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OBJECTIVE: We aim to report the epidemiology, surgical outcomes, and survival rates of pediatric patients with posterior fossa tumors in a large single-center case series. METHODS: A retrospective analysis was conducted on pediatric patients who underwent surgical treatment for posterior fossa tumors between January 2011 and January 2019. RESULTS: A total of 135 pediatric patients, with an average age of 7.5 years at diagnosis and a mean follow-up of 35.7 months, were included in the study. Most tumors were located within the midline, with ventriculomegaly observed in 71.4% of the patients. Pilocytic astrocytomas encompassed the majority of tumors (34.1%), followed by medulloblastomas (27.4%) and ependymomas (11.8%). Gross total resection (GTR) was achieved in 71.8% of the patients, with a recurrence rate of 20%. Surgical complications were observed in 25.9% of the patients. GTR significantly impacted 5-year overall survival (OS) and 4-year progression-free survival (PFS) in patients with posterior fossa tumors. Patients who underwent GTR had a 5-year OS of 89.7%, compared to 72.7% for near-total resection and 70.8% for subtotal resection. The 4-year PFS for patients who underwent GTR was 82.5%, whereas it was 63.6% for patients who underwent near-total resection and 54.2% for patients who underwent subtotal resection. CONCLUSION: Surgical resection remains the main treatment for pediatric posterior fossa tumors, and higher resection rates are linked to better survival outcomes. Despite limited resources for molecular diagnosis, our institution has demonstrated that a specialized neurooncological center with a high surgical volume can still achieve favorable survival outcomes for these patients.

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Posterior fossa tumors are the most common pediatric brain tumors, and present unique challenges in terms of their location and surgical management. The posterior fossa comprehends complex anatomy and represents the smallest and deepest of the three cranial base fossae. An in-depth understanding of posterior fossa anatomy is crucial when it comes to the surgical resection of pediatric brain tumors. Mastering the knowledge of posterior fossa anatomy helps the neurosurgeon in achieving a maximal and safe volumetric resection, that impacts in both overall and progression free survival. With the advancements in microsurgery, the telovelar approach has emerged as the workhorse technique for the resection of posterior fossa tumors in pediatric patients. This approach involves meticulously dissecting of the natural clefts present in the cerebellomedullary fissure, making a comprehensive understanding of the underlying anatomy key for its success.

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While in adults most intracranial tumors develop around the cerebral hemispheres, 45 to 60% of pediatric lesions are found in the posterior fossa, although this anatomical region represents only 10% of the intracranial volume. The latest edition of the WHO classification for CNS tumors presented some fundamental paradigm shifts that particularly affected the classification of pediatric tumors, also influencing those that affect posterior fossa. Molecular biomarkers play an important role in the diagnosis, prognosis, and treatment of childhood posterior fossa tumors and can be used to predict patient outcomes and response to treatment and monitor its effectiveness. Although genetic studies have identified several posterior fossa tumor types, differing in terms of their location, cell of origin, genetic mechanisms, and clinical behavior, recent management strategies still depend on uniform approaches, mainly based on the extent of resection. However, significant progress has been made in guiding therapy decisions with biological or molecular stratification criteria and utilizing molecularly targeted treatments that address specific tumor biological characteristics. The primary focus of this review is on the latest advances in the diagnosis and treatment of common subtypes of posterior fossa tumors in children, as well as potential therapeutic approaches in the future.   Conclusion: Molecular biomarkers play a central role, not only in the diagnosis and prognosis of posterior fossa tumors in children but also in customizing treatment plans. They anticipate patient outcomes, measure treatment responses, and assess therapeutic effectiveness. Advances in neuroimaging and treatment have significantly enhanced outcomes for children with these tumors. What is Known: • Central nervous system tumors are the most common solid neoplasms in children and adolescents, with approximately 45 to 60% of them located in the posterior fossa. • Multimodal approaches that include neurosurgery, radiation therapy, and chemotherapy are typically used to manage childhood posterior fossa tumors What is New: • Notable progress has been achieved in the diagnosis, categorization and management of posterior fossa tumors in children, leading to improvement in survival and quality of life.

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BACKGROUND: The main treatments for hydrocephalus due to posterior fossa tumors are tumor resection with or without an external ventricular drain, ventriculoperitoneal shunt (VPS), and endoscopic third ventriculostomy. Although preoperative cerebrospinal fluid diversion by any of these techniques improves clinical outcomes, evidence comparing the efficacy of these techniques is scarce. Therefore, we aimed to retrospectively evaluate each treatment modality. METHODS: This single-center study analyzed 55 patients. Treatments were classified as successful (hydrocephalus resolution with a single surgical event) or failed and compared with a χ2 test. Kaplan-Meier curves and log-rank tests were employed. A Cox proportional hazard model was used to determine relevant covariates predicting outcomes. RESULTS: Mean patient age was 36.3 years, 43.4% of patients were men, and 50.9% of patients presented with uncompensated intracranial hypertension. Mean tumor volume was 33.4 cm3, and extent of resection was 90.85%. Tumor resection with or without an external ventricular drain was successful in 58.82% of cases, VPS was successful in 100%, and endoscopic third ventriculostomy was successful in 76.19% (P = 0.014). Mean follow-up time was 15.12 months. Log-rank test found statistically significant differences between survival curves of treatments (P = 0.016) favoring the VPS group. Postoperative surgical site hematoma was a significant covariate in the Cox model (hazard ratio = 17; 95% confidence ratio, 2.301-81.872; P = 0.004). CONCLUSIONS: This study favored VPS as the most reliable treatment of hydrocephalus due to posterior fossa tumors in adult patient; however, several factors influence clinical outcomes. We proposed an algorithm based on our findings and other authors' findings to facilitate the decision-making process.

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PURPOSE: Posterior fossa tumors are the most frequent pediatric solid tumor. Its main treatment is a surgical resection. Being a frequent procedure does not mean that it is exempt from complications, such as surgical site infections (SSI). The main objective of this paper is to study the risk factors associated with SSI following a resection of posterior fossa tumors in a purely pediatric population. METHODS: A retrospective case-control study including all posterior fossa tumor surgeries performed at our hospital between January 2014 and December 2019 was conducted. All patients with a diagnosis of a postoperative SSI have been included as cases, and those who had surgery and no infectious complications have been considered as controls. RESULTS: When analyzing risk factors, we have found that patients with ventriculoperitoneal shunt (VPS) (p = 0.03) or external ventricular drainage (EVD) (p = 0.005) placement had a greater chance of presenting a postoperative surgical site infection. Prolonged operative time (p < 0.001) and cerebrospinal fluid (CSF) leak through the wound (p = 0.002) also caused an increase in the risk of SSI in the postoperative period. A higher hemoglobin value (p = 0.002) would seem to be a preventive factor. CONCLUSIONS: Some strategies that could help to reduce the risk of infections are managing hydrocephalus preferably with endoscopic third ventriculostomy, minimizing the needed operative time to perform the procedure, obtaining an adequate serum hemoglobin level, and avoiding CSF leak through the wound.

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Introducción: Los tumores de fosa posterior son la patología oncológica más frecuente en pediatría con la exéresis quirúrgica como principal tratamiento1­3. El hecho de que sea un procedimiento frecuente no hace que se encuentre exento de complicaciones, como infecciones del sitio quirúrgico 4,5. El objetivo principal es conocer los factores de riesgo que se asocian a infecciones del sitio quirúrgico posterior a la exéresis de tumores de fosa posterior en una población completamente pediátrica. Metodología: Se realizó un estudio de casos y controles retrospectivo donde se analizaron todas las cirugías de tumores de fosa posterior que se realizaron en nuestro hospital entre enero del 2014 a mayo del 2020. Se incluyeron como casos a todos los pacientes que presentaron una infección del sistema nervioso central postoperatoria y como controles a los que pacientes que se operaron y no presentaron infección. Resultados: Al analizar los factores de riesgo encontramos que los pacientes con colocación de DVP (p=0.03) o DVE (p=0.005) tienen mayor chance de presentar infección del sitio quirúrgico en el postoperatorio. Mayor valor de Hemoglobina (p=0.002) parecería ser un factor preventivo. Las cirugías prolongadas (p<0.001) y la fístula a través de la herida (p=0.002) aumentan s las chances de infección del sitio quirúrgico en el postoperatorio. Conclusiones: Algunas estrategias que podrían ayudar a reducir el número de infecciones son: un adecuado control de la hidrocefalia prequirúrgica, preferentemente mediante TVE; reducir al mínimo el tiempo quirúrgico necesario para realizar el procedimiento; ingresar a quirófano con una hemoglobina adecuada y evitar la fístula de líquido por la herida

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PURPOSE: In neurogenesis, ASPM (abnormal spindle-like microcephaly-associated) gene is expressed mainly in the ventricular zone of posterior fossa and is the major determinant in the cerebral cortex. Besides its role in embryonic development, ASPM overexpression promotes tumor growth, including central nervous system (CNS) tumors. This study aims to investigate ASPM expression levels in most frequent posterior fossa brain tumors of childhood and adolescence: medulloblastoma (MB), ependymoma (EPN), and astrocytoma (AS), correlating them with clinicopathological characteristics and tumor solid portion size. METHODS: Quantitative reverse transcription (qRT-PCR) is used to quantify ASPM mRNA levels in 80 pre-treatment tumor samples: 28 MB, 22 EPN, and 30 AS. The tumor solid portion size was determined by IOP-GRAACC Diagnostic Imaging Center. We correlated these findings with clinicopathological characteristics and tumor solid portion size. RESULTS: Our results demonstrated that ASPM gene was overexpressed in MB (p = 0.007) and EPN (p = 0.0260) samples. ASPM high expression was significantly associated to MB samples from patients with worse overall survival (p = 0.0123) and death due to disease progression (p = 0.0039). Interestingly, two patients with AS progressed toward higher grade showed ASPM overexpression (p = 0.0046). No correlation was found between the tumor solid portion size and ASPM expression levels in MB (p = 0.1154 and r = - 0.4825) and EPN (p = 0.1108 and r = - 0.3495) samples. CONCLUSION: Taking in account that ASPM gene has several functions to support cell proliferation, as mitotic defects and premature differentiation, we suggest that its overexpression, presumably, plays a critical role in disease progression of posterior fossa brain tumors of childhood and adolescence.

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Central nervous system tumors are the most frequent solid neoplasms in childhood and are frequently located in posterior fossa (PF). In Brazil, this diagnosis is associated to high mortality rates and this context is increasingly worrisome outside of large urban centers in which delayed diagnosis and defaulting from treatment compromise survivorship. Moreover, the lesion and therapeutic toxicity compromise cognitive domains. This study investigated the impact of PF tumors and their treatments on the intellectual capacity of 37 children and adolescents aged 6 to 16, divided into two groups: patients with low grade tumors, submitted only to neurosurgery (G1) and with tumors of higher malignancy submitted to neurosurgery, chemotherapy, and/or radiotherapy (G2). Using the Wechsler Intelligence Scales for Children, data reveal that G1 presented preserved mean performance, while G2 presented low average performance. Cluster-type analysis divided the participants into two groups regarding intelligence, clinical and sociodemographic variables. Inferential statistical analysis highlighted the influence of antineoplastic treatment on nonverbal domains. Mother's schooling demonstrated influence on verbal domains, revealing an important dissociation pattern. The results suggest the relevance of sociocultural factors on the expression of the damage, as well as the administration of radiotherapy at critical neurodevelopmental stages.

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Rosette-forming glioneuronal tumor (RGNT) is a rare and distinct primary nervous system tumor. The literature on this novel neoplasm is sparse and limited to mostly case reports. Reviews on the characteristics of this tumor are fewer and far between with the latest up to a decade old. We thus provide a comprehensive review of recent literature to characterize presenting symptoms, radiological evidence, treatment options, and prognosis of this novel neoplasm. A Medline search for case reports detailing primary rosette-forming glioneuronal tumors was performed. RGNTs are a benign tumor of indolent course with mixed glial and neurocytic components. There is a slight female predominance with mean age of presentation at 23.57 years. Headaches, visual disturbances, and nausea and vomiting are the most common symptoms. Most RGNTs have solid and cystic components, arising most frequently in the fourth ventricle or cerebellum. Management is usually through surgery with gross total resection (GTR) providing better prognosis.

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