Updates in the Management of Central and Peripheral Nervous System Tumors among Patients with Neurofibromatosis Type 1 and Neurofibromatosis Type 2
Background: Neurofibromatosis type 1 and neurofibromatosis type 2 are unrelated, distinct genetic disorders characterized by the development of central and peripheral nervous system tumors.
Summary: Neurofibromatosis type 1 is the most common inherited tumor predisposition syndrome with a lifelong increased risk of benign and malignant tumor development, such as glioma and nerve sheath tumors. Neurofibromatosis type 2 classically presents with bilateral vestibular schwannoma, yet is also associated with non-vestibular schwannoma, meningioma, and ependymoma. Historically, the number of effective therapies for neurofibromat...
Source: Pediatric Neurosurgery - February 6, 2023 Category: Neurosurgery Source Type: research
Practical Algorithm for the management of multisutural craniosynostosis with associated Chiari malformation and/or hydrocephalus
The association between multisutural craniosynostosis with Chiari malformation (CM), venous hypertension and hydrocephalus is widely described in the literature, especially in children with pediatric craniofacial syndromes. Some efforts have been done in the last years to understand the complex pathogenetic mechanisms underlying this association and several theories have been proposed. However, whatever the pathophysiological mechanism, it is still unclear what is the best management and treatment of CM and hydrocephalus in multisutural craniosynostosis patients. The aim of this study is to report our twenty-five years ’...
Source: Pediatric Neurosurgery - January 31, 2023 Category: Neurosurgery Source Type: research
Peri-Insular hemispherotomy: A systematic review and institutional experience
Conclusion: PIH delivers Engel I outcomes for over 4 in 5 patients selected for this procedure, greater than described in combined hemispherectomy analyses. It is an effective technique in patients with deve lopmental and acquired pathologies, despite general preference of VPH in this patient group. Finally, very young patients may have significant seizure and cognitive benefits from PIH, however hydrocephalus is most common in this group warranting careful risk-benefit assessment. This review delivers a dedicated PIH outcomes analysis to inform clinical and patient decision making. (Source: Pediatric Neurosurgery)
Source: Pediatric Neurosurgery - January 13, 2023 Category: Neurosurgery Source Type: research
Advances and Clinical Trials Update in the Treatment of Diffuse Intrinsic Pontine Gliomas
Diffuse intrinsic pontine gliomas (DIPG) are high grade glioma (HGG) that occur primarily in children, and represent a leading cause of death in pediatric patients with brain tumors with a median overall survival of only 8-11 months. While these lesions were previously thought to behave similarly to adult HGG, emerging data have demonstrated that DIPG are a biologically distinct entity from adult HGG frequently driven by mutations in the histone genes H3.3 and H3.1 not found in adult glioma. While biopsy of DIPG was historically felt to confer unacceptable risk of morbidity and mortality, multiple studies have demonstrated...
Source: Pediatric Neurosurgery - January 13, 2023 Category: Neurosurgery Source Type: research
Letter to the Editor regarding the Article “Comparison of Follow-Up Length-Matched Single-Center Myelomeningocele Postnatal Closure Cohort to the Management of Myelomeningocele Study (MOMS) Trial Results”
(Source: Pediatric Neurosurgery)
Source: Pediatric Neurosurgery - January 10, 2023 Category: Neurosurgery Source Type: research
Comparison of Follow-Up Length-Matched Single-Center Myelomeningocele Postnatal Closure Cohort to the Management of Myelomeningocele Study (MOMS) Trial Results
NA (Source: Pediatric Neurosurgery)
Source: Pediatric Neurosurgery - January 10, 2023 Category: Neurosurgery Source Type: research
Review of the Recent Changes in the WHO Classification for Pediatric Brain and Spinal Cord Tumors
Background: Periodic updates to the World Health Organization (WHO) classification system for central nervous system (CNS) tumors reflect advances in the pathological diagnosis, categorization, and molecular underpinnings of primary brain, spinal cord, and peripheral nerve tumors. The 5th edition of the WHO Classification of CNS Tumors was published in 2021. This review discusses the guiding principles of the revision, introduces the more common new diagnostic entities, and describes tumor classification and nomenclature changes that are relevant for pediatric neurological surgeons.
Summary: Revisions to the WHO CNS tumor...
Source: Pediatric Neurosurgery - January 6, 2023 Category: Neurosurgery Source Type: research
Review of Pediatric Extraosseous Chordomas With A Unique, Illustrative Case
Conclusion: Our case is the first to present in such a manner, was shown to be external to the spinal canal, encasing the nerve root, and was the first such case in a pediatric patient. We reviewed the growing body of literature on spinal extraosseous chordomas and their characteristics within the pediatric patient population. We also reviewed chordoma pathogenesis theories as well as current and future treatment options. (Source: Pediatric Neurosurgery)
Source: Pediatric Neurosurgery - January 2, 2023 Category: Neurosurgery Source Type: research
Immunotherapy for Pediatric Brain and Spine Tumors: Current State and Future Directions
Background: Brain tumors are the most common solid tumors and the leading cause of cancer-related deaths in children. Incidence in the USA has been on the rise for the last 2 decades. While therapeutic advances in diagnosis and treatment have improved survival and quality of life in many children, prognosis remains poor and current treatments have significant long-term sequelae.Summary: There is a substantial need for the development of new therapeutic approaches, and since the introduction of immunotherapy by immune checkpoint inhibitors, there has been an exponential increase in clinical trials to adopt these and other i...
Source: Pediatric Neurosurgery - December 22, 2022 Category: Neurosurgery Source Type: research
Immunotherapy for Pediatric Brain and Spine Tumors, Current State and Future Directions.
Background
Brain tumors are the most common solid tumors and the leading cause of cancer-related deaths in children. Incidence in the US has been on the rise for the last 2 decades. While therapeutic advances in diagnosis and treatment have improved survival and quality of life in many children, prognosis remains poor and current treatments have significant long-term sequelae.
Summary
There is a substantial need for the development of new therapeutic approaches, and since the introduction of immunotherapy by immune checkpoint inhibitors there has been an exponential increase in clinical trials to adopt these and other i...
Source: Pediatric Neurosurgery - December 22, 2022 Category: Neurosurgery Source Type: research
