical genomic profiling data of 113 patients with ES, a cohort including more adult patients (> 18 years) and more patients with advanced stage at presentation than previous genomic cohorts.MethodsThe data set consisted of patients with ES prospectively tested with the US Food and Drug Administration-cleared Memorial Sloan Kettering-Integrated Mutation Profiling of Actionable Cancer Targets large panel, hybrid capture-based next-generation sequencing assay. To assess the functional significance of ERF loss, we generated ES cell lines with increased expression of ERF and lines with knockdown of ERF. We assessed cell viability, clonogenic growth, and motility in these ES lines and performed transcriptomic and epigenetic analyses. Finally, we validated our findings in vivo using cell line xenografts.ResultsNovel subsets were defined by recurrent secondary alterations in ERF, which encodes an ETS domain transcriptional repressor, in 7% of patients (five truncating mutations, one deep deletion, and two missense mutations) and in FGFR1 in another 2.7% (one amplification and two known activating mutations). ERF alterations were nonoverlapping with STAG2 alterations. In vitro, increased expression of ERF decreased tumor cell growth, colony formation, and motility in two ES cell lines, whereas ERF loss induced cellular proliferation and clonogenic growth. Transcriptomic analysis of cell lines with ERF loss revealed an increased expression of genes and pathways associated with aggressive tumor biology, and epigenetic, chromatin-based studies revealed that ERF competes with EWSR1-FLI1 at ETS-binding sites.ConclusionOur findings open avenues to new insights into ES pathobiology and to novel therapeutic approaches in a subset of patients with ES.

The remarkable skill of changing its grasp status and relocating its fingers to perform continuous in-hand manipulation is essential for a multifingered anthropomorphic hand. A commonly utilized method of manipulation involves a series of basic movements executed by a high-level controller. However, it remains unclear how these primitives evolve into sophisticated finger gaits during manipulation. Here, we propose an adaptive finger gait-based manipulation method that offers real-time regulation by dynamically changing the primitive interval to ensure the force/moment balance of the object. Successful manipulation relies on contact events that act as triggers for real-time online replanning of multifinger manipulation. We identify four basic motion primitives of finger gaits and create a heuristic finger gait that enables the continuous object rotation of a round cup. Our experimental results verify the effectiveness of the proposed method. Despite the constant breaking and reengaging of contact between the fingers and the object during manipulation, the robotic hand can reliably manipulate the object without failure. Even when the object is subjected to interfering forces, the proposed method demonstrates robustness in managing interference. This work has great potential for application to the dexterous operation of anthropomorphic multifingered hands.

Glioblastoma IDH-wildtype presents with a wide histological spectrum. Some features are so distinctive that they are considered as separate histological variants or patterns for the purpose of classification. However, these usually lack defined (epi-)genetic alterations or profiles correlating with this histology. Here, we describe a molecular subtype with overlap to the unique histological pattern of glioblastoma with primitive neuronal component. Our cohort consists of 63 IDH-wildtype glioblastomas that harbor a characteristic DNA methylation profile. Median age at diagnosis was 59.5 years. Copy-number variations and genetic sequencing revealed frequent alterations in TP53, RB1 and PTEN, with fewer gains of chromosome 7 and homozygous CDKN2A/B deletions than usually described for IDH-wildtype glioblastoma. Gains of chromosome 1 were detected in more than half of the cases. A poorly differentiated phenotype with frequent absence of GFAP expression, high proliferation index and strong staining for p53 and TTF1 often caused misleading histological classification as carcinoma metastasis or primitive neuroectodermal tumor. Clinically, many patients presented with leptomeningeal dissemination and spinal metastasis. Outcome was poor with a median overall survival of only 12 months. Overall, we describe a new molecular subtype of IDH-wildtype glioblastoma with a distinct histological appearance and genetic signature.

In adult mammals, spermatogenesis embodies the complex developmental process from spermatogonial stem cells (SSCs) to spermatozoa. At the top of this developmental hierarchy lie a series of SSC subpopulations. Their individual identities as well as the relationships with each other, however, remain largely elusive. Using single-cell analysis and lineage tracing, we discovered both in mice and humans the quiescent adult SSC subpopulation marked specifically by forkhead box protein C2 (FOXC2). All spermatogenic progenies can be derived from FOXC2+ SSCs and the ablation of FOXC2+ SSCs led to the depletion of the undifferentiated spermatogonia pool. During germline regeneration, FOXC2+ SSCs were activated and able to completely restore the process. Germ cell-specific Foxc2 knockout resulted in an accelerated exhaustion of SSCs and eventually led to male infertility. Furthermore, FOXC2 prompts the expressions of negative regulators of cell cycle thereby ensures the SSCs reside in quiescence. Thus, this work proposes that the quiescent FOXC2+ SSCs are essential for maintaining the homeostasis and regeneration of spermatogenesis in adult mammals.

Suppose that a finite solvable group G acts faithfully, irreducibly and quasi-primitively on a finite vector space V , and G is not metacyclic. Then G always has a regular orbit on V except for a few "small" cases. We completely classify these cases in this paper. [ABSTRACT FROM AUTHOR]

In this work, we investigate the differential geometric characteristics of pedal and primitive curves in a Minkowski plane. A primitive is specified by the opposite structure for creating the pedal, and primitivoids are known as comparatives of the primitive of a plane curve. We inspect the relevance between primitivoids and pedals of plane curves that relate with symmetry properties. Furthermore, under the viewpoint of symmetry, we expand these notions to the frontal curves in the Minkowski plane. Then, we present the relationships and properties of the frontal curves in this category. Numerical examples are presented here in support of our main results.

The porous and cellular architecture of scaffolds plays a significant role in mechanical strength and bone regeneration during the healing of fractured bones. In this present study, triply periodic minimal surface (TPMS)‐based gyroid and primitive lattice structures were used to design the cellular porous biomimetic scaffolds with different unit cell sizes (4, 5, and 6). The fused filament fabrication‐based 3D printing technology was used for the fabrication of polylactic acid scaffolds. The surface morphology and mechanical compressive strength of differently structured scaffolds were observed using scanning electron microscopy and a universal testing machine. The unit cell size of 4 showed higher compressive strength in both gyroid and primitive structured scaffolds compared to unit cell sizes 5 and 6. Moreover, the gyroid structured scaffolds have higher compressive strengths as compared to primitive structured scaffolds due to the higher bonding surface area at the intercalated layers of the scaffold. Hence, the mechanical strength of scaffolds can be tailored by varying the unit cell size and cellular structures to avoid stress shielding and ensure implant safety. These TPMS‐based scaffolds are promising and can be used as bone substitute materials in tissue engineering and orthopedic applications. [ABSTRACT FROM AUTHOR]

In the present study, the trend of divergence and fragmentation of different sections of Astragalus in the Old World has been investigated. Based on previous studies, Astragalus species are divided into three groups: primitive, intermediate, and advanced. The distribution maps of each of these groups in the Old World were presented in the study. The primitive group consists mainly of herbaceous sections with simple hairs and an old drought-tolerant section with woody stems and bifurcating hairs that are densely distributed in Asia and Africa and scattered in Europe. The intermediate group, with thorny cushion shapes, is often scattered in the Zagros and Alborz mountains, mountainous regions of Turkey, Lebanon, and Jordan, and extend from the east to Afghanistan, the Hindu Kush, and the southern parts of the Himalayas. The results of the study show that the distribution pattern of the advanced group is very heterogeneous and its pattern is consistent with the primitive group. The simple hair sections are often scattered below 35 degrees north latitude and the bifurcated hairs sections are more frequently distributed above 35 degrees north latitude. Annual Astragalus species are heterogeneously distributed in all three main groups. In all three groups, parallel evolution has taken place at the level of sections and species. At each point of distribution, their number has a limited distribution. In addition, some species have achieved non-genetic diversity by functional or regulatory genetic, hereditary or structural mechanisms and created distinct species that have adapted in nature to the ecological conditions of the same areas and continue to regenerate with even more distributions.

Human behaviors, with whole-body coordination, involve large-scale sensorimotor interaction. Spontaneous bodily movements in the early developmental stage potentially lead toward acquisition of such coordinated behavior. These movements presumably contribute to the structuration of sensorimotor interaction, providing specific regularities in bidirectional information among muscle activities and proprioception. Whether and how spontaneous movements, despite being task-free, structure and organize sensorimotor interactions in the entire body during early development remain unknown. Herein, to address these issues, we gained insights into the structuration process of the sensorimotor interaction in neonates and 3-mo-old infants. By combining detailed motion capture and musculoskeletal simulation, sensorimotor information flows among muscle activities and proprioception throughout the body were obtained. Subsequently, we extracted spatial modules and temporal state in sensorimotor information flows. Our approach demonstrated that early spontaneous movements elicited body-dependent sensorimotor modules, revealing age-related changes in them, depending on the combination or direction. The sensorimotor interactions also displayed temporal non-random fluctuations analogous to those seen in spontaneous activities in the cerebral cortex and spinal cord. Furthermore, we found recurring state sequence patterns across multiple participants, characterized by a substantial increase in infants compared to the patterns in neonates. Therefore, early spontaneous movements induce the spatiotemporal structuration in sensorimotor interactions and subsequent developmental changes. These results implicated that early open-ended movements, emerging from a certain neural substrate, regulate the sensorimotor interactions through embodiment and contribute to subsequent coordinated behaviors. Our findings also provide a conceptual linkage between early spontaneous movements and spontaneous neuronal activity in terms of spatiotemporal characteristics. [ABSTRACT FROM AUTHOR]

Perivascular epithelioid cell tumours (PEComas) are a growing family of tumours composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. Cutaneous primitive PEComas (cPEComas) are very rare, with 65 cases described in the English literature, and occur as a painless lesion predominantly in female patients, with a wide age range. We present a new case of cPEComa found on the left thigh of a 53-year-old patient with histopathological, immunohistochemical, and molecular information. The lesion was positive for HMB-45 and focal for smooth muscle actin and desmin but negative for melan-A, S-100 protein, CD31, and CD34. Next generation sequencing (NGS) analysis demonstrated the presence of genomic aberration for baculoviral IAP repeats containing BIRC3 splice site 1622-27_1631del37. Although there are little molecular data regarding this entity, our case adds to this knowledge, considering the importance of detecting genomic aberrations in the context of specific therapies such as mTOR inhibitors. [ABSTRACT FROM AUTHOR]

Ljubomir Micić was the founder of Zenitism and the editor of Zenit, the international avant-garde magazine published in Zagreb and Belgrade from 1921 to 1926. Sharply criticising a decadent European culture after the Great War and accepting progressive avant-garde ideas, Micić praised the New art founded on the principles of NEO-primitivism and Russian constructivism, following the technological and scientific progress of the 20th century. Analysing the Zenitist Balkanisation of Europe project led by Barbarogenius, I will point out to Micić's attitude towards the old continent, his efforts to oppose the image of the Balkans as "the inner other of Europe", and his aspiration to revive European culture with the primordial Balkan energy. Declaring the Balkans the sixth continent, the geographical space of the poets, Micić postulated a model of cultural barbarism by which he stood against the established primacy of Western European nations over those that were characterised "less civilised". This paper aims to point out to Micić's understanding of the relationship between the Balkans and Europe, as well as the so-called primitive-civilised opposition, in order to highlight internationalism, pacifism and cosmopolitanism as the key elements of his Zenitosophy

The problem description language (PDL) has been developed. The main architectural features of the PDL language are considered. The advantages of using PDL are discussed. The rules for creating descriptions in PDL are described. A universal software configurator for the Web server security software package is considered. Here is an example of testing PDL using a translator that converts the task description to the executable Perl code. The structure of the PDL task description translator in Perl (analysis phase and synthesis phase) is given. The features of this translator at the synthesis stage [13-15, 24, 26, 27] are described. There are 3 modes of operation at the synthesis phase: 1. If the task description does not specify any of the action operators (a special class of operators in the PDL). 2. If the description contains at least one action operator and you need to search the algorithm in the database. 3. If the required module is not found in the algorithm bank under the same conditions as in point 2. In the first case, primitives are used. The second one searches for modules in the algorithm bank. In the 3rd case, the required module must be partially written by the programmer himself. The procedure for generating a web server security software package in Perl using the PDL task description is considered. Examples are given and the reasons for reducing the number of program errors when using the PDL translator in Perl are described.

Copyright of ZARCH: Journal of Interdisciplinary Studies in Architecture & Urbanism is the property of Universidad de Zaragoza and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Como ciencia de la cultura, la historia del arte ha estudiado el fenómeno sobre la inclinación por lo primitivo y su influencia en las artes visuales del siglo XX. Este fenómeno también puede entenderse paralelamente desde la historia y teoría de la arquitectura al explicar las características de determinados movimientos que surgieron como reacción a la estandarización de la arquitectura moderna. En este texto se reflexiona sobre el caso del neobrutalismo, movimiento propio del periodo de la segunda posguerra en Inglaterra. El artículo se desarrolla reconstruyendo la gestación del mismo, exponiendo cómo lo primitivo fue interpretado a través de la arquitectura de Alison y Peter Smithson y recordando la posición sobre la imagen memorable del crítico Reyner Banham. Esta lectura permite explicar el síntoma de algo más profundo desde los significados culturales, sugiriendo el neobrutalismo y su relación con lo primitivo como el inicio de la posmodernidad, en la que la imagen arquitectónica se posiciona como un nuevo principio.

In this work I ponder about the archive based on three images from the late nineteenth and early twentieth centuries: the drawing of a monster from the Teratology collection of the Museo Nacional de México preserved today in the Biblioteca Nacional de Antropología e Historia; the photography of animals that are part of what was the collection of the Museo de Historia Natural del Chopo and that today protects the Fototeca Nacional; and the copy of a drawing of a hysterical woman that is part of the iconography of the French hospital de la Salpêtrière and that was reproduced in Mexico in a scientific journal. With the perspective offered by the “archival turn”, these individual images speak about the procedures of classification, hierarchy and exclusion that organized the knowledge system. They also show three ways of articulating madness, the bestial and the primitive, three figures of the Other from the West, three paths that lead to the “Indian”, that descendant of the pre-Hispanic inhabitants of the American continent on whose erasure was erected the scientific normalizing discourse and the nation-state.

The ontology of time and space has always been a subject of materialist prospectus bearing a halo effect of 'modernity' and 'progress'. The enquiry into the sign of modern is a mechanical category of production where substantial copies of 'progress' have religiously been equated with a break from the past. This breaking away from the centre (soul) is, of course, associated with a desire for the non-native design. Simultaneously, the past becomes historicized as primitive dangers while the present/'modern' morphs into a non-past spectacular diffusion. Satyajit Ray reloads his artillery of the cerebral one last time in his masterpiece titled, Agantuk (The Stranger), where he pits the idea of a spectral past having an agency to redo the class binary against the totalitarian time(s) in a modern urban space which prides itself on the abuse of power-as-civility. Ray introduces a nuclear family of three (a married couple and their son) where the protagonist, Manmohan Mitra, returns as an archived data in the body of a forgotten relative. His entry into the house ruptures the canny knots of the 'home' where the director exposes limits of the modernized time. This paper tries to analyze how Ray uses the motif of 'travel' in its cinematic cloth to critique the ingestion of global progress as nothing but an accumulation of fallen spectacles that commodify both a subject who is consuming the object-in-time (progress) and also the object that is all the time getting alienated from its own subjective merit. Mitra becomes the mouthpiece of the director for conveying the paradoxes of time-as-capital in the burgeoning of speculative modernity. [ABSTRACT FROM AUTHOR]

Introduction: Primary bronchial cancer in women is a major public health problem; its incidence has increased steadily over the past 20 years. Objective: The objective of this work was to analyze the epidemiological, clinical, therapeutic and prognostic characteristics of lung cancer in women in the region of Sidi Bel Abbés. Materials and methods: A descriptive retrospective study of 50 women diagnosed with primary lung cancer collected in the oncology and radiotherapy department of anti cancer centre between January 2008 and May 2019. Results: The average age of our patients was 64 years (±15,063). Respiratory symptoms were dominated by chest pain. The main risk factors were passive smoking (26% of cases), family history of cancer, hormonal factors, environmental factors, history of respiratory diseases. The most common histological type was adenocarcinoma (74% of cases), stages III and IV accounted for 95% of cases. The main metastatic locations were bone and cervical (23.8%). Treatment management was based mainly on chemotherapy (50% of cases), of which 19% were palliative. Discussion: Our results confirm that bronchial cancer in women is steadily increasing. Its risk factors are different from those of men. The dominant histological type in women was adenocarcinoma, which has a better prognosis, however, the diagnosis remains late, which explains the predominance of stages III and IV in women. Conclusion: The female primary broncho-pulmonary cancer has anatomopathological, hormonal and genetic peculiarities that characterize it, which should encourage us to apprehend it differently from the therapeutic strategies better for women. [ABSTRACT FROM AUTHOR]

Three histological variants are known within the family of embryonal rosette-forming neuroepithelial brain tumors. These include embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma (EBL), and medulloepithelioma (MEPL). In this study, we performed a comprehensive clinical, pathological, and molecular analysis of 97 cases of these rare brain neoplasms, including genome-wide DNA methylation and copy number profiling of 41 tumors. We identified uniform molecular signatures in all tumors irrespective of histological patterns, indicating that ETANTR, EBL, and MEPL comprise a single biological entity. As such, future WHO classification schemes should consider lumping these variants into a single diagnostic category, such as embryonal tumor with multilayered rosettes (ETMR). We recommend combined LIN28A immunohistochemistry and FISH analysis of the 19q13.42 locus for molecular diagnosis of this tumor category. Recognition of this distinct pediatric brain tumor entity based on the fact that the three histological variants are molecularly and clinically uniform will help to distinguish ETMR from other embryonal CNS tumors and to better understand the biology of these highly aggressive and therapy-resistant pediatric CNS malignancies, possibly leading to alternate treatment strategies.

Perivascular epithelioid cell tumours (PEComas) are a growing family of tumours composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. Cutaneous primitive PEComas (cPEComas) are very rare, with 65 cases described in the English literature, and occur as a painless lesion predominantly in female patients, with a wide age range. We present a new case of cPEComa found on the left thigh of a 53-year-old patient with histopathological, immunohistochemical, and molecular information. The lesion was positive for HMB-45 and focal for smooth muscle actin and desmin but negative for melan-A, S-100 protein, CD31, and CD34. Next generation sequencing (NGS) analysis demonstrated the presence of genomic aberration for baculoviral IAP repeats containing BIRC3 splice site 1622-27_1631del37. Although there are little molecular data regarding this entity, our case adds to this knowledge, considering the importance of detecting genomic aberrations in the context of specific therapies such as mTOR inhibitors.

Primitive neuroectodermal tumors (PNETs) are rare and aggressive members of the small round cell carcinoma family. Generally, PNETs are classified into two main groups: PNETs of the central nervous system and PNETs of the peripheral nervous system. Herein, we report the therapy response assessment of a rare case of isolated cardiac PNET using 18 F-fluorodeoxyglucose ( 18 F-FDG) positron emission tomography/computed tomography (PET/CT) imaging. Given that physiological cardiac FDG uptake is typically observed, assessing FDG avid lesions in the myocardium presents a challenge for FDG PET/CT. This case holds significance because of the rarity of the disease and the challenging nature of the site for FDG PET/CT imaging., Competing Interests: Conflict of Interest: No conflicts of interest were declared by the authors., (Copyright© 2024 The Author. Published by Galenos Publishing House on behalf of the Turkish Society of Nuclear Medicine.)

Copyright of Electronic Turkish Studies is the property of Electronic Turkish Studies and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Embryonal tumor with multilayered rosettes (ETMR, previously known as ETANTR) is a highly aggressive embryonal CNS tumor, which almost exclusively affects infants and is associated with a dismal prognosis. Accurate diagnosis is of critical clinical importance because of its poor response to current treatment protocols and its distinct biology. Amplification of the miRNA cluster at 19q13.42 has been identified previously as a genetic hallmark for ETMR, but an immunohistochemistry-based assay for clinical routine diagnostics [such as INI-1 for atypical teratoid rhabdoid tumor (AT/RT)] is still lacking. In this study, we screened for an ETMR-specific marker using a gene-expression profiling dataset of more than 1,400 brain tumors and identified LIN28A as a highly specific marker for ETMR. The encoded protein binds small RNA and has been implicated in stem cell pluripotency, metabolism and tumorigenesis. Using an LIN28A specific antibody, we carried out immunohistochemical analysis of LIN28A in more than 800 childhood brain-tumor samples and confirmed its high specificity for ETMR. Strong LIN28A immunoexpression was found in all 37 ETMR samples tested, whereas focal reactivity was only present in a small (6/50) proportion of AT/RT samples. All other pediatric brain tumors were completely LIN28A-negative. In summary, we established LIN28A immunohistochemistry as a highly sensitive and specific, rapid, inexpensive diagnostic tool for routine pathological verification of ETMR.

Book Review: Modern Production to Imagined Primitive: The Social World of Coffee from Papua New Guinea

Une femme de 29 ans, a consulté pour une tuméfaction de la région parotidienne gauche légèrement sensible avec limitation de l´ouverture de la bouche évoluant depuis 3 mois. Son anamnèse ne retrouve pas notion de chirurgie ou d´extraction dentaire. L´examen clinique retrouve une tuméfaction de la région parotidienne gauche ferme, légèrement sensible mal limité sans signes inflammatoires en regard, l´examen endobucal ne retrouve pas de foyer infectieux dentaire ou d´écoulement purulent du canal de Sténon. La tomodensitométrie a montré un remaniement osseux de la face externe de la branche montante de la mandibule, une image d´ostéolyse mal limitée sous condylienne gauche, des séquestres osseux et un épaississement des parties molles et muscles autour de la branche en question. Une biopsie osseuse a montré des remaniements inflammatoires chroniques en rapport avec une ostéomyélite chronique primitive sans germe identifiable ni signes de malignité. La patiente a bénéficié d´un traitement antibiotique à base de pénicilline et un traitement chirurgical de décortication et de séquestréctomie. L´évolution était favorable. L´ostéomyélite primitive de la mandibule est une affection rare d´étiologie pas claire, elle est dite chronique quand l´évolution dépasse 1mois. Elle se distingue de l´ostéomyélite secondaire par l´absence de pus ou de fistule. Les données radiologiques comprennent l´ostéolyse, l´ostéosclérose et parfois l´hypertrophie des muscles masséters. L´histologie est essentielle pour éliminer des diagnostics différentiels de malignité. Le traitement est basé principalement sur les antibiotiques et la décortication, parfois on peut associer les corticostéroïdes, et l´oxygénothérapie hyperbare.

L´appendagite épiploïque primitive est une cause rare d´abdomen aigue. Elle peut simuler le tableau clinique d´autre processus inflammatoire tels que la diverticulite ou l´appendicite. Le diagnostic repose sur le scanner. Le traitement est médical en dehors des complications.

В статье рассматривается творческое наследие ученицы Иркутской художественной мастерской-студии И.Л. Копылова Антонины Емельяновой. Она принимала активное участие в культурной жизни Иркутска 1920-х, была отмечена своим учителем, а также художественными критиками как перспективный график и один из представителей «сибирского стиля» в искусстве. Предпринята попытка обобщить разрозненный материал о художнице, встречающийся в искусствоведческой литературе, дополнить его новыми сведениями, полученными в процессе работы с источниками: документами, хранящимися в архиве Иркутского областного художественного музея имени В.П. Сукачева, публикациями в периодике 1920-х. Одна из задач исследования – ответить на вопрос, что есть «сибирское» в искусстве для А. Емельяновой.

Ewing's sarcomas/primitive neuroectodermal tumors (PNET) are a group of tumors characterized by small round cells that rarely affect the bone and soft tissues of the nasal cavities. Herein, we describe the case of a 38-year-old man who presented with a one-year history of epistaxis, headache, hyposmia, anosmia, night sweats, weight loss, hearing loss, dysphagia, and nasal blockage. A computed tomography (CT) scan and magnetic resonance imaging (MRI) studies revealed a lesion in the posterior nasal cavities with extension into the sphenoid and ethmoid sinuses. Histological examination of a biopsied specimen obtained via flexible fiberoptic endoscopy showed small dark round cells with scanty cytoplasm arranged in solid nests and pseudorosette formations. The diagnosis of Ewing's sarcoma was confirmed by the positive reactive pattern of the neoplastic cells for CD99 by immunohistochemistry studies and also identification of a chromosomal translocation of FLI1 in PCR studies. Cervical lymph nodes and bone marrow involvement were also observed. The patient was treated by adjuvant chemotherapy including vincristine, endoxan, and adriamycin for 6 cycles followed by radiotherapy (eleven courses). Unfortunately, before completion of treatment, the patient died due to disease progression and brain metastasis.

Epiploic appendagitis is a rare cause of acute abdomen. It can mimic other inflammatory processes such as diverticulitis or appendicitis. Diagnosis is based on CT scan. Medical management is the approach of choice in the absence of complications. [ABSTRACT FROM AUTHOR]

Ewing's sarcomas are a less common malignant neoplasms that can be located both in skeletal and extra skeletal areas. The primitive neuroectodermic tumor (PNET) shows more neuronal differentiation than Ewing's sarcoma and rarely invades the skin and soft tissues. This tumor occasionally presents as a primary skin cancer which has distinctive features such as a superficial tumor with a 2-3 cm, soft, mobile and eventually painful. Most affected areas of the body are paravertebral spaces, lower limbs, head, neck and pelvis. We report here the case of a 13 year old male presenting with 2 superficial tumors located on the scalp, the histo pathological examination revealed a proliferation of small, basophilic and monomorphic cells that showed FLI1 positive immunohistochemistry pattern compatible with PNET. [ABSTRACT FROM AUTHOR]

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Peripheral primitive neuroectodermal tumors (pPNETs) are rare, small cell carcinomas with a poor prognosis. The aim of the present study was to describe therapeutic approaches, outcomes and probable prognostic factors. The clinical features, treatments, and outcomes of 89 consecutive patients with pPNET treated at the Peking Union Medical College Hospital from 1999 to 2018 were retrospectively reviewed. A total of 43 males and 46 females were included in the study, with a median age of 25 years (range, 5–73 years). The predominantly affected regions were the abdomen and pelvis, followed by the thoracopulmonary region. The mean primary tumor size was 12.6 cm (range, 1–30 cm). A total of 16 patients (18%) initially presented with metastasis. A total of 46 patients received combined therapy, 35 received mono-therapy, and 8 underwent only biopsy with no further treatment. The period of observation ranged from 1–232 months. The median overall survival (OS) time was 15 months [95% confidence interval (CI), 9–21 months], with 3- and 5-year OS rates of 32 and 25%, respectively. Large tumor size [adjusted hazard ratio (aHR)=3.65; 95% CI, 2.07–6.42; P<0.001), metastasis at initial presentation (aHR=4.34, 95% CI, 2.23–8.42; P<0.001), and combined modality treatment (aHR=0.16, 95% CI, 0.06–0.39; P<0.001) were significantly associated with OS. The prognosis of pPNET is, overall, poor. Large tumor size and metastasis at initial presentation are associated with poorer outcomes. This highly malignant tumor requires an aggressive combination of radical resection, chemotherapy and radiotherapy, when indicated. [ABSTRACT FROM AUTHOR]

Objective: To describe the clinical and epidemiological characteristics, and to determine prognostic factors, event-free survival (EFS) and overall survival (OS) of patients with Ewing sarcoma family tumors (ESFT). Materials and methods: A retrospective study conducted in patients under 18 years of age with ESFT, treated at the Unit of Pediatric and Adolescent Oncology of the Hospital Edgardo Rebagliati between January 2006 and June 2016. The descriptive analysis was performed by frequency distribution. Kaplan-Meier curves were used for EFS and OS analysis. Univariate and multivariate analyses were performed according to the Cox regression model for demographic, clinical and surgical variables, and prognostic factors. The measure of strength of association was expressed in hazard ratio (HR) and 95% confidence interval (95% CI), and p

Background: Functional upper extremity (UE) motion enables humans to execute activities of daily living (ADLs). There currently exists no universal language to systematically characterize this type of motion or its fundamental building blocks, called functional primitives. Without a standardized classification approach, pooling mechanistic knowledge and unpacking rehabilitation content will remain challenging.Methods: We created a taxonomy to characterize functional UE motions occurring during ADLs, classifying them by motion presence, temporal cyclicity, upper body effector, and contact type. We identified five functional primitives by their phenotype and purpose: reach, reposition, transport, stabilize, and idle. The taxonomy was assessed for its validity and interrater reliability in right-paretic chronic stroke patients performing a selection of ADL tasks. We applied the taxonomy to identify the primitive content and motion characteristics of these tasks, and to evaluate the influence of impairment level on these outcomes.Results: The taxonomy could account for all motions in the sampled activities. Interrater reliability was high for primitive identification (Cohen's kappa = 0.95–0.99). Using the taxonomy, the ADL tasks were found to be composed primarily of transport and stabilize primitives mainly executed with discrete, proximal motions. Compared to mildly impaired patients, moderately impaired patients used more repeated reaches and axial-proximal UE motion to execute the tasks.Conclusions: The proposed taxonomy yields objective, quantitative data on human functional UE motion. This new method could facilitate the decomposition and quantification of UE rehabilitation, the characterization of functional abnormality after stroke, and the mechanistic examination of shared behavior in motor studies.

Yugoslav writer, art critic and curator Oto Bihalji-Merin (1904–1993) lived in Berlin during the 1920s, where he joined the German Communist Party and published literary critique in the left-wing press. Born into a Jewish family, he moved to Czechoslovakia, France, Switzerland, and Spain after the rise of the Nazi regime. In this period, he established close contact with left-wing intellectuals from all over Europe. During the Second World War, he was imprisoned as a Yugoslav soldier by the Germans. After the war, he became the most important cultural theorist and administrator in Tito’s Yugoslavia. After Tito’s break with Stalin, he promoted the concept of naïve folk art as an authentic, primordial expression of proletarian and peasant creativity. His doctrine, destined to bridge the gap between socialist realism and Western non-figurative art, was inspired by Miroslav Krleža’s cultural theory of the Third Way and by the non-aligned movement.

Background: Functional upper extremity (UE) motion enables humans to execute activities of daily living (ADLs). There currently exists no universal language to systematically characterize this type of motion or its fundamental building blocks, called functional primitives. Without a standardized classification approach, pooling mechanistic knowledge and unpacking rehabilitation content will remain challenging. Methods: We created a taxonomy to characterize functional UE motions occurring during ADLs, classifying them by motion presence, temporal cyclicity, upper body effector, and contact type. We identified five functional primitives by their phenotype and purpose: reach, reposition, transport, stabilize , and idle. The taxonomy was assessed for its validity and interrater reliability in right-paretic chronic stroke patients performing a selection of ADL tasks. We applied the taxonomy to identify the primitive content and motion characteristics of these tasks, and to evaluate the influence of impairment level on these outcomes. Results: The taxonomy could account for all motions in the sampled activities. Interrater reliability was high for primitive identification (Cohen's kappa = 0.95–0.99). Using the taxonomy, the ADL tasks were found to be composed primarily of transport and stabilize primitives mainly executed with discrete, proximal motions. Compared to mildly impaired patients, moderately impaired patients used more repeated reaches and axial-proximal UE motion to execute the tasks. Conclusions: The proposed taxonomy yields objective, quantitative data on human functional UE motion. This new method could facilitate the decomposition and quantification of UE rehabilitation, the characterization of functional abnormality after stroke, and the mechanistic examination of shared behavior in motor studies. [ABSTRACT FROM AUTHOR]

Abstract The aim of this paper is to present some contributions to the theory of finite transformation monoids. The dominating influence that permutation groups have on transformation monoids is used to describe and characterise transitive transformation monoids and primitive transitive transformation monoids. We develop a theory that not only includes the analogs of several important theorems of the classical theory of permutation groups but also contains substantial information about the algebraic structure of the transformation monoids. Open questions naturally arising from the substantial paper of Steinberg (2010) [11] have been answered. Our results can also be considered as a further development in the hunt for a solution of the Černý conjecture. [ABSTRACT FROM AUTHOR]

Artist and art historian Roger Fry used Paul Gauguin’s 1896 painting, Poèmes barbares, to advertise his 1910 exhibition, Manet and the Post-Impressionists. In Vision and Design (1920), Fry promoted the so-called “primitive” art of Oceania and sub-Saharan Africa as depending on unmediated perception that he associated with the Impressionists and Post-Impressionists. Although Fry’s assumption of an “ultra-primitive directness of vision” on the part of African makers ignores their own mediating conventions, his reliance on the Vischers’ notion of empathetic connection enhances the possibility of regarding the cultural products of peoples foreign to the percipient with what Paul C. Taylor terms an “ethical attentiveness that combats reductionism and objectification.” Further, such a form of attention can displace attempts to privilege European and Eurocentric art and thought, by referring to “primitive” art and thought, promoting recognition of their value in their own right. [ABSTRACT FROM AUTHOR]

At face value, the term "synergy" provides a unifying concept within a fractured field that encompasses complementary neural, computational, and behavioral approaches. However, the term is not used synonymously by different researchers but has substantially different meanings depending on the research approach. With so many operational definitions for the one term, it becomes difficult to use as either a descriptive or explanatory concept, yet it remains pervasive and apparently indispensable. Here we provide a summary of different approaches that invoke synergies in a descriptive or explanatory context, summarizing progress, not within the one approach, but across the theoretical landscape. Bernstein's framework of flexible hierarchical control may provide a unifying framework here, since it can incorporate divergent ideas about synergies. In the current motor control literature, synergy may refer to conceptually different processes that could potentially operate in parallel, across different levels within the same hierarchical control scheme. There is evidence for the concurrent existence of synergies with different features, both "hard-wired" and "soft-wired," and task independent and task dependent. By providing a comprehensive overview of the multifaceted ideas about synergies, our goal is to move away from the compartmentalization and narrow the focus on one level and promote a broader perspective on the control and coordination of movement. [ABSTRACT FROM AUTHOR]

Background: Intramedullary primitive neuroectodermal tumors (PNETs) are tumors found rarely in the cervical region, with only five such cases described in the literature. The available literature contains only one report regarding cervicomedullary junction PNET. Case Description: The authors present a case report of a 45-year-old male patient who had undergone urgent hospitalization owing to progressive tetraparesis and subtle impairment of respiratory function. He underwent magnetic resonance imaging, which showed an extensive enhancing cervical intramedullary tumor extending from C5 to the bulbar region. Since he developed severe impairment of respiratory function, he required tracheostomy. He then underwent microsurgery 2 days after his admission, and a partial tumor resection was performed. The pathological diagnosis of PNET of the cervicomedullary junction (CMJ) was made. He had slight worsening of strength after surgery with subsequent deterioration over the next 3 weeks. The tumor displayed aggressive growth; thus, radiotherapy was indicated. Unfortunately, he developed severe febrile neutropenia and died after 2 weeks of radiotherapy. Given the rarity of the condition, we wish to review the epidemiology, pathophysiology, and treatment options of his population. Conclusion: Intramedullary PNETs of the cervical spine and CMJ are exceedingly rare in adults; treatment of such patients remains a challenge, despite the modern neurosurgical armamentarium that is available. [ABSTRACT FROM AUTHOR]

Cerebellar mutism is a rare finding associated with resection of posterior fossa tumors or cerebellar hemorrhages. We reviewed the medical records of six children, aged 6 to 12 years, who developed cerebellar mutism after resection of a posterior fossa mass or as a result of posterior fossa trauma. From 1989 to 1994, 210 children underwent posterior fossa resection at our institution, and four developed mutism (an incidence of 1.6%). All four patients had primitive neuroectodermal tumors. The fifth patient experienced trauma, and another patient had an arteriovenous malformation (AVM). In four children, hydrocephalus developed as a result of their tumor or AVM. Four developed cerebellar mutism 24 to 48 hours after surgery or trauma, and one developed cerebellar mutism 5 days after surgery, coincident with hydrocephalus. In one, mutism occurred after a second resection was performed for a recurrence of his posterior fossa tumor. Cerebellar mutism lasted 10 days in one patient and 2 to 8 weeks in the other four. Dysarthria was apparent in four patients during the recovery phase. We suggest trauma to the dentate nucleus and/or its outflow tract, the superior cerebellar peduncle, as a cause of reversible mutism. Because posterior fossa tumors are common in children, mutism should be recognized as an important side effect of surgery.