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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

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

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

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

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

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

World Neurosurg 2022 Jul 9;163:38. Epub 2022 Apr 9.

Department of Neurosurgery and Brain Repair, University of South Florida, Tampa, Florida, USA.

Ependymomas are rare primary tumors of the brain and spinal cord that arises from the ependymal cell layer. Cranial ependymomas commonly occur in the posterior fossa; however, approximately 30% of all tumors can be found in the supratentorial region. Supratentorial ependymomas have a shorter progression-free and overall survival than their infratentorial counterparts. Read More

AJNR Am J Neuroradiol 2022 04 31;43(4):603-610. Epub 2022 Mar 31.

Department of Medical Imaging (A.J.), Ann and Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois

Background And Purpose: Pediatric supratentorial tumors such as embryonal tumors, high-grade gliomas, and ependymomas are difficult to distinguish by histopathology and imaging because of overlapping features. We applied machine learning to uncover MR imaging-based radiomics phenotypes that can differentiate these tumor types.

Materials And Methods: Our retrospective cohort of 231 patients from 7 participating institutions had 50 embryonal tumors, 127 high-grade gliomas, and 54 ependymomas. Read More

NMR Biomed 2022 Jun 27;35(6):e4673. Epub 2022 Jan 27.

Institute of Cancer and Genomic Sciences, University of Birmingham, Birmingham, UK.

MRS can provide high accuracy in the diagnosis of childhood brain tumours when combined with machine learning. A feature selection method such as principal component analysis is commonly used to reduce the dimensionality of metabolite profiles prior to classification. However, an alternative approach of identifying the optimal set of metabolites has not been fully evaluated, possibly due to the challenges of defining this for a multi-class problem. Read More

Oper Neurosurg (Hagerstown) 2022 03;22(3):e120-e121

Research Department, Association of Future African Neurosurgeons, Yaounde, Cameroon.

Methods Mol Biol 2022 ;2423:187-214

Department of Radiation Oncology, Neuro-Oncology Disease Management Group, Tata Memorial Centre, Homi Bhabha National Institute (HBNI), Mumbai, India.

Medulloblastoma, the most common malignant primary brain tumor in children, is now considered to comprise of four distinct molecular subgroups-wingless (WNT), sonic hedgehog (SHH), Group 3, and Group 4 medulloblastoma, each associated with distinct developmental origins, unique transcriptional profiles, diverse phenotypes, and variable clinical behavior. Due to its exquisite anatomic resolution, multiparametric nature, and ability to image the entire craniospinal axis, magnetic resonance imaging (MRI) is the preferred and recommended first-line imaging modality for suspected brain tumors including medulloblastoma. Preoperative MRI can reliably differentiate medulloblastoma from other common childhood posterior fossa masses such as ependymoma, pilocytic astrocytoma, and brainstem glioma. Read More

Cancers (Basel) 2021 Dec 3;13(23). Epub 2021 Dec 3.

Children's Brain Tumour Research Centre, Department of Paediatric Oncology, Royal Manchester Children's Hospital, Manchester University NHS Foundation Trust, Manchester M13 9WL, UK.

Brain tumors are the leading cause of childhood cancer deaths in developed countries. They also represent the most common solid tumor in this age group, accounting for approximately one-quarter of all pediatric cancers. Developments in neuro-imaging, neurosurgical techniques, adjuvant therapy and supportive care have improved survival rates for certain tumors, allowing a future focus on optimizing cure, whilst minimizing long-term adverse effects. Read More

J Magn Reson Imaging 2022 Jul 29;56(1):147-157. Epub 2021 Nov 29.

Radiological Sciences, Mental Health & Clinical Neuroscience, School of Medicine, University of Nottingham, Nottingham, UK.

Background: Medulloblastoma, ependymoma, and pilocytic astrocytoma are common pediatric posterior fossa tumors. These tumors show overlapping characteristics on conventional MRI scans, making diagnosis difficult.

Purpose: To investigate whether apparent diffusion coefficient (ADC) values differ between tumor types and to identify optimum cut-off values to accurately classify the tumors using different performance metrics. Read More

Brain Tumor Res Treat 2021 Oct;9(2):111-116

Department of Neurosurgery, Daegu Fatima Hospital, Daegu, Korea.

A 25-year-old female presented with a generalized tonic-clonic seizure. She had no previous history of seizures. A brain magnetic resonance imaging scan revealed a solitary enhancing mass in the right fronto-parietal cortex. Read More

Indian J Pathol Microbiol 2021 Oct-Dec;64(4):633-637

Department of Neurosurgery, GIPMER, Jawaharlal Nehru Marg, New Delhi, India.

Background: Tanycytic ependymoma (TE) (WHO grade II) is a rare and morphologically distinct variant of ependymoma with only 77 cases reported worldwide so far. Variable clinical and radio-pathological features lead to misdiagnosis as WHO grade 1 tumors. On imaging, differentials of either schwannoma, meningioma, low-grade glial (like angiocentric glioma), or myxopapillary ependymoma are considered. Read More

Contemp Oncol (Pozn) 2021 6;25(2):95-99. Epub 2021 May 6.

Department of Radiology, Hanoi Medical University, Hanoi, Vietnam Department of Radiology, Pham Ngoc Thach University of Medicine, Ho Chi Minh City, Vietnam Department of Radiology, Children's Hospital 2, Ho Chi Minh City, Vietnam.

Introduction: The distinction of medulloblastomas and ependymomas plays an important role in the care plans and prognosis of children. We aimed to investigate the role of magnetic resonance spectroscopy (MRS) in the differentiation between medulloblastomas and ependymomas in children.

Materials And Methods: The institutional review board approved this prospective study. Read More

World Neurosurg 2022 01 11;157:99-105. Epub 2021 Oct 11.

Department of Neurological Surgery, Northwestern University Feinberg School of Medicine, Division of Pediatric Neurosurgery, Ann and Robert H. Lurie Children's Hospital, Chicago, Illinois, USA. Electronic address:

Objective: Artificial intelligence (AI) has facilitated the analysis of medical imaging given increased computational capacity and medical data availability in recent years. Although many applications for AI in the imaging of brain tumors have been proposed, their potential clinical impact remains to be explored. A systematic review was performed to examine the role of AI in the analysis of pediatric brain tumor imaging. Read More

NMR Biomed 2022 02 13;35(2):e4630. Epub 2021 Oct 13.

Institute of Cancer and Genomic Sciences, University of Birmingham, Birmingham, UK.

H-magnetic resonance spectroscopy (MRS) provides noninvasive metabolite profiles with the potential to aid the diagnosis of brain tumours. Prospective studies of diagnostic accuracy and comparisons with conventional MRI are lacking. The aim of the current study was to evaluate, prospectively, the diagnostic accuracy of a previously established classifier for diagnosing the three major childhood cerebellar tumours, and to determine added value compared with standard reporting of conventional imaging. Read More

Sci Transl Med 2021 Oct 6;13(614):eabc0497. Epub 2021 Oct 6.

Department of Neurosurgery, University of Michigan, Ann Arbor, MI 48109, USA.

Childhood posterior fossa group A ependymomas (PFAs) have limited treatment options and bear dismal prognoses compared to group B ependymomas (PFBs). PFAs overexpress the oncohistone-like protein EZHIP (enhancer of Zeste homologs inhibitory protein), causing global reduction of repressive histone H3 lysine 27 trimethylation (H3K27me3), similar to the oncohistone H3K27M. Integrated metabolic analyses in patient-derived cells and tumors, single-cell RNA sequencing of tumors, and noninvasive metabolic imaging in patients demonstrated enhanced glycolysis and tricarboxylic acid (TCA) cycle metabolism in PFAs. Read More

J Neurooncol 2021 Oct 27;155(1):13-23. Epub 2021 Sep 27.

Department of Pediatrics, Pediatric Oncology Institute-GRAACC, Federal University of Sao Paulo, Sao Paulo, SP, Brazil.

Purpose: Ependymoma (EPN) accounts for approximately 10% of all primary central nervous system (CNS) tumors in children and in most cases, chemotherapy is ineffective and treatment remains challenging. We investigated molecular alterations, with a potential prognostic marker and therapeutic target in EPNs of childhood and adolescence, using a next-generation sequencing (NGS) panel specific for pediatric neoplasms.

Methods: We selected 61 samples with initial diagnosis of EPN from patients treated at Pediatric Oncology Institute-GRAACC/UNIFESP. Read More

Ecancermedicalscience 2021 30;15:1257. Epub 2021 Jun 30.

Department of Pathology, Basavatarakam Indo-American Cancer Hospital and Research Institute, Road number 10, Banjara Hills, Hyderabad-500034, Telangana, India.

Background: Primary intracranial malignancies with extra-skeletal myxoid chondrosarcoma (EMC) features are extremely rare. EMC constitutes a distinct genomic entity characterised by reciprocal translocation of fusion genes, most commonly EWS RNA Binding Protein 1 (EWSR1) in 22q12 with Nuclear Receptor Subfamily 4 Group A Member 3 (NR4A3) in 9q2-q31.1. Read More

Neurol India 2021 Jul-Aug;69(4):1045-1047

Department of Neurosurgery, Chettinad Hospital and Research Institute, Kelambakkam, Chennai, Tamil Nadu, India.

Anaplastic ependymoma in the pineal region is rare. Here, we present a rare case of anaplastic ependymoma of the pineal region on a 42-year-old woman who came to our hospital with headache associated with blurring of vision since one month. MRI brain showed a contrast enhancing mass lesion measuring 30 × 30 × 35 mm in the pineal region with obstructive hydrocephalus. Read More

J Comput Assist Tomogr 2021 May-Jun 01;45(3):463-471

From the Department of Radiology, Huashan Hospital of Fudan University, Shanghai.

Objective: To improve the understanding and the diagnosis of intracranial ependymal tumors.

Methods: The clinical, radiological and prognostic features of 48 supratentorial extraventricular ependymomas and 74 intraventricular ependymomas were summarized and compared.

Results: Supratentorial extraventricular ependymomas, most often located in the frontal lobe (33. Read More

Neurooncol Pract 2021 Aug 8;8(4):475-484. Epub 2021 Mar 8.

Department of Neurology, Clinical Neuroscience Center, University Hospital and University of Zurich, Zurich, Switzerland.

Background: The incidence of spinal cord gliomas, particularly in adults is low, and the role of chemotherapy has remained unclear.

Methods: We performed a multicenter, retrospective study of 21 patients diagnosed with spinal cord glioma who received chemotherapy at any time during the disease course. Benefit from chemotherapy was estimated by magnetic resonance imaging. Read More

Childs Nerv Syst 2021 10 16;37(10):2967-2974. Epub 2021 Jul 16.

Department of Neurosurgery, Acibadem Maslak Hospital, Istanbul, Turkey.

Introduction: Astroblastoma, MN1-altered (old name: high-grade neuroepithelial tumor/HGNET with MN1 alteration) is a recently described central nervous system tumor mostly affecting pediatric patients and profoundly young girls. Differential pathological diagnoses of these tumors include ependymoma, pleomorphic xanthoastrocytoma, embryonal tumor with multilayered rosettes, meningioma, and even glioblastoma. As the treatment approaches to these tumors differ, it is essential to increase the awareness about these tumors in the neurosurgical community. Read More

Surg Neurol Int 2021 14;12:279. Epub 2021 Jun 14.

Department of Neurosurgery, Geneva University Hospitals, Geneva, Switzerland.

Background: Full endoscopic resection of solid brain tumors represents a challenge for neurosurgeons. This can be achieved with modern technology and advanced surgical tools.

Case Description: A 23-years-old male was referred to our unit with raised intracranial pressure. Read More

Acta Neuropathol Commun 2021 06 23;9(1):113. Epub 2021 Jun 23.

Keck School of Medicine, University of Southern California, Los Angeles, CA, USA.