FET:CREB fusions have been described in a variety of tumors from various phenotypes. Recently, these fusion transcripts were reported in intracranial tumors, variably named intracranial mesenchymal myxoid tumors or angiomatoid fibrous histiocytomas. Controversy remains concerning the terminology for these tumors. Here, we report 11 cases of central nervous system mesenchymal tumors with proven FET:CREB fusion. Most DNA methylation profiles were not classifiable using the Heidelberg Brain Tumor or Sarcoma Classifier (v11b4/v12.2). However, by using unsupervised t-SNE and hierarchical clustering analyses, six of the cases constituted a distinct cluster. The remaining four tumors showed no obvious relation to any of the other referenced classes but were close to the clusters of extra-CNS angiomatoid fibrous histiocytomas (n = 1), clear cell sarcomas (n = 1), or solitary fibrous tumors (n = 2). Our findings confirm that intracranial FET:CREB-fused tumors do not represent a single molecular tumor entity, although most samples clustered close to each other, indicating the existence of a distinct epigenetic group that could potentially be partially masked by the low number of cases included. Further analyses are needed to characterize intracranial FET:CREB fused-defined tumors in more detail.

https://onlinelibrary.wiley.com/doi/pdf/10.1111/bpa.13010

An integrative histopathological and epigenetic characterization of primary intracranial mesenchymal tumors, FET:CREB-fused broadening the spectrum of tumor entities in comparison with their soft tissue counterparts.

A novel histomolecular tumor of the central nervous system, the "intracranial mesenchymal tumor (IMT), FET-CREB fusion-positive" has recently been identified in the literature and will be added to the 2021 World Health Organization Classification of Tumors of the Central Nervous System. However, our latest study using DNA-methylation analyses has revealed that intracranial FET-CREB fused tumors do not represent a single molecular tumor entity. Among them, the main subgroup presented classical features of angiomatoid fibrous histiocytoma, having ultrastructural features of arachnoidal cells, for. Another tumor type with clear cell component and histopathological signs of aggressivity clustered in close vicinity with clear cell sarcoma of soft tissue. Herein, we report one case of IMT with a novel SMARCA2-CREM fusion which has until now never been described in soft tissue or the central nervous system. We compare its clinical, histopathological, immunophenotypic, genetic and epigenetic features with those previously described in IMT, FET-CREB fusion-positive. Interestingly, the current case did not cluster with IMT, FET-CREB fusion-positive but rather presented histopathological (clear cell morphology with signs of malignancy), clinical (with a dismal course with several recurrences, metastases and finally the patient's death), genetic (fusion implicating the CREM gene), and epigenetic (DNA-methylation profiling) similarities with our previously reported clear cell sarcoma-like tumor of the central nervous system. Our results added data suggesting that different clinical and histomolecular tumor subtypes or grades seem to be included within the terminology "IMT, FET-CREB fusion-positive", and that further series of cases are needed to better characterize them.

/pdf/a-novel-smarca2-crem-fusion-expanding-the-molecular-spectrum-53070w6g1y.pdf

A novel SMARCA2-CREM fusion: expanding the molecular spectrum of intracranial mesenchymal tumors beyond the FET genes.

To review the epidemiology, the differential diagnosis, and the clinical and laboratory factors associated with the care management of headaches in patients with systemic lupus erythematosus (SLE).

Headache and systemic lupus erythematosus: A narrative review

Background In recent years, hepatic arterial infusion chemotherapy (HAIC) has gained popularity in the treatment of hepatocellular carcinoma. Although several studies have been published, no bibliometric analysis have been conducted on this topic. Objectives To understand the development status and future trends in the application of HAIC, we conducted bibliometric analysis to examine the cooperation and influence among countries, institutions, authors, and journals. Methods All relevant articles and reviews on the use of HAIC in HCC treatment were retrieved from the Web of Science database. A bibliometric analysis of countries, institutions, journals, authors, and keywords related to this field was performed using R and VOSviewer software. The main aspects analyzed were the research status and key fields of HAIC in HCC treatment. Results A total of 1026 articles published in 292 journals by 4937 authors from 959 institutions between 1974 and 2021 were retrieved. A rapid increase in articles published after 1990 was observed, which reached the peak in 2021. Japan had the most publications and citations. Yonsei University, Sun Yat-sen University, and Hiroshima University were the three leading institutions in research on this topic. Kwang-Hyub Han and Masatoshi Kudo have the greatest academic influence in this field. Most publications were made in the Hepato-Gastroenterology, whereas cancer had the most citations. The main aspects of HAIC treatment of HCC include HAIC and TACE, chemotherapy drug selection, HAIC and targeted therapy and immunotherapy, HAIC and surgery, and hepatotoxicity. Keywords such as FOLFOX, lenvatinib, hepatic arterial infusion chemotherapy are hot words in this field in recent years. Conclusion The research on the use of HAIC in the treatment of HCC has been on the rise. Currently, HAIC combined with targeted therapy or immunotherapy has attracted significant attention. 

/pdf/hepatic-arterial-infusion-chemotherapy-in-hepatocellular-k4vo5zhs.pdf

Hepatic arterial infusion chemotherapy in hepatocellular carcinoma: A bibliometric and knowledge-map analysis

The fifth edition of the World Health Organization Classification of Tumors of the Central Nervous System (CNS) now includes mesenchymal tumors that occur uniquely or frequently in the CNS. Moreover, this version has aligned the terminology of mesenchymal tumors with their soft tissue counterparts. New tumor types have been added, such as the "intracranial mesenchymal tumor, FET-CREB fusion-positive", the "CIC-rearranged sarcoma", and the "Primary intracranial sarcoma, DICER1-mutant". Other entities (such as rhabdomyosarcoma) have remained in the current WHO classification because these tumor types may present specificities in the CNS as compared to their soft tissue counterparts. Based on an extensive literature review, herein, we will discuss these newly recognized entities in terms of clinical observation, radiology, histopathology, genetics and outcome, and consider strategies for an accurate diagnosis. In light of this literature analysis, we will also introduce some potentially novel tumor types. 

/pdf/mesenchymal-non-meningothelial-tumors-of-the-central-nervous-2972215x.pdf

Mesenchymal non-meningothelial tumors of the central nervous system: a literature review and diagnostic update of novelties and emerging entities

Primary Intracranial Angiomatoid Fibrous Histiocytoma: Two Case Reports and Literature Review

Background Spheno-orbital meningiomas (SOMs) are often benign. The association of meningioma and systemic lupus erythematosus (SLE) is rarely discussed in the literature. Here, we report two patients with high-grade, SOMs with a prolonged history of SLE and review the literature. Case description The first case is a 52-year-old female patient with a 15-year history of SLE diagnosis who was referred to our center with a 1-year history of proptosis and excessive tearing of the left eye. This patient was operated for the left SOM with histopathological diagnosis of the World Health Organization (WHO) Grade III rhabdoid meningioma. The second case is a 36-year-old female patient with a 12-year history of SLE diagnosis who presented to our clinic with a 5-year-history of progressive right eye proptosis and occasional headaches. She was operated for the right SOM with histopathological diagnosis of the WHO Grade II chordoid meningioma. Conclusion Rhabdoid and chordoid SOMs are uncommon and no previous report discussed their occurrence in patients with SLE. The association of high-grade meningiomas and SLE deserves further exploration.

High-grade spheno-orbital meningioma in patients with systemic lupus erythematosus: Two case reports and literature review.

AIM Epidural hematoma (EDH) is a commonly encountered neurosurgical condition. Numerous articles have been published on EDH. Bibliometric analysis studies the chronological trends and ranks the most impactful articles in a given field. The aim of this paper is to analyze the most-cited articles on cranial and spinal EDH. MATERIAL AND METHODS A title-specific search was performed on the Scopus database using the term "epidural hematoma" in June 2020, with no publication date restrictions. The top 100 most-cited articles were collected, reviewed, and analyzed. RESULTS A total of 2165 articles were published on EDH from 1949 to 2020, and the top 100 most-cited ones were published between 1966 and 2014, receiving an average citation of 84.7 per paper. Most papers were published in Neurosurgery and Journal of Neurosurgery (JNS). 48% of the most-cited articles on EDH originated from the United States of America (USA). Notably, studies on spinal EDH represented 75% of the most-cited articles in our review. The most-cited article on EDH was published by Lawton et al. in 1995, receiving a total of 412 citations at an annual citation rate of 16.4%. CONCLUSION This report identifies the most influential publications on EDH as well as the publications trends over the last 70 years. Recognition of the most impactful work is an important tool for clinicians and researchers as it can reflect the enormous changes in the clinical practice. This report can serve as a guide for developing evidence-based practices and identifying areas of research inadequacy.

A bibliometric analysis of the most-cited articles on craniospinal epidural hematoma.

BACKGROUND Digital subtraction angiography (DSA) is still considered the gold standard test to evaluate arteriovenous malformation's (AVM) residual after microsurgical resection. OBJECTIVE To evaluate the safety and reliability of intraoperative computed tomography angiography (iCTA) as an immediate method of evaluating the surgical results of AVM resection. METHODS We performed a retrospective review for all cases of cerebral AVMs at our institute from January 2015 to April 2020 who underwent surgical resection of cerebral AVM and had iCTA. All included patients underwent a postoperative DSA, and the results were compared with iCTA. RESULTS Twenty-eight cases were included. All cases showed complete resection (100%) in the iCTA, and the results were consistent with the postoperative DSA results. The sensitivity of iCTA was 100%. The added operative time ranged from 25 to 30 min. There were no complications related to the use of iCTA. CONCLUSION Intraoperative assessment of AVMs surgical results with an iCTA is safe and reliable. The sensitivity of iCTA following AVM resection merits further investigations.

Sarah Bin Abdulqader

Papers

Primary Intracranial Angiomatoid Fibrous Histiocytoma: Two Case Reports and Literature Review

High-grade spheno-orbital meningioma in patients with systemic lupus erythematosus: Two case reports and literature review.

A bibliometric analysis of the most-cited articles on craniospinal epidural hematoma.

How Reliable Is the Intraoperative Computed Tomography Angiography in Assessing Complete Surgical Resection of Cerebral Arteriovenous Malformations