Cancer stem cells in meningiomas: novel insights and therapeutic implications.

Meningiomas (MGs), which arise from meningothelial cells of the dura mater, represent a significant proportion of primary tumours of the central nervous system (CNS). Despite advances in treatment, the management of malignant meningioma (MMG) remains challenging due to diagnostic, surgical, and resection limitations. Cancer stem cells (CSCs), a subpopulation within tumours capable of self-renewal and differentiation, are highlighted as key markers of tumour growth, metastasis, and treatment resistance. Identifying additional CSC-related markers enhances the precision of malignancy evaluations, enabling advancements in personalised medicine. The review discusses key CSC biomarkers that are associated with high levels of expression, aggressive tumour behaviour, and poor outcomes. Recent molecular research has identified CSC-related biomarkers, including Oct-4, Sox2, NANOG, and CD133, which help maintain cellular renewal, proliferation, and drug resistance in MGs. This study highlights new therapeutic strategies that could improve patient prognosis with more durable tumour regression. The use of combination therapies, such as hydroxyurea alongside diltiazem, suggests more efficient and effective MG management compared to monotherapy. Signalling pathways such as NOTCH and hedgehog also offer additional avenues for therapeutic development. CRISPR/Cas9 technology has also been employed to create meningioma models, uncovering pathways related to cell growth and proliferation. Since the efficacy of traditional therapies is limited in most cases due to resistance mechanisms in CSCs, further studies on the biology of CSCs are warranted to develop therapeutic interventions that are likely to be effective in MG. Consequently, improved diagnostic approaches may lead to personalised treatment plans tailored to the specific needs of each patient.

Applications of Augmented Reality in Neuro-Oncology: A Case Series.

Augmented reality (AR) is a technological tool that superimposes two-dimensional virtual images onto three-dimensional real-world scenarios through the integration of neuronavigation and a surgical microscope. The aim of this study was to demonstrate our initial experience with AR and to assess its application in oncological neurosurgery. This is a case series with 31 patients who underwent surgery at Santa Casa BH for the treatment of intracranial tumors in the period from March 4, 2022, to July 14, 2023. The application of AR was evaluated in each case through three parameters: whether the virtual images auxiliated in the incision and craniotomy and whether the virtual images aided in intraoperative microsurgery decisions. Of the 31 patients, 5 patients developed new neurological deficits postoperatively. One patient died, with a mortality rate of 3.0%. Complete tumor resection was achieved in 22 patients, and partial resection was achieved in 6 patients. In all patients, AR was used to guide the incision and craniotomy in each case, leading to improved and precise surgical approaches. As intraoperative microsurgery guidance, it proved to be useful in 29 cases. The application of AR seems to enhance surgical safety for both the patient and the surgeon. It allows a more refined immediate operative planning, from head positioning to skin incision and craniotomy. Additionally, it helps decision-making in the intraoperative microsurgery phase with a potentially positive impact on surgical outcomes.

Translation and validation of the Neurological Assessment in Neuro-Oncology scale to Brazilian Portuguese.

Introduction: The Neurological Assessment for Neuro-Oncology (NANO) scale was elaborated to assess neurologic function in integration with radiological criteria to evaluate neuro-oncological patients in clinical setting and enable the standardization of neurological assessment in clinical trials. The objective of this study is the translation to Brazilian Portuguese and transcultural adaptation of NANO scale in patients with the diagnosis of glioblastoma, brain metastasis and low-grade glioma. Methods: Patients with diagnosis of glioblastoma, brain metastasis, and low-grade glioma were prospectively evaluated between July 2019 and July 2021. The process of translating and cross-culturally adapting the NANO scale included: translation from English to Portuguese, synthesis and initial revision by an expert committee, back-translation from Portuguese to English, a second revision by the expert committee, and the application of the NANO scale. Regarding the reliability of the NANO scale, Cronbach's alpha was employed to measure the internal consistency of all scale items and assess the impact of item deletion. Additionally, Spearman's correlation test was used to evaluate the convergent validity between the NANO scale and Karnofsky Performance Scale (KPS). Results: One hundred and seventy-four patients were evaluated. A statistically significant inverse relation (p < 0.001) between KPS and NANO scale was founded. The Cronbach's alpha values founded for NANO scale were 0.803 for glioblastoma, 0.643 for brain metastasis, and 0.482 for low grade glioma. Discussion: The NANO scale Brazilian Portuguese version proves to be reproducible and valid to evaluate neuro-oncological patients with glioblastoma and brain metastasis, presenting a strong correlation with KPS scale. Further studies are warranted to assess the validity and reliability of the scale in patients diagnosed with low-grade glioma.

Resources for the practice of pediatric neuro-oncology in Mexico: a cross-sectional evaluation.

Background: The evaluation of existing resources and services is key to identify gaps and prioritize interventions to expand care capacity for children with central nervous system (CNS) tumors. We sought to evaluate the resources for pediatric neuro-oncology (PNO) in Mexico. Methods: A cross-sectional online survey with 35 questions was designed to assess PNO resources and services, covering aspects including number of patients, infrastructure, human resources, and diagnostic and treatment time intervals. The survey was distributed to the members of the Mexican Association of Pediatric Oncology and Hematology (AMOHP) who belong to the nation's many different health systems. Results: Responses were obtained from 33 institutions, distributed throughout the country and part of the many health systems that exist in Mexico. Twenty-one (64%) institutions had less than 10 new cases of pediatric CNS tumors per year. Although 30 (91%) institutions saw pediatric patients up to the age of 18 years, 2 (6%) had a cutoff of 15 years. Twenty-four (73%) institutions had between 1 and 3 pediatric oncologists providing care for children with CNS tumors. Six (18%) institutions did not have a neurosurgeon, while 19 (57%) institutions had a pediatric neurosurgeon. All centers had a pathology department, but 13 (39%) institutions only had access to basic histopathology. Eleven (33%) institutions reported histopathological diagnoses within one week, but 3 (9%) took more than 4 weeks. Radiotherapy for pediatric CNS tumors was referred to outside centers at 18 (55%) institutions. All centers had access to conventional cytotoxic chemotherapy, but only 6 (18%) had access to targeted therapy. Eighteen (55%) respondents estimated a survival rate of less than 60%. Fifteen (45%) centers attributed the main cause of mortality to non-tumor related factors, including infection and post-surgical complications. Conclusions: This is the first national assessment of the resources available in Mexico for the treatment of CNS tumors. It shows disparities in resource capacity and a lack of the specific and efficient diagnoses that allow timely initiation of treatment. These data will enable the prioritization of collaborative interventions in the future.

Surgical treatment of orbital tumors in a single center: Analysis and results.

Background: Orbital tumors, arising within the bony orbit and its contents, present diverse challenges due to their varied origins and complex anatomical context. These tumors, classified as primary, secondary, or metastatic, are further subdivided into intraconal and extraconal based on their relationship with the muscle cone. This classification significantly influences surgical approach and management. This study highlights surgical experiences with orbital tumors, underscoring the importance of tailored surgical approaches based on the lesion's site and its proximity to the optic nerve. Methods: This retrospective study at the National Institute of Cancer's Head and Neck Department (2005-2014) analyzed 29 patients with orbital tumors treated with surgery, radiotherapy, chemotherapy, or combinations of them. Patient demographics, tumor characteristics, and treatment responses were evaluated using computed tomography (CT), magnetic resonance imaging, and positron emission tomography-CT imaging. Malignant tumors often required orbital exenteration and reconstruction, highlighting the study's commitment to advancing orbital tumor treatment. Results: 29 patients (18 females and 11 males, age 18-88 years, mean 53.5 years) with orbital tumors exhibited symptoms such as decreased vision and exophthalmos. Tumors included primary lesions like choroidal melanoma and secondary types like epidermoid carcinoma. Treatments varied, involving a multidisciplinary team for surgical approaches like exenteration, with follow-up from 1 to 9 years. Radiotherapy and chemotherapy were used for specific cases. Conclusion: Our study underscores the need for a multidisciplinary approach in treating orbital tumors, involving various surgical specialists and advanced technologies like neuronavigation for tailored treatment. The integration of surgery with radiotherapy and chemotherapy highlights the effectiveness of multidimensional treatment strategies.

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.

SEOM-GEINO clinical guidelines for grade 2 gliomas (2023).

The 2021 World Health Organization (WHO) classification has updated the definition of grade 2 gliomas and the presence of isocitrate dehydrogenase (IDH) mutation has been deemed the cornerstone of diagnosis. Though slow-growing and having a low proliferative index, grade 2 gliomas are incurable by surgery and complementary treatments are vital to improving prognosis. This guideline provides recommendations on the multidisciplinary treatment of grade 2 astrocytomas and oligodendrogliomas based on the best evidence available.

Clinical Characteristics and Surgical Outcomes of 2542 Patients with Spinal Schwannomas: A Systematic Review and Meta-Analysis.

OBJECTIVE: This study was conducted to systematically analyze the data on the clinical features, surgical treatment, and outcomes of spinal schwannomas. METHODS: We conducted a systematic review and meta-analysis under the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A search of bibliographic databases from January 1, 2001, to May 31, 2021, yielded 4489 studies. Twenty-six articles were included in our final qualitative systematic review and quantitative meta-analysis. RESULTS: Analysis of 2542 adult patients' data from 26 included studies showed that 53.5% were male, and the mean age ranged from 35.8 to 57.1 years. The most common tumor location was the cervical spine (34.2%), followed by the thoracic spine (26.2%) and the lumbar spine (18.5%). Symptom severity was the most common indicator for surgical treatment, with the most common symptoms being segmental back pain, sensory/motor deficits, and urinary dysfunction. Among all patients analyzed, 93.8% were treated with gross total resection, which was associated with better prognosis and less chance of recurrence than subtotal resection. The posterior approach was the most common (87.4% of patients). The average operative time was 4.53 hours (95% confidence interval [CI], 3.18-6.48); the average intraoperative blood loss was 451.88 mL (95% CI, 169.60-1203.95). The pooled follow-up duration was 40.6 months (95% CI, 31.04-53.07). The schwannoma recurrence rate was 5.3%. Complications were particularly low and included cerebrospinal fluid leakage, wound infection, and the sensory-motor deficits. Most of the patients experienced complete recovery or significant improvement of preoperative neurological deficits and pain symptoms. CONCLUSIONS: Our analysis suggests that segmental back pain, sensory/motor deficits, and urinary dysfunction are the most common symptoms of spinal schwannomas. Surgical resection is the treatment of choice with overall good reported outcomes and particularly low complication rates. gross total resection offers the best prognosis with the slightest chance of tumor recurrence and minimal risk of complications.

Palliative care for children with central nervous system tumors: results of a Spanish multicenter study.

BACKGROUND: Brain tumors represent the most common cause of cancer-related death in children. Few studies concerning the palliative phase in children with brain tumors are available. OBJECTIVES: (i) To describe the palliative phase in children with brain tumors; (ii) to determine whether the use of palliative sedation (PS) depends on the place of death, the age of the patient, or if they received specific palliative care (PC). METHODS: Retrospective multicenter study between 2010 and 2021, including children from one month to 18 years, who had died of a brain tumor. RESULTS: 228 patients (59.2% male) from 10 Spanish institutions were included. Median age at diagnosis was 5 years (IQR 2-9) and median age at death was 7 years (IQR 4-11). The most frequent tumors were medulloblastoma (25.4%) and diffuse intrinsic pontine glioma (DIPG) (24.1%). Median number of antineoplastic regimens were 2 (range 0-5 regimens). During palliative phase, 52.2% of the patients were attended by PC teams, while 47.8% were cared exclusively by pediatric oncology teams. Most common concerns included motor deficit (93.4%) and asthenia (87.5%) and communication disorders (89.8%). Most frequently prescribed supportive drugs were antiemetics (83.6%), opioids (81.6%), and dexamethasone (78.5%). PS was administered to 48.7% patients. Most of them died in the hospital (85.6%), while patients who died at home required PS less frequently (14.4%) (p = .01). CONCLUSION: Children dying from CNS tumors have specific needs during palliative phase. The optimal indication of PS depended on the center experience although, in our series, it was also influenced by the place of death.

Manejo quirúrgico de los tumores difusos de tronco pediátricos / Surgical management of diffuse brainstem high-grade gliomas

Introducción: Los tumores difusos de tronco representan aproximadamente el 10% de los tumores pediátricos de SNC. Dentro de este grupo se encuentran los gliomas difusos de línea media H3K27M, los cuales presentan mal pronóstico, con una media de supervivencia de entre 9 a 15 meses. La búsqueda de marcadores moleculares es una gran herramienta oncológica al momento de desarrollar nuevas terapéuticas para estos pacientes. Es por esto que la biopsia quirúrgica representa, hoy en día, un eslabón fundamental. Objetivos: El objetivo de este artículo es comunicar, mediante la presentación de dos casos clínicos, el manejo de los tumores difusos de tronco pediátricos mediante su biopsia quirúrgica a cielo abierto por abordaje subtemporal. Descripción de casos: Se presentan dos casos de pacientes pediátricos a quienes se les realizó una biopsia de tronco mediante abordaje subtemporal en el año 2022 en el servicio de neurocirugía pediátrica de la Fundación para la Lucha contra las Enfermedades Neurológicas de la Infancia (Fleni). El resultado de la anatomía patológica en ambos pacientes resultó de glioma difuso de línea media H3K27M. Se realiza además una revisión de la técnica quirúrgica del abordaje subtemporal. Intervención: Ambos pacientes evolucionaron sin complicaciones en el postoperatorio y mantuvieron seguimiento y tratamiento dirigido por oncología. Aun cuando la práctica más utilizada es la biopsia estereotáctica, nosotros preferimos la vía subtemporal a cielo abierto. La misma ha probado resultar efectiva para la obtención de suficiente material tumoral para realizar marcadores oncológicos, con buen control de hemostasia, sin mayores comorbilidades ni demoras en el inicio del tratamiento. Conclusión: Los tumores difusos de tronco pediátricos presentan un pronóstico ominoso, aún realizando tratamiento oncológico dirigido. La biopsia quirúrgica resulta fundamental a fin de obtener un mayor entendimiento respecto a la gran diversidad biológica que subyace a estas entidades y sus opciones terapéuticas, tanto actuales como futuras. El abordaje subtemporal es una excelente vía para realizar su biopsia(AU)

Background: Brainstem diffuse tumors represent approximately 10% of pediatric CNS tumors. Among them, H3K27M-mutant diffuse midline gliomas (DMGs) are rare childhood central nervous system tumors that carry a dismal prognosis, with a mean survival time of 9-15 months. Tumor biopsy is not only helpful for addressing a correct diagnosis, but is also an important strategy for the continuous study of further therapeutic options, given the former known bad prognosis with standard treatment. Objective: Our objective is to communicate, through the presentation of two clinical cases, our experience with these tumors by performing a biopsy through a subtemporal approach. Cases description: Two patients underwent tumor biopsy through a subtemporal approach at our institution in 2022. Both patients presented with MRIs compatible with diffuse pons gliomas. These cases were retrospectively reviewed and the surgical approach described.We also review and compare the different surgical approaches as well as treatment options for this pathology. Surgery: The patients presented no postoperative complication. Both patient's biopsy results were H3K27M-mutant diffuse midline gliomas and underwent oncological treatment and follow up. Although stereotactic biopsy is the most commonly used practice, at our institution we choose to use open surgery through a subtemporal approach. It has proven to provide a better surgical view with a chance of greater biopsy samples, satisfactory bleeding control with no added comorbidities. Nowadays, biopsy has proven fundamental for the treatment and follow up of these patients. Conclusion: DMGs are rare childhood central nervous system tumors that carry a dismal prognosis. Historically, biopsy for brainstem diffuse tumors was used only when the diagnosis was not clear. Nowadays, with the forthcoming of different surgical techniques and the development of new subtypes of molecular targets, the biopsy seems a fundamental step. The subtemporal approach is an excellent alternative to biopsy this pathology(AU)

Preoperative tractography algorithm for safe resection of tumors located in the descending motor pathways zone.

Background: Diffusion tensor imaging (DTI) tractography facilitates maximal safe resection and optimizes planning to avoid injury during subcortical dissection along descending motor pathways (DMPs). We provide an affordable, safe, and timely algorithm for preoperative DTI motor reconstruction for gliomas adjacent to DMPs. Methods: Preoperative DTI reconstructions were extracted from a prospectively acquired registry of glioma resections adjacent to DMPs. The surgeries were performed over a 7-year period. Demographic, clinical, and radiographic data were extracted from patients' electronic medical records. Results: Nineteen patients (12 male) underwent preoperative tractography between January 1, 2013, and May 31, 2020. The average age was 44.5 years (range, 19-81 years). A complete radiological resection was achieved in nine patients, a subtotal resection in five, a partial resection in three, and a biopsy in two. Histopathological diagnoses included 10 patients with high-grade glioma and nine with low-grade glioma. A total of 16 perirolandic locations (10 frontal and six frontoparietal) were recorded, as well as two in the insula and one in the basal ganglia. In 9 patients (47.3%), the lesion was in the dominant hemisphere. The median preoperative and postoperative Karnofsky Performance Scores were 78 and 80, respectively. Motor function was unchanged or improved over time in 15 cases (78.9%). Conclusion: This protocol of DTI reconstruction for glioma removal near the DMP shows good results in low-term neurological functional outcomes.

Modified Trans-Sinusal Transglabellar Approach for Anterior Cranial Fossa Meningiomas: Technical Note and Literature Review.

Introduction Currently, skull base surgery faces the dilemma of achieving the maximum possible tumor resection through less aggressive approaches and with minimal retraction of brain tissue. The objective of this work is to report a minimally invasive step-by-step approach to anterior cranial fossa tumors and to perform a literature review. Methods In our work, we describe a step-by-step approach, with images, which is a variation of the transglabellar approach. Results In all cases, we achieved maximum total resection of the lesion. There were no postoperative complications related to the surgery. In one case, we used the access to remove a foreign body in the frontal lobe. Conclusion The frontal trans-sinusal transglabellar access allows direct access to anterior cranial fossa tumors and other frontal lobe lesions close to the floor of the anterior fossa, without the need for brain retraction, allowing early devascularization of the tumor. However, this access is not recommended for all types of tumors, and is being improved for more anteriorly located lesions.

Virtual preoperative planning and 3D tumoral reconstruction with Horos open-source software.

Background: Presurgical three-dimensional (3D) reconstructions allow spatial localization of cerebral lesions and their relationship with adjacent anatomical structures for optimal surgical resolution. The purpose of the present article is to present a method of virtual preoperative planning aiming to enhance 3D comprehension of neurosurgical pathologies using free DICOM image viewers. Case Description: We describe the virtual presurgical planning of a 61-year-old female presenting a cerebral tumor. 3D reconstructions were created with the "Horos®" Digital Imaging and Communications in Medicine viewer, utilizing images obtained from contrast-enhanced brain magnetic resonance imaging and computed tomography. The tumor and adjacent relevant structures were identified and delimited. A sequential virtual simulation of the surgical stages for the approach was performed with the identification of local gyral and vascular patterns of the cerebral surface for posterior intraoperative recognition. Through virtual simulation, an optimal approach was gained. Accurate localization and complete removal of the lesion were achieved during the surgical procedure. Virtual presurgical planning with open-source software can be utilized for supratentorial pathologies in both urgent and elective cases. Virtual recognition of vascular and cerebral gyral patterns is helpful reference points for intraoperative localization of lesions lacking cortical expression, allowing less invasive corticotomies. Conclusion: Digital manipulation of cerebral structures can increase anatomical comprehension of neurosurgical lesions to be treated. 3D interpretation of neurosurgical pathologies and adjacent anatomical structures is essential for developing an effective and safe surgical approach. The described technique is a feasible and accessible option for presurgical planning.

Uncommon metastases to the brain: Frontal lobe myoepithelial carcinoma.

Background: Myoepithelial tumors have been widely described as a rare form of salivary gland neoplasm, although currently soft-tissue phenotypes have also been identified. These are tumors composed entirely of myoepithelial cells that exhibit a dual epithelial and smooth muscle phenotype. The occurrence of myoepithelial tumors within the central nervous system is also extremely rare, with only a few cases reported. Treatment options include surgical resection, chemotherapy, radiotherapy, or a combination of these approaches. Case Description: The authors present a case of soft-tissue myoepithelial carcinoma with an unusual brain metastasis, rarely described in the literature. The purpose of this article is to present an update on the diagnosis and treatment of this pathology when affecting the central nervous system, through the review of the current evidence. Conclusion: However, despite complete surgical resection, there is about a significative high rate of local recurrence and metastasis. Careful patient follow-up and staging is essential for better characterization and understanding of this tumor's behavior.

Risk for hydrocephalus, hygroma, and tumor dissemination after ventricular opening during resection of supratentorial neoplasms in children.

PURPOSE: The possibility that ventricular opening generates postoperative complications after surgical tumor treatment often restricts the degree of tumor resection. This study aims to determine whether the ventricular opening is associated with more complications in surgeries for resectioning supratentorial intra-axial brain tumors in the pediatric population. METHODS: A retrospective review analysis was performed of patients treated at IOP/GRAACC between 2002 and 2020 under 19 years of age and underwent surgery for supratentorial intra-axial primary brain tumor resection. Data were collected from 43 patients. RESULTS: Glial tumor was more common than non-glial (65% vs. 35%, p = 0.09). The ventricular opening was not related to neoplastic spreads to the neuroaxis (6% vs. 0, p > 0.9) or leptomeningeal (3% vs. 0, p > 0.9). Of the patients whose ventricle was opened, 10% developed hydrocephalus requiring treatment, while none of the patients in the group without ventricular opening developed hydrocephalus (p = 0.5). There was also no statistical difference regarding ventriculitis. Postoperative subdural hygroma formation correlated with the ventricular opening (43% vs. 0, p = 0.003). The survival at 1, 5, and 10 years of cases with the ventricular opening was 93.2%, 89.7%, and 75.7%, respectively, while in cases without ventricular opening, it was 100%, 83%, and 83%, respectively, respectively, with no statistical difference between the mortality curves. CONCLUSION: Our study demonstrated that ventricular violation was not associated with the occurrence of significant complications. It was related to the formation of subdural hygroma, which did not require additional treatment.

Mortality of central nervous system tumors in pediatric patients of Brazil from 1979 to 2019.

PURPOSE: Cancer is the second leading cause of death in children from 2001 to 2005 in Brazil. This study aimed to describe the pattern of mortality from central nervous system (CNS) tumors in children in Brazil from 1979 to 2019. METHODS: A descriptive study was carried out using data from the Mortality Information System (SIM) of the Ministry of Health, according to the International Classification of Diseases (ICD-9 and ICD-10), between 1979 and 2019. The frequencies of the distribution of available variables were calculated: age (0 - 19 years), s skin color, tumor behavior, year and place of death (by region), ICD-10, and all of these, excluding skin color (by ICD-9). Mortality rates in general, mortality from neoplasms, and specific rates of CNS tumors were calculated considering the variables described above. RESULTS: In 40 years (1979 - 2019), there were 21,940 deaths due to brain tumors in children. A different pattern of the mortality rate of brain tumors was shown in children per age (increasing until age 5 - 9 years (28.9%) and then decreasing until age 15 - 19 years (20.2%)). The Southeast (44.3%), Northeast (23.4%), and South (17.5%) regions of Brazil had the highest rates; 94.7% of tumors were malignant, and 91.1% of deaths were observed in hospitals. CONCLUSION: To our knowledge, this is the first description of the mortality rate epidemiology of brain/CNS tumors in children in Brazil over 40 years. Furthermore, tumor malignancy, hospitals, and the Southeast and Northeast region of Brazil are factors associated with a higher mortality rate.

Fluorescein-guided resection for pediatric low-grade gliomas: institutional experience on two cases and a narrative literature review.

PURPOSE: Low-grade gliomas compose 30% of pediatric central nervous system tumors and outcomes of disease-free progression, and survival is directly correlated to the extent of resection. The use of sodium fluorescein (Na-Fl) is an intraoperative method in the localization of tumor cells in adult patients to optimize resection. Our purpose is to describe the use of Na-Fl in pediatric low-grade gliomas and its outcomes. METHODS: Patients under 18 years of age with low-grade gliomas at the author's institution underwent resection with the use of Na-Fl, with review of preoperative imaging findings, intraoperative results, and follow-up. Then, a comprehensive, narrative literature review of the use of Na-Fl in pediatric low-grade glioma was performed. RESULTS: Our single-institution use of Na-Fl in pediatric patients with suspected low-grade glioma demonstrated excellent results of intraoperative enhancement of tumor cells as well as gross total resection. The literature demonstrated 84% Na-Fl staining and 59.2% of gross total resection in pediatric low-grade gliomas with few small case studies, a range of reported findings, and few side effects. CONCLUSION: Na-Fl has a promising use in low-grade glioma resection in the pediatric patient population. Further research is warranted, such as randomized controlled studies, to assess Na-Fl as a potential tool in improving resection and long-term favorable outcomes.

Infantile metastatic ependymoma with a novel molecular profile and favorable outcome to intensive chemotherapy without irradiation: Case-based review.

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. We provide a review of the literature on clinical characteristics and radiation-sparing treatments for metastatic ependymoma in children under 3 years of age treated. The majority (73%) of the identified cases were above 12 months old and had the PF as the primary site at diagnosis. Chemotherapy-based approaches, in different regimens, were used with radiation reserved for progression or relapse. The prognosis varied among the studies, with an average of 50%-58% overall survival. This study also describes the case of a 7-month-old boy with metastatic posterior fossa (PF) ependymoma, for whom we identified a novel SPECC1L-RAF1 gene fusion using a patient-centric comprehensive molecular profiling protocol. The patient was successfully treated with intensive induction chemotherapy followed by high-dose chemotherapy and autologous hematopoietic progenitor cell rescue (AuHSCR). Currently, the patient is in continuous remission 5 years after his diagnosis, without radiation therapy. The understanding of the available therapeutic approaches may assist physicians in their management of such patients. This report also opens the perspective of newly identified molecular alterations in metastatic ependymomas that might drive more chemo-sensitive tumors.