Search Our Scientific Publications & Authors

Pharmaceuticals (Basel) 2022 May 26;15(6). Epub 2022 May 26.

Department of Pediatric Oncology, Dana-Farber Boston Children's Cancer and Blood Disorders Center, Boston, MA 02215, USA.

Pediatric brain tumors have surpassed leukemia as the leading cause of cancer-related death in children. Several landmark studies from the last two decades have shown that many pediatric brain tumors are driven by epigenetic dysregulation within specific developmental contexts. One of the major determinants of epigenetic control is the histone code, which is orchestrated by a number of enzymes categorized as writers, erasers, and readers. Read More

J Clin Med 2022 Jun 10;11(12). Epub 2022 Jun 10.

Department of Neurosurgery, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, and Humboldt-Universität zu Berlin, and Berlin Institute of Health, 13353 Berlin, Germany.

The utilization of fluorescein-guided biopsies has recently been discussed to improve and expedite operative techniques in the detection of tumor-positive tissue, as well as to avoid making sampling errors. In this study, we aimed to report our experience with fluorescein-guided biopsies and elucidate distribution patterns in different histopathological diagnoses in order to develop strategies to increase the efficiency and accuracy of this technique. We report on 45 fluorescence-guided stereotactic biopsies in 44 patients (15 female, 29 male) at our institution from March 2016 to March 2021, including 25 frame-based stereotactic biopsies and 20 frameless image-guided biopsies using VarioGuide. Read More

Neuropathology 2022 Jun 23. Epub 2022 Jun 23.

Department of Neurosurgery, University of Miami Miller School of Medicine, Miami, Florida, USA.

Intracranial myxoid mesenchymal tumor, FET::CREB fusion positive is a rare, recently described central nervous system neoplasm. It is characterized by EWSR1::CREB family transcription factor fusion, typically arises in children and adolescents, and is locally aggressive even after gross total resection. Currently, there are little data available to guide management and gauge long-term prognosis. Read More

J Neurosurg Case Lessons 2022 May 30;3(22):CASE22141. Epub 2022 May 30.

Norton Neuroscience Institute.

Background: Ependymomas are the most frequent tumors of the adult spinal cord, representing 1.9% of all central nervous system tumors and 60% of spinal cord tumors. Spinal ependymomas are usually solitary, intramedullary lesions. Read More

J Neurooncol 2022 Jun 22. Epub 2022 Jun 22.

Children's Hospital Colorado, University of Colorado School of Medicine, 13001 East 17th Place, Aurora, CO, 80045, USA.

Purpose: The role of white blood cells (WBC) in the pediatric central nervous system (CNS) tumor microenvironment is incompletely defined. We hypothesized that the WBC profile in cerebrospinal fluid (CSF) correlates with the presence of tumor cells and prognosis in pediatric CNS tumors, as well as other patient and disease characteristics, and differs by tumor type, thus giving insight into the tumor immune response.

Methods: We conducted a retrospective analysis of CSF WBC profiles at CNS tumor diagnosis in 269 patients at our institution. Read More

Oper Neurosurg (Hagerstown) 2022 Jul 11;23(1):e58. Epub 2022 May 11.

Department of Neurosurgery, Rutgers Robert Wood Johnson Medical School & University Hospital, New Brunswick, New Jersey, USA.

Front Neurol 2022 2;13:887544. Epub 2022 Jun 2.

Institut d'Hématologie et d'Oncologie Pédiatrique, Lyon, France.

Background: The clinical management of ependymoma in childhood and adolescence is complex and the clinicobiopathological correlates of outcome remain poorly understood. This international SIOP Ependymoma II (SIOP EPII) trial aims to improve the outcome of patients with ependymoma.

Methods: SIOP EPII includes any patient <22 years at diagnosis with ependymoma, stratified by age, tumor location, and outcome of the initial surgery. Read More

Genes Chromosomes Cancer 2022 Jun 18. Epub 2022 Jun 18.

The Division of Hematology, Oncology, Blood and Marrow Transplant, Nationwide Children's Hospital and The Ohio State University, 700 Children's Drive, Columbus, OH, USA.

Ependymal tumors are the third most common brain tumor under 14 years old. Even though metastatic disease is a rare event, it affects mostly young children and carries an adverse prognosis. The factors associated with dissemination and the best treatment approach have not yet been established and there is limited published data on how to manage metastatic disease, especially in patients under 3 years of age. Read More

Sci Rep 2022 Jun 16;12(1):10151. Epub 2022 Jun 16.

Department of Neurosurgery, Seoul National University Hospital, Seoul, Republic of Korea.

MRI is the primary diagnostic modality for spinal cord tumors. However, its validity has never been vigorously scrutinized in daily routine clinical practice, where MRI tissue diagnosis is usually not a single one but multiple ones with several differential diagnoses. Here, we aimed to assess the validity of MRI in terms of predicting the pathology and location of the tumor in routine clinical settings. Read More

Am J Med Genet A 2022 Jun 11. Epub 2022 Jun 11.

Department of Onco-Hematology, Cell Therapy, Gene Therapy and Hemopoietic Transplant, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

Ependymoma is the third most common pediatric brain tumor. Predisposition to develop ependymomas has been reported in different hereditary diseases, but the pathogenic variants related to the familial syndromes have rarely been detected in sporadic ependymomas. De novo variants in POLR2A, the gene encoding the largest subunit of RNA polymerase II, cause a neurodevelopmental disorder with a wide range of clinical manifestations, characterized by severe infantile-onset hypotonia, developmental delay, feeding difficulties, palatal anomalies, and facial dysmorphisms. Read More

Clin Neuroradiol 2022 Jun 8. Epub 2022 Jun 8.

Department of Radiology, Division of Neuroradiology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Purpose: This study aimed to evaluate the application of apparent diffusion coefficient (ADC) histogram analysis to differentiate posterior fossa tumors (PFTs) in children.

Methods: A total of 175 pediatric patients with PFT, including 75 pilocytic astrocytomas (PA), 59 medulloblastomas, 16 ependymomas, and 13 atypical teratoid rhabdoid tumors (ATRT), were analyzed. Tumors were visually assessed using DWI trace and conventional MRI images and manually segmented and post-processed using parametric software (pMRI). Read More

Neurochirurgie 2022 Jun 3. Epub 2022 Jun 3.

Institute of Cancer & Genomic Science, University of Birmingham, B152TT, Birmingham, United Kingdom.

Background: Differential diagnosis between medulloblastoma (MB), ependymoma (EP) and astrocytoma (PA) is important due to differing medical treatment strategies and predicted survival. The aim of this study was to investigate non-invasive MRI-based radiomic analysis of whole tumors to classify the histologic tumor types of pediatric posterior fossa brain tumor and improve the accuracy of discrimination, using a random forest classifier.

Methods: MRI images of 99 patients, with 59 MBs, 13 EPs and 27 PAs histologically confirmed by surgery and pathology before treatment, were included in this retrospective study. Read More

Neurooncol Adv 2022 Jan-Dec;4(1):vdac062. Epub 2022 Apr 22.

Department of Neurology/Brain Tumor Center, Erasmus MC Cancer Institute, Rotterdam, The Netherlands.

Background: Ependymomas, pilocytic astrocytomas, medulloblastomas, and intracranial germ cell tumors occur relative frequently in children, but are rare central nervous system (CNS) tumors in adults. In this population-based survey, we established incidence, treatment, and survival patterns for these tumors diagnosed in adult patients (≥18 years) over a 30-year period (1989-2018).

Methods: Data on 1384 ependymomas, 454 pilocytic astrocytomas, 205 medulloblastomas, and 112 intracranial germ cell tumors were obtained from the Netherlands Cancer Registry (NCR) on the basis of a histopathological diagnosis. Read More

Anticancer Res 2022 Jun;42(6):3203-3207

Department of Neurological Surgery, Hospital Padilla, Tucumán, Argentina.

Background/aim: Extraventricular supratentorial ependymomas are rare entities. Most ependymomas are located at the infratentorial and intraventricular level, and only in a small group of cases they do not present continuity with the ventricular system. This is a case report of a patient with an atypical location of a cerebral ependymoma, which required the implementation of a complex and infrequent approach for its complete microsurgical removal. Read More

Neuro Oncol 2022 May 31. Epub 2022 May 31.

Department of Pediatrics, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, SP, 14049-900, Brazil.

Background: Supratentorial RELA fusion (ST-RELA) ependymomas (EPNs) are resistant tumors without an approved chemotherapeutic treatment. Unfortunately, the molecular mechanisms that leads to chemoresistance traits of ST-RELA remains elusive. The aim of this study was to assess RELA fusion-dependent signaling modules, specifically the role of the Hedgehog (Hh) pathway as a novel targetable vulnerability in ST-RELA. Read More

J Child Neurol 2022 Jun 26;37(7):609-616. Epub 2022 May 26.

Department of Pediatrics, 5447Nicklaus Children's Hospital, Miami, FL, USA.

Radiation treatment is widely used to address unresectable intracranial tumors. Owing to the nature of therapy, healthy tissue and diseased regions will be affected. New insights have shown that not only does this impact brain parenchyma but it causes changes in fluid status, myelination, and the integrity of the blood-brain barrier. Read More

Clin Neurol Neurosurg 2022 Jul 18;218:107303. Epub 2022 May 18.

Department of Neurosurgery, Wessex Neurological Centre, Southampton General Hospital, Southampton, UK. Electronic address:

Tanycytic ependymomas are a rare spinal cord tumour arising from tanycyte cells lining the ventricle or spinal central canal. This is the first report of familial spinal tanycytic ependymoma occurring in two first degree relatives. Both patients underwent surgical resection of the intra-medullary tumours with good overall recovery. Read More

Brain Tumor Pathol 2022 May 22. Epub 2022 May 22.

Department of Pathology, Toulouse University Hospital, Toulouse, France.

J Clin Neurosci 2022 Jul 18;101:154-161. Epub 2022 May 18.

Division of Neurosurgery, Department of Surgery, National University Health System, Singapore.

Paediatric brain tumours (PBTs) are the most common solid tumours in children. Previous publications reflect variations in incidence rates and frequency of histological types in different global populations. However, there are limited studies on the epidemiology of PBTs in Singapore. Read More

Brain Tumor Pathol 2022 Jul 21;39(3):171-178. Epub 2022 May 21.

Department of Human Pathology, Gunma University Graduate School of Medicine, 3-39-22, Showa-machi, Maebashi, Gunma, 371-8511, Japan.

BCORL1 encodes a transcriptional corepressor homolog to BCOR. BCORL1 rearrangements have been previously described as rare events, and among them, CREBBP-BCORL1 has been reported only in 2 cases of ossifying fibromyxoid tumors. Herein, we present the first case of diffusely infiltrating glioma with CREBBP-BCORL1 involving a 17-year-old female patient. Read More

Neurooncol Adv 2022 Jan-Dec;4(1):vdac053. Epub 2022 Apr 13.

Department of Paediatric Oncology, Leeds Children's Hospital, The Leeds Teaching Hospitals NHS Trust, Leeds, UK.

Background: Relapsed ependymoma has a dismal prognosis, and the role of chemotherapy at relapse remains unclear. This study prospectively evaluated the efficacy of intensive intravenous (IV) etoposide in patients less than 21 years of age with relapsed intracranial ependymoma (NCT00278252).

Methods: This was a single-arm, open-label, phase II trial using Gehan's two-stage design. Read More

Acta Neuropathol 2022 May 19. Epub 2022 May 19.

Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, Bethesda, MD, 20814, USA.

Neurotherapeutics 2022 May 16. Epub 2022 May 16.

Department of Pathology, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA.

Advances in the understanding of the molecular biology of central nervous system (CNS) tumors prompted a new World Health Organization (WHO) classification scheme in 2021, only 5 years after the prior iteration. The 2016 version was the first to include specific molecular alterations in the diagnoses of a few tumors, but the 2021 system greatly expanded this approach, with over 40 tumor types and subtypes now being defined by their key molecular features. Many tumors have also been reconceptualized into new "supercategories," including adult-type diffuse gliomas, pediatric-type diffuse low- and high-grade gliomas, and circumscribed astrocytic gliomas. Read More

Front Oncol 2022 27;12:831016. Epub 2022 Apr 27.

Department of Neurosurgery, Trauma Center, Gamma Knife Center, Cannizzaro Hospital, Catania, Italy.

Background: Primary intracranial ependymomas (IE) are rare brain tumors rarely metastasizing outside the central nervous system. We systematically reviewed the literature on extra-neural metastases from primary IEs.

Methods: PubMed, Scopus, Web-of-Science, and Cochrane were searched following the PRISMA guidelines to include studies of extra-neural metastases from primary IEs. Read More

Chin Neurosurg J 2022 May 12;8(1):11. Epub 2022 May 12.

Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.

Background: To describe the epidemiological characteristics of central nervous system (CNS) tumors in children, based on the neurosurgery department of Beijing Tiantan Hospital.

Methods: From January 2015 to December 2019, 3180 children were histopathologically diagnosed with CNS tumors based on the 2016 World Health Organization (WHO) classification of tumors. Patients were 0 to 15 years old. Read More

Neurol Med Chir (Tokyo) 2022 May 10. Epub 2022 May 10.

Department of Neurological Surgery, School of Medicine, Wakayama Medical University.

To assess the clinicopathological features and prognostic factors of pediatric intracranial ependymomas and to explore the current diagnostic practice, we analyzed clinical data from the Brain Tumor Registry of Japan (BTRJ). Data of fifty children under 18 years of age diagnosed with intracranial ependymoma were extracted from the BTRJ database. Cases were reviewed for overall survival (OS) and progression-free survival (PFS), with attention to gender, preoperative Karnofsky performance status score, location of the tumor, the extent of resection, World Health Organization (WHO) histopathological grading, and adjuvant therapy. Read More

Childs Nerv Syst 2022 Apr 25. Epub 2022 Apr 25.

Department of Pathology, Chonnam National University Medical School, Chonnam National University Hwasun Hospital, Gwangju, Republic of Korea.

Introduction: Neurofibromatosis type 2 (NF-2) is an inherited disease, linked with abnormalities in the NF-2 gene, which is located on chromosome 22 and involved in merlin production. Many craniospinal tumors are common in individuals with NF-2. We present a case of NF-2 with the rapid symptomatic progression of multiple craniospinal tumors. Read More

Children (Basel) 2022 Apr 2;9(4). Epub 2022 Apr 2.

Pediatric Neurosurgery, Asklepios Children's Hospital, 53757 Sankt Augustin, Germany.

Brain tumors are the most common solid tumors in children and are associated with high mortality. The most common childhood brain tumors are grouped as low-grade gliomas (LGG), high grade gliomas (HGG), ependymomas, and embryonal tumors, according to the World Health Organization (WHO). Advances in molecular genetics have led to a shift from pure histopathological diagnosis to integrated diagnosis. Read More

Clin Case Rep 2022 Apr 15;10(4):e05723. Epub 2022 Apr 15.

Child Development Center King Abdullah Abdulaziz University Hospital Princess Nourah Bint Abdulrahman University Riyadh Saudi Arabia.

The neurobehavioral syndrome known as spatial neglect, which could be a result of a brain tumor, is common but difficult to diagnose and manage. This case study describes the evaluation of spatial neglect syndrome psychologically in detail and also builds an initial discussion of the role of the Arabic language, which requires a right-to-left-oriented spatial frame, to understand the severity of the symptoms. We report a case of spatial neglect syndrome after a brain tumor. Read More

Oper Neurosurg (Hagerstown) 2022 05;22(5):e189-e197

UCL Queen Square Institute of Neurology, University College London, London, UK.

Background: Despite the importance of complete, gross total resection (GTR) of fourth ventricular ependymomas, significant morbidity and/or subtotal resections are reported, particularly when the ventricular floor is infiltrated. Step-by-step technique descriptions are lacking in the literature.

Objective: To describe monitoring and stimulation mapping techniques and surgical nuances in the challenging subgroup of infiltrating fourth ventricular ependymomas by a highly illustrated, step-by-step description. Read More