New CNS tumor classification: The importance in pediatric neurosurgical practice.

Background: The management of the central nervous system (CNS) tumors in the pediatric population is crucial in neurosurgical practice. The World Health Organization (WHO) has evolved its classification of CNS tumors from the 19th century to the 5th edition, published in 2021, incorporating molecular advancements. This transition from morphology to molecular characterization is ongoing. Methods: This manuscript analyzes the modifications introduced in the 5th edition of WHO's CNS tumor classification, particularly focusing on pediatric tumor families. The paper integrates clinical, morphological, and molecular information, aiming to guide pediatric neurosurgeons in their daily practice and interdisciplinary discussions. Results: The 5th edition of the WHO classification introduces a hybrid taxonomy that incorporates both molecular and histological components. The terminology shifts from "entity" to "type" and "subtype," aiming to standardize terminology. Tumor grading experiences changes, integrating molecular biomarkers for prognosis. The concept of integrated layered diagnosis is emphasized, where molecular and histological information is combined systematically. Conclusion: The 5th edition of the WHO CNS classification signifies a paradigm shift toward molecular characterization. The incorporation of molecular advances, the layered diagnostic approach, and the inclusion of clinical, morphological, and molecular information aim to provide comprehensive insights into pediatric CNS tumors. This classification offers valuable guidance for pediatric neurosurgeons, aiding in precise diagnosis and treatment planning for these complex neoplasms.

Pediatric hemispheric cerebellar low-grade gliomas: clinical approach, diagnosis, and management challenges-experience at a tertiary care children's hospital.

PURPOSE: This study aims to provide an exhaustive analysis of pediatric low-grade gliomas (pLGGs) in the cerebellar hemispheres, focusing on incidence, clinical characteristics, surgical outcomes, and prognosis. It seeks to enhance understanding and management of pLGGs in the pediatric population. METHODS: We conducted an observational, descriptive, retrospective, and cross-sectional study at a pediatric hospital, reviewing medical records of 30 patients with cerebellar hemispheric pLGGs treated from December 2014 to January 2023. Data collection included demographics, clinical presentation, imaging findings, surgical approach, postoperative complications, histopathological diagnosis, hydrocephalus management, and follow-up. Molecular markers and adjuvant therapies were also analyzed. RESULTS: The cohort predominantly presented with cerebellar symptoms, with 60% showing hydrocephalus at diagnosis. MRI with gadolinium was crucial for diagnosis. Surgical focus was on achieving gross total resection (GTR), accomplished in 70% of cases. Postsurgical hydrocephalus was less common, and cerebellar mutism was not reported. While a complete molecular analysis was not performed in all cases, available data suggest significant influence of molecular markers on prognosis and therapeutic options of pLGGs. CONCLUSIONS: This study highlights the unique clinical and molecular characteristics of cerebellar hemispheric pLGGs in children. The lower incidence of postoperative hydrocephalus and absence of cerebellar mutism are notable findings. Emphasizing a multidisciplinary approach, our findings contribute to a deeper understanding of pediatric pLGGs, underscoring the need for personalized treatment strategies and vigilant follow-up.

Tumors of the central nervous system. Classification of the World Health Organization 2021. Towards a paradigm shift. / Tumores del sistema nervioso central. Clasificación de la Organización Mundial de la Salud 2021. Hacia un cambio de paradigma.

The study of central nervous system (CNS) tumors is a subject of great interest and such knowledge is of great importance in medical practice. The classifications of CNS neoplasms began in the mid-19th century, until the World Health Organization (WHO) published, in 1979, the first edition of a useful systematic review for the purpose of establishing a common language for all medical specialties. To date, 5 updated editions of neoplastic taxonomy have been published. The fifth edition, from 2021, consolidates the paradigm shift brought about by molecular advances, although the transition between morphological and molecular biological characterization is still in progress. In this article, the new modifications introduced in the different most frequent families of tumors in pediatrics are analyzed, emphasizing useful information for pediatricians in their daily practice and multidisciplinary consultations.

El estudio de los tumores del sistema nervioso central (SNC) resulta ser un tema de gran consideración y su conocimiento reviste una alta importancia en la práctica médica. Las clasificaciones de las neoplasias del SNC comenzaron a mediados del siglo XIX hasta que en 1979 la Organización Mundial de la Salud (OMS) publicó la primera edición de una sistemática útil con el objetivo de establecer un lenguaje común para todas las especialidades médicas. Al día de hoy, 5 ediciones actualizaron la taxonomía neoplásica. La quinta edición del año 2021 consolida el cambio de paradigma dado por los avances moleculares, si bien todavía la transición se encuentra en proceso entre la caracterización morfológica y la biológica molecular. En este artículo, se analizan las nuevas modificaciones incorporadas en las diferentes familias tumorales más frecuentes en pediatría haciendo hincapié en aquella información de utilidad para el médico pediatra en su práctica diaria y la consulta multidisciplinaria.

Inflammatory myofibroblastic tumor of the lung involving the central nervous system in pediatric patients.

INTRODUCTION: The inflammatory myofibroblastic tumor (IMT) is a very rare lesion with an incidence of less than 0.1% of total neoplasms and with main affection in the lungs. Involvement in the central nervous system is extremely rare, but with a much more aggressive course than IMT diagnosed in the rest of the body. We report the 2 cases presented in our neurosurgery department to date; both were treated satisfactorily without intercurrences in 10 years of follow-up. HISTORICAL BACKGROUND: The World Health Organization described the IMT as a distinctive lesion composed of myofibroblastic spindle cells accompanied by an inflammatory infiltrate of plasma cells, lymphocytes, and eosinophils. CLINICAL PRESENTATION: Clinical manifestations of patients with CNS IMT vary and may consist of headache, vomiting, seizures, and blindness. Seizures are the most common symptom in patients with focal lesions. DIAGNOSIS: The true origin of this entity remains to be elucidated, but to date, etiologies ranging from chromosomal alterations to autoimmune or postinfectious mechanisms have been described. Due to its rarity and non-specificity in imaging, the final diagnosis of IMT in the brain parenchyma relies on pathological examination. MANAGEMENT: Treatment options are controversial and include total or subtotal removal, high-dose steroids, and radiation therapy. In the last decade, the development of ALK Tyrosine Kinase Inhibitors allows the possibility of chemotherapy in those patients harboring ALK mutations. CONCLUSION: IMT is a rare tumor that can exceptionally be found in the CNS. The cause is still unknown although the different studies focus on a neoplastic origin. The diagnosis is based in the use of different modalities of imaging and with histological confirmation. Optimal management is gross total resection whenever possible, is the only established curative treatment. Further research with longer follow-up is needed to clarify the natural history of this rare tumor.

Abusive head trauma, protocols, and legal concerns in a South American Country.

The abusive head trauma (AHT) is a form of child abuse and is a frequent entity all over de world. It is particularly unique among medical diagnoses because of the legal implications imposed by the diagnosis. Therefore, it has been the subject of much legal controversy over the decades. Knowledge of the clinical signs and imaging findings of abusive head trauma is vitally important for early diagnosis. An oriented anamnesis, as well as a complete physical examination and obtaining adequate images of the central nervous system, play a significant role in confirming the presumptive diagnosis. The interdisciplinary approach (pediatricians, neurosurgeons, neuroradiologists, social workers, and other specialists) is the key in the management of these patients. The purpose of this article is to familiarize the pediatric neurosurgeon with some of the more common medicolegal issues surrounding AHT as well as to discuss legal commitments and ethical obligations of the neurosurgeon in Argentina (South America) based on 2 clinical cases.

Method to perform safety stereotactic procedures in children under 2 years of age.

INTRODUCTION: Stereotactic procedures have been used in neurosurgery for many years. In children especially, care should be considered to avoid complication caused by fixation of the frame in a not fully developed skull bone. We present our method to adapt the frame in children under 2 years of age. METHODS: Twelve procedures in patients under 24 months were performed between 2003 and 2015. Micromar frame was used. It was adapted with a small pillow made of gauss attached to the posterior part to hold the head, then four pins were fixed without adjustment. We analyze for each patient age, indication, histopathology, and complications. RESULTS: Eleven patients with a mean age of 13.5 months (range 9 to 22 months) underwent 12 stereotactic procedures. In all cases, biopsy samples were obtained, histopathology was positive in 11/12 cases. No complications occurred. CONCLUSION: Stereotactic frame procedures need special attention in small children where the skull bones are not fully developed. We present a simple method to perform this surgery in patients under 2 years of age.

Reacción paradojal al tratamiento antituberculoso en un paciente inmunocompetente: a propósito de un caso / Paradoxical reaction during tuberculosis treatment in immunocompetent children: case report

Tuberculosis is an important public health problem. It is estimated that around 5-10% of patients with tuberculosis present with central nervous system involvement; meningitis and tuberculoma being two of the most frequent manifestations. The paradoxical reaction in patients undergoing antituberculosis treatment is infrequent, nevertheless it is an important consideration in patients, who after an appropriate initial response to specific treatment, present with worsening clinical and radiological signs or the appearance of new lesions.

La tuberculosis (TBC) es un problema de importancia en salud pública. Se estima que alrededor de 5 a 10% de los pacientes con TBC presentan compromiso de sistema nervioso central, siendo la meningitis y el tuberculoma las manifestaciones más frecuentes. La reacción paradojal en pacientes con tratamiento antituberculoso es infrecuente, sin embargo, es importante considerarla en pacientes que durante la terapia, luego de una respuesta inicial apropiada, presentan un empeoramiento clínico y/o radiológico o aparición de nuevas lesiones.

[Paradoxical reaction during tuberculosis treatment in immunocompetent children: case report]. / Reacción paradojal al tratamiento antituberculoso en un paciente inmunocompetente: a propósito de un caso.

Tuberculosis is an important public health problem. It is estimated that around 5-10% of patients with tuberculosis present with central nervous system involvement; meningitis and tuberculoma being two of the most frequent manifestations. The paradoxical reaction in patients undergoing antituberculosis treatment is infrequent, nevertheless it is an important consideration in patients, who after an appropriate initial response to specific treatment, present with worsening clinical and radiological signs or the appearance of new lesions.

Metástasis supratentorial de meduloblastoma de fosa posterior / Supratentorial metastasis of P-fosa medulloblastoma

Objective: our objective is to analyze the patients that presented supratentorial metastases. Material and methods: we studied 170 patients with medulloblastoma from the year 1991 to 2005. Twenty seven presentedsupratentorial metastases. We considered age at presentation, pathology, staging, dissemination to other places and outcome. Results: twenty seven patients (15,8%) with medulloblastoma presented supratentorial metastases, the average age was 5,87 years range from 3 to 11 years. 13/27 were staged high risk and 14/27 standard. The average time between surgery and presence of recurrence was 12,65 month. 12/27 presented desmoplasia. 25/27 patients died. Conclusion: 15,8% of patients with medulloblastoma presented supratentorial metastases before 16 months of the first surgery. Allthe patients were younger than 12 years. The appearance of supratentorial metastases is not related with the stage or the presence of desmoplasia. The outcome was unfavorable once the dissemination was diagnosed.

Tratamiento temprano de la cuadriparesia espástica y distonia severa con bombas infusión intratecal de flujo fijo / Spastic tetraparesis and severe dystonia treatment with intratechal fixed-flow bombs

Objective: to establish that the implantation of smaller fixed rate intrathecal infusion pumps is the best choice for the early treatment of severe spastic quadriparesis and dystonia and to determine its optimal placementMaterials and method: seven patients were implanted with fixed rate pumps. In two patients the indication was chronic pain and were excluded from the present study. Three patients were operated with 5.6 centimeters of diameter Tricumed pumps and two patients with Codman Arquimedes pumps. Results: two patients implanted with tricumed pumps and two with codman pump evolved favorably up to date, with a refill of 190, 200, 232 and 245 micrograms respectively. The device had to be explanted from the last patient due to post-op infection and dysfunction. Conclusion: early implantation of a baclofen pump facilitates and simplifies neuro-orthopedic treatments and gastrostomy implants. With an adequate subfascial technique and smaller infusion pumps satisfactory results can be achieved.