Adv Tech Stand Neurosurg
January 2023
Pediatric pineal region tumors consist of tumors of pineal gland origin and parapineal origin. The former are comprised of germ cell tumor (GCT) and pineal parenchymal tumor. The latter originate from the surrounding neural structures, such as the midbrain and thalamus; thus, they are often benign gliomas during childhood.
View Article and Find Full Text PDFChilds Nerv Syst
October 2023
Objectives: In the past 50 years, pediatric neurosurgery has made tremendous strides, and gained its own identity as a distinct subspecialty. I have personally observed this progress and evolution in pediatric neurosurgery in multiple dimensions, which are described based upon my own experience and reflection.
Methods: The development and evolutions of multiple domains of pediatric neurosurgery, including neuroimaging, hydrocephalus, pediatric brain tumor, spinal dysraphism, craniosynostosis, vascular malformation, functional neurosurgery and spinal disorders were reviewed and commented on based upon my own experience and reflection.
Pediatric high-grade gliomas (pHGGs) are common malignant brain tumors without effective treatment and poor patient survival. Abnormal posttranslational modification at the histone H3 tail plays critical roles in tumor cell malignancy. We have previously shown that the trimethylation of lysine 4 at histone H3 (H3K4me3) plays a significant role in pediatric ependymoma malignancy and is associated with tumor therapeutic sensitivity.
View Article and Find Full Text PDFOver the last half a century, diagnostic neuroimaging has made tremendous strides following the introduction of computerized tomography (CT) and subsequent magnetic resonance imaging (MR). Prior to that time, the neurological diagnosis was conducted with careful history taking, physical examinations, and invasive testing such as cerebral angiography, encephalography, and myelography. Techniques and contrast media for these tests have been refined and progressed over time.
View Article and Find Full Text PDFPurpose: The aim of this study is to analyze rates of ventriculopleural (VPL) shunt failure and complications among patients with pediatric hydrocephalus, and to analyze which factors may predict early (< 1 year) or late (> 1 year) VPL shunt failure in this sample.
Methods: A retrospective chart review was conducted of all consecutive VPL shunt placements from 2000 to 2019 at our institution. Data was collected on patient characteristics, shunt history, and shunt type.
Glioblastomas presenting topographically at the cerebellopontine angle (CPA) are exceedingly rare. Given the specific anatomical considerations and their rarity, overall survival (OS) and management are not discussed in detail. The authors performed an integrative survival analysis of CPA glioblastomas.
View Article and Find Full Text PDFHow abnormal neurodevelopment relates to the tumour aggressiveness of medulloblastoma (MB), the most common type of embryonal tumour, remains elusive. Here we uncover a neurodevelopmental epigenomic programme that is hijacked to induce MB metastatic dissemination. Unsupervised analyses of integrated publicly available datasets with our newly generated data reveal that SMARCD3 (also known as BAF60C) regulates Disabled 1 (DAB1)-mediated Reelin signalling in Purkinje cell migration and MB metastasis by orchestrating cis-regulatory elements at the DAB1 locus.
View Article and Find Full Text PDFChilds Nerv Syst
February 2023
Introduction: Intracranial germ cell tumor (iGCT) is a rare disorder and often occurs during childhood and adolescence. iGCTs are frequently localized in pineal region and hypothalamic-neurohypophyseal axis (HNA). In spite of well-established clinical and pathological entity, histogenesis of iGCTs remains unsettled.
View Article and Find Full Text PDFObjectives: Controversy remains regarding surgical managements of sylvian fissure arachnoid cyst (SFAC). This review presents our experience in the microsurgical fenestration of pediatric patients with SFAC to define surgical indication, and risks and benefits with special emphasis on postoperative subdural fluid collection (SDFC) and cyst size reduction.
Methods: Thirty-four children with SFAC who underwent microsurgical cyst fenestration at a single institution over a 10-year period were retrospectively reviewed for their clinical presentation, neuroimaging findings, and postsurgical course.
Purpose: Resecting pineal region tumors in children is often challenging. Several approaches have been proposed and practiced. A personal series of pediatric pineal region tumors resected through craniotomy with posterior interhemispheric occipital transtentorial (OT) approach are reviewed.
View Article and Find Full Text PDFRadiation induced cavernomas among children with medulloblastoma are common following external beam radiation (XRT) treatment with either photon or proton beams. However, with the increased utilization of proton beam therapy over the last decade we sought to determine if there was any difference in the development or natural history of these cavernous malformations (CM) or CM-like lesions. We performed a retrospective analysis of 79 patients from 2003 to 2019 who had undergone resection of medulloblastoma and subsequent XRT (30 photon or 49 proton beam therapy).
View Article and Find Full Text PDFINTRODUCTION: Posthemorrhagic hydrocephalus (PHH) is a common disease process encountered in neonates. Management often includes cerebrospinal fluid (CSF) aspiration through ventricular access devices (VADs). However, a common concern surrounding serial access of implanted subcutaneous reservoirs includes introduction of infection.
View Article and Find Full Text PDFSuprasellar germ cell tumors (S-GCTs) are rare, presenting in either solitary or multifocal fashion. In this study, we retrospectively examine 22 solitary S-GCTs and 20 bifocal germ cell tumors (GCTs) over a 30-year period and demonstrate clinical, radiographic, and prognostic differences between the two groups with therapeutic implications. Compared to S-GCTs, bifocal tumors were almost exclusively male, exhibited higher rate of metastasis, and had worse rates of progression free and overall survival trending toward significance.
View Article and Find Full Text PDFObjective: Choroid plexus tumors (CPTs) are rare pediatric intracranial neoplasms, and mostly occur in the lateral ventricle. CPTs located in the infratentorial location are considered to be rare in the pediatric population. We present a series of eight patients treated in the last decade at our institution focusing on clinical presentations and their outcome after excision.
View Article and Find Full Text PDFMalignant gliomas are heterogeneous neoplasms. Glioma stem-like cells (GSCs) are undifferentiated and self-renewing cells that develop and maintain these tumors. These cells are the main population that resist current therapies.
View Article and Find Full Text PDFIn a previous study, we showed that folate receptor-α (FRα) translocates to the nucleus where it acts as a transcription factor and upregulates Hes1, Oct4, Sox2, and Klf4 genes responsible for pluripotency. Here, we show that acetylation and phosphorylation of FRα favor its nuclear translocation in the presence of folate and can cause a phenotypic switch from differentiated glial cells to dedifferentiated cells. shRNA-FRα mediated knockdown of FRα was used to confirm the role of FRα in dedifferentiation.
View Article and Find Full Text PDFAtypical teratoid rhabdoid tumors (ATRTs) are rare malignant central nervous system tumors, commonly occurring before 3 years of age. Median overall survival (OS) of patients with these tumors is about 1 year, despite aggressive multimodal therapy. Pediatric primary spinal ATRTs are even more rare, with fewer than 50 cases reported.
View Article and Find Full Text PDFPurpose: This manuscript describes our management philosophy of Chiari I malformation in children based on a single neurosurgeon's personal experience.
Methods: Based on 61 infants and children with Chiari I malformation treated from 2007 to 2017, typical symptoms, surgical indications, types of surgery, and evaluation of surgical decompression are reviewed.
Results: Sixty-one patients had 69 decompressions, with 90% having symptom improvement.
Polo-like kinase 4 (PLK4) is a cell cycle-regulated protein kinase (PK) recruited at the centrosome in dividing cells. Its overexpression triggers centrosome amplification, which is associated with genetic instability and carcinogenesis. In previous work, we established that PLK4 is overexpressed in pediatric embryonal brain tumors (EBT).
View Article and Find Full Text PDFBackground: Ependymomas (EPNs) are the third most common brain tumor in children. These tumors are resistant to available chemotherapeutic treatments, therefore new effective targeted therapeutics must be identified. Increasing evidence shows epigenetic alterations including histone posttranslational modifications (PTMs), are associated with malignancy, chemotherapeutic resistance and prognosis for pediatric EPNs.
View Article and Find Full Text PDFBackground: Gene-mediated cytotoxic immunotherapy (GMCI) is a tumor-specific immune stimulatory strategy implemented through local delivery of aglatimagene besadenovec (AdV-tk) followed by anti-herpetic prodrug. GMCI induces T-cell dependent tumor immunity and synergizes with radiotherapy. Clinical trials in adult malignant gliomas demonstrated safety and potential efficacy.
View Article and Find Full Text PDFNeuroblastoma (NB) is the most common extracranial solid tumor in pediatrics, with rare occurrences of primary and metastatic tumors in the central nervous system (CNS). We previously reported the overexpression of the polo-like kinase 4 (PLK4) in embryonal brain tumors. PLK4 has also been found to be overexpressed in a variety of peripheral adult tumors and recently in peripheral NB.
View Article and Find Full Text PDFCirsoid aneurysms, also known as scalp arteriovenous malformations (AVM), are rare congenital extracranial vascular anomalies that often present as an enlarging pulsatile scalp mass. A 14-yr-old male presented with a pulsatile scalp lesion that was first noticed 3 yr prior and had progressively enlarged. No history of trauma was reported.
View Article and Find Full Text PDFObjective: In this study, we investigate a neuroprotective agent, erythropoietin (EPO), in animal hydrocephalus model and its potential reversal effects on hydrocephalus by altering the expression of aquaporin-4 (AQP4).
Methods: Obstructive hydrocephalus was induced in 2-week-old rat pups by injecting kaolin (50 μl, 10 mg/ml in saline) into the cisterna magna, while the control pups received only saline. Kaolin-injected pups were divided into two groups on the fifth day after kaolin injection; one group received intra-peritoneal (i.