Intraventricular Glioma in Pediatric Patients: A Systematic Review of Demographics, Clinical Characteristics, and Outcomes.

OBJECTIVE: We conducted a systematic review on pediatric intraventricular gliomas to survey the patient population, tumor characteristics, management, and outcomes. METHODS: PubMed, Scopus, Web-of-Science, and Cochrane were searched using PRISMA guidelines to include studies reporting pediatric patients with intraventricular gliomas. RESULTS: A total of 30 studies with 317 patients were included. Most patients were male (54%), diagnosed at a mean age of 8 years (0.2-19), and frequently exhibited headache (24%), nausea and vomiting (21%), and seizures (15%). Tumors were predominantly located in the fourth (48%) or lateral ventricle (44%). Most tumors were WHO grade 1 (68%). Glioblastomas were rarely reported (2%). Management included surgical resection (97%), radiotherapy (27%), chemotherapy (8%), and cerebrospinal fluid diversion for hydrocephalus (38%). Gross total resection was achieved in 59% of cases. Cranial nerve deficit was the most common postsurgical complication (28%) but most were reported in articles published prior to the year 2000 (89%). Newer cases published during or after the year 2000 exhibited significantly higher rates of gross total resection (78% vs. 39%, P < 0.01), lower rates of recurrence (26% vs. 47%, P < 0.01), longer average overall survival time (42 vs. 21 months, P = 0.02), and a higher proportion of patients alive (83% vs. 70%, P = 0.03) than in older cases. CONCLUSIONS: Pediatric intraventricular gliomas correlate with parenchymal pediatric gliomas in terms of age at diagnosis and general outcomes. The mainstay of management is complete surgical excision and more recent studies report longer overall survival rates and less cranial nerve complications.

Primary intraventricular tumors - Imaging characteristics, post-treatment changes and relapses.

Primary intraventricular neoplasms are rare tumors that originate from the ependymal or subependymal, septum pellucidum, choroid plexus and the supporting arachnoid tissue. Knowledge of the common locations of these tumors within the ventricular system, together with key imaging characteristics and presentation age, can significantly narrow the differential diagnosis. In 2016, the WHO reorganized the classification of several primary CNS tumors by combining histopathological and molecular data. This study highlights the imaging characteristics, histopathological and molecular data, treatment strategies and post-treatment changes of primary intraventricular tumors. Molecular-based diagnosis can not only aid in patient stratification and personalized treatment, but it can also provide prognostic and predictive value independent of WHO classification.

Preoperative obliteration of choroidal arteries in the treatment of large hypervascular tumors in the lateral ventricle.

BACKGROUND: Removal of large hypervascular tumors in the lateral ventricle still poses a surgical challenge. These tumors are usually fed from choroidal arteries, and vascular control is typically performed late during the removal. We aimed to evaluate the clinical efficacy of our strategy for persistent preoperative obliteration of feeders from the choroidal arteries to manage large hypervascular tumors in the lateral ventricle. METHODS: We retrospectively analyzed six patients with hypervascular tumors in the lateral ventricle. We first attempted to obstruct feeders using endovascular treatment, and, if unavailable, performed initial microsurgical occlusion through the temporal horn for the staged tumor removal. RESULTS: In all patients, feeder obliteration was successfully performed; the anterior choroidal arteries were occluded by the endovascular treatment and microsurgical occlusion in one and five patients, respectively, while the lateral posterior choroidal arteries were occluded via endovascular treatment in four patients. No patients had permanent symptoms due to feeder obliteration, and tumor devascularization was achieved at the mean rate of 69.9%. During the tumor removal, the mean blood loss volume was 253 ml. No postoperative hemorrhage had occurred, and all patients scored ≤ 2 on the modified Rankin Scale at six months post-removal. CONCLUSIONS: Although further studies are warranted, persistent feeder obliteration of choroidal arteries could be an effective treatment strategy against large hypervascular tumors in the lateral ventricle.

Dissemination Patterns and Short-Term Management of Multifocal Rosette-Forming Glioneuronal Tumors.

BACKGROUND: Multifocal rosette-forming glioneuronal tumors (RGNTs) are challenging to manage. Gross total resection is often impossible, and data on adjunctive therapies are limited. We reviewed cases of multifocal RGNTs in the literature with special focus on dissemination patterns and management. METHODS: A literature review was conducted using PubMed and the key words "(multifocal OR multicentric OR satellite OR dissemination) AND glioneuronal." RESULTS: There were 21 cases of multifocal RGNTs identified. Follow-up was available in 18 cases at a median of 17 months. Progression-free survival and overall survival at 1 year were 84% and 94%, respectively. Of all cases, 43% had cerebrospinal fluid (CSF) dissemination, 48% had intraparenchymal spread, and 10% had both. The presence of CSF dissemination led to palliative care and/or death in 20% of cases (n = 2). None of the cases with intraparenchymal spread progressed. Radiotherapy was used in 50% of cases with CSF dissemination, chemotherapy was used in 20%, and CSF shunting was used in 36%. No tumors with intraparenchymal spread required adjunctive therapy or shunting. CONCLUSIONS: RGNTs with CSF dissemination are more likely to behave aggressively, and early adjunctive therapies should be discussed with patients. Tumors with intraparenchymal spread grow slowly, and maximal safe resection followed by observation is likely sufficient in the short term. Long-term behavior of multifocal RGNTs is still unclear.

Intraventricular choroid plexus tumors: clinical characteristics and impact of current management on survival.

INTRODUCTION: Choroid plexus tumors (CPTs) represent one of the most common intraventricular tumors. Although most are benign, they often reach considerable sizes before clinical manifestation, challenging their surgical management. We aim to describe the clinical characteristics and the impact of current management on the survival of patients harboring intraventricular CPT. METHODS: The National Cancer Database (NCDB) was queried to identify biopsy-proven intraventricular CPT patients (2004-2015). Demographic and patterns of care were described, the log-rank method was used to independently analyze survival according to age, WHO grade and extent of resection (EOR). Multivariate analysis was performed to investigate the impact of prognostic factors on overall survival (OS). RESULTS: A total of 439 CPT patients with known WHO grade were included. WHO grade I tumors were more frequent in adults, while WHO grade III tumors were more common in pediatric population. Most CPTs were benign, with a median tumor size of 3-4 cm. Mean tumor size in pediatric population was greater than in adult population (4.39 cm vs. 2.7 cm; p < 0.01). Frequency was similar between males and females (51.7% vs. 48.3%; p > 0.0.5). Five- and ten-year OS among all patients was 87% and 84%, respectively. EOR was not associated with survival for any WHO grade. On multivariable analysis, only patient age (p = 0.022), WHO grade (p = 0.003) and medical comorbidity scores (p = 0.002) were independently associated with OS after diagnosis. CONCLUSION: Patients with CPTs present at different stages of life, with sizable tumor burden and distinct WHO grade prevalence. Considering their favorable survival, efforts to improve tumor control should be meticulously weighed against the long-term risk associated with surgery, radiation, and chemotherapy.

Radiation-Induced Brain Aneurysms: Institutional Experience and State of the Art in the Contemporary Literature.

BACKGROUND: Brain aneurysms (BAs) are the most common intracranial vascular condition, with an overall incidence of 1%-2%. Among the common causes of their initial formation and growth, the role of radiation therapy (RT) has been reported in some studies. The aim of the present study is to report the most relevant features of BA related to a previous cranial RT. METHODS: Data deriving from 1 patient treated for RT-induced BA in our institution were added to reports of another 66 BAs retrieved from the literature. The following parameters were evaluated: age, sex, location, primary lesion, clinical presentation, dosage/amount of radiation delivered, type of treatment for the BA, dimension, morphology, chemotherapy, comorbidities, risk factors, and number of BAs. RESULTS: The most commonly involved vessel was the internal carotid artery (34%). In general, the anterior circulation showed higher vulnerability compared with the posterior circulation and middle cerebral artery (56.7%). The average latency between RT and the first imaging showing the BA was 9.01 ± 6.85 years. Vessels coursing in the posterior cranial fossa showed a significant univariate association with lower X-ray dosages (P = 0.014) compared with the other locations. No statistically significant correlation between the continuous variables age, latency of BA appearance, RT delivered dose, and dimension of the BA was shown. CONCLUSIONS: The apparent higher fragility of the vascular structures of the posterior cranial fossa was statistically outlined, and the X-ray dosage, the primary condition target of the RT, the age of the patients, and no statistically significant correlation were outlined. Biological factors could play a significant role.

Surgical Management of Isolated Fourth Ventricular Hydrocephalus Associated with Injury to the Guillain-Mollaret Triangle.

BACKGROUND: The occurrence of isolated fourth ventricle and injury to the Guillain-Mollaret triangle in the setting of posterior fossa ependymoma represents a new association. In this case report, we discuss the clinical, theoretical, and therapeutic aspects of this problem. We describe a lateral transcerebellar trajectory and shunt valve configuration for safe fourth ventricle shunting in a patient with prior posterior fossa surgery. CASE DESCRIPTION: A 45-year-old woman underwent subtotal resection of a fourth ventricle ependymoma (World Health Organization grade III) followed by radiation therapy to control the residual tumor. Her course was complicated by a cerebral abscess and subsequent communicating hydrocephalus, for which she received a lateral ventriculoperitoneal shunt. After placement of the lateral ventricle shunt, there was a progressive increase in the volume of the fourth ventricle over the next 2 years, from 2.5 to 12.0 mL. She developed palatal myoclonus, hand incoordination, bilateral foot numbness, and progressive ataxia. Neuroimaging also revealed hypertrophic degeneration of the inferior olivary nuclei bilaterally. The isolated fourth ventricle was treated by a separate fourth ventriculoperitoneal shunt inserted through a lateral transcerebellar trajectory. A programmable variable pressure valve was implemented. CONCLUSIONS: Development of an isolated fourth ventricle and injury to the Guillain-Mollaret triangle in the setting of fourth ventricular ependymoma is a newly encountered complication. Choice of treatment modality and timing of intervention should be carefully considered on a case-by-case basis. The data presented in this report may assist in the selection of surgical treatment for isolated fourth ventricle.

Survival of Ventricular and Periventricular High-Grade Gliomas: A Surveillance, Epidemiology, and End Results Program-Based Study.

BACKGROUND: Aggressiveness of surgical resection for periventricular/ventricular high-grade gliomas (HGGs) is determined by operative risks and assumed effectiveness of radiation therapy (RT) on residual tumor. We aimed to clarify the impact of surgery and postoperative RT on patient survival in a population-based study. METHODS: This population-based study used the Surveillance, Epidemiology, and End Results (SEER) database. Patients with ventricular malignant tumors were screened for HGGs. In accordance with the World Health Organization (WHO) 2016 classification, we included cases with "diffuse astrocytic and oligodendroglial tumors," "other astrocytic tumors," "ependymal tumors," and "other gliomas". Tumor grading followed definitions established by the WHO with supplementation from SEER classifications. Only grades III and IV were included. Individual factors were assessed by hazard ratio (HR) from multivariable survival analysis using accelerated failure time (AFT) regression. RESULTS: We included 353 patients after application of inclusion and exclusion criteria. The mean patient age was 38.77 ± 24.95 years, and the cohort was 61.5% male. Overall median survival was 12 months, with notable improvement over the last 3 decades. In a multivariate AFT model, older age (per 10-year increase, HR, 1.19; P < 0.001) was the sole nontreatment variable found to predict survival, whereas postoperative RT had a significant survival benefit (HR, 0.50; P < 0.001). No tumor characteristic (e.g., size, extent of invasion) predicted prognosis. Interestingly, neither partial resection nor TR/GTR was associated with improved outcome. CONCLUSIONS: The prognosis of ventricular HGGs is poor, with worse prognosis in older patients. We found no evidence to support aggressive surgical resection. Postoperative chemoradiation should be administered; however, the benefit of modification of the protocol for chemoradiation specifically for ventricular HGGs remains unknown and warrants further investigation.

Clinical, radiological, pathological and prognostic aspects of intraventricular oligodendroglioma: comparison with central neurocytoma.

Studies comparing intraventricular oligodendroglioma (IVO) and central neurocytoma (CN) in terms of their clinical, radiological and pathological features are scarce. We, therefore, investigated the similarities and differences between these types of tumors to get a better understanding of how they may be more properly diagnosed and treated. The clinical manifestations, CT/MRI findings, pathological characteristics and clinical outcomes of 8 cases of IVOs and 12 cases of CNs were analyzed retrospectively. Both IVO and CN occurred most commonly in young adults and manifested with symptoms of increased intracranial pressure secondary to obstructive hydrocephalus. However, they were radiologically different in location (p = 0.007), diffusion-weighted imaging (p = 0.001), "scalloping" appearance (p = 0.006), flow void sign (p = 0.006) and ventricular wall invasion (p = 0.000). Histologically, significant differences in mitotic count (p = 0.008) and parenchymal infiltration (p = 0.01) were noted. Immunohistochemically, significant differences in the expression of Olig2 (p = 0.000), Syn (p = 0.01) and NeuN (p = 0.000) were observed. In addition, MIB-1 labeling index (p = 0.035) and case fatality rate (p = 0.021) of IVO were much higher than those of CN, while survival rate of IVO was much lower than that of CN (p = 0.028). IVO and CN are similar in onset age and clinical manifestations, but have different imaging and pathological features. Patients with IVOs may have a relatively poorer prognosis compared to those with CNs.

Giant Cell Ependymoma of Lateral Ventricle: Case Report, Literature Review, and Analysis of Prognostic Factors and Genetic Profile.

BACKGROUND: Giant cell ependymoma (GCE) is a rare primary central nervous system neoplasm. We report a case of GCE arising in the lateral ventricle. CASE DESCRIPTION: A 22-year-old female presented with generalized seizures. Magnetic resonance imaging demonstrated a diffuse, nonenhancing, multicystic mass centered in the atrium of the right lateral ventricle with extension throughout the frontal and temporal horns. An initial subtotal resection yielded the signature biphasic pattern of GCE. The dominant component contained pleomorphic, bizarre-appearing giant cells with low mitotic index, and a minor component comprised monomorphic, highly cellular, mitotically active cells that formed perivascular pseudorosettes. Array-comparative genomic hybridization showed copy number abnormalities consistent with chromosomal instability without evidence of RELA- or YAP1-fusion-features most often seen in posterior fossa ependymoma group B. Given expectedly poor radiation sensitivity, a second-look surgery was undertaken to minimize residual before proton beam radiotherapy. LITERATURE REVIEW: Review of the literature identified 28 reported cases, with a median age of 34 and bimodal peaks at approximately 20 and 50 years of age, including 9 supratentorial, 5 infratentorial, and 15 spinal ependymomas. Two infratentorial cases involved the fourth ventricle; no cases arose from the third or lateral ventricles. Supratentorial tumors predominated in younger patients, whereas other locations were observed among older patients (21.6 vs. 46.3 years of age; P = 0.01). Cases with Ki-67 index ≥10% showed worse progression-free survival than those of <10% (P = 0.049). CONCLUSION: Although rare, GCE should be considered in the differential of young patients with atypical intraventricular lesions, particularly given that extent of resection is associated with increased survival and GCE is thought to be radiation resistant.

Management in chordoid glioma: Avoiding the pitfalls in this rare and challenging entity.

Chordoid glioma (CG) of the third ventricle is an unusual neoplasm of glial nature, which is almost exclusively located in the anterior wall of the third ventricle, in close relation with the hypothalamus. Magnetic resonance images show CG as a suprasellar, hypo- to isointense mass, homogeneously enhancing after the administration of gadolinium. Since its description in 1998 by Brat et al., approximately 85 cases have been reported. Some of its pathological features are under discussion and its histological origin still remains unclear. In this study, we present a patient having this rare entity. We review the management of CG reported in literature. We also studied its pathological features, the postoperative mortality and morbidity related to radical surgical resection, and the implemented adjuvant therapies. Due to its classical clinical features and its close resemblance to other lesions in the region, it is an entity unlikely to be suspected prior to its histological diagnosis. Despite the benign nature of this tumor, the clinical outcome might be poor. Its treatment may represent a real challenge because it involves critical anatomical areas, leading to high postoperative morbidity and mortality rates. An initial minimally invasive management and adjuvant therapies, such as radiosurgery, in case of symptomatic recurrences, can be effective handling strategies.

Rosette-forming glioneuronal tumor outside the fourth ventricle: a case-based update.

INTRODUCTION: Rosette-forming glioneuronal tumors (RGNTs) that do not involve the fourth ventricle are rare. RGNTs were originally thought to be exclusively localized into the fourth ventricle but were found in various anatomical localizations. MATERIAL AND METHODS: We review the literature and found 32 cases of this particular RGNT. The outcome was excellent with no mortality reported after surgical treatment. Only two patients had received adjuvant therapy for progression. We added one case of a RGNT located in the cerebellar hemisphere. CASE AND CONCLUSION: She underwent a subtotal removal with no evidence of progression after. This WHO grade I tumor with a specific biphasic histopathology is of a good oncological outcome after surgical treatment. A long follow-up is needed as recurrence or metastatic progressions exist.

Evaluation of treatment response using integrated 18F-labeled choline positron emission tomography/magnetic resonance imaging in adolescents with intracranial non-germinomatous germ cell tumours.

The efficacy of hybrid 18F-Fluroethyl-Choline (FEC) positron emission tomography (PET)/magnetic resonance imaging (MRI) was investigated as an imaging modality for diagnosis and assessment of treatment response and remission status in four patients with proven or suspected intracranial non-germinomatous germ cell tumours (NGGCT). In two patients faint or absent choline avidity correlated with negative histology, whereas in other two patients, persistent choline avidity in the residual mass was suggestive of presence of viable tumour, subsequently confirmed histologically. We conclude that FEC-PET/MRI may be an effective imaging tool in detecting viable residual tumour in patients with intracranial NGGCT post treatment.

Análisis coste-efectividad de la cirugía del glioma maligno guiada por fluorescencia con ácido 5-aminolevulínico / Cost-effectiveness of 5-aminolevulinic acid-induced fluorescence in malignant glioma surgery

Objetivo: Evaluar el coste-efectividad del ácido 5-aminolevulínico (5-ALA, Gliolan(R)) en pacientes intervenidos quirúrgicamente de glioma maligno, en condiciones de práctica médica habitual en España. Material y métodos: Se determinaron las ratios de coste incremental por resección completa (RC) y de coste incremental por año de vida ajustado por calidad (AVAC) ganado, sobre la base de los datos recogidos en el estudio observacional VISIONA. Resultados. El coste incremental con 5-ALA frente a la cirugía convencional con luz blanca asciende a 4.550 Euros por RC adicional conseguida y a 9.021 Euros por AVAC ganado. Estos resultados se muestran consistentes en un análisis de sensibilidad. Conclusión: La cirugía del glioma maligno guiada por fluorescencia con 5-ALA conlleva un incremento de costes moderado respecto a la práctica quirúrgica actual y muestra una relación coste-efectividad favorable

Objective: This study evaluates the cost-effectiveness of 5-aminolevulinic acid (5-ALA, Gliolan®) in patients undergoing surgery for malignant glioma, in standard clinical practice conditions in Spain. Material and methods: Cost-effectiveness ratios were determined in terms of incremental cost per complete resection (CR) and incremental cost per additional quality-adjusted life year (QALY), based on data collected in the VISIONA observational study. Results: Incremental cost with 5-ALA versus conventional surgery using white light only amounts to Euros 4550 per additional CR achieved and Euros 9021 per QALY gained. A sensitivity analysis shows these results to be robust. Conclusion: Malignant glioma surgery guided by 5-ALA fluorescence entails a moderate increase in hospital costs compared to current surgical practice and can be considered a cost-effective innovation

A comparative study of intraventricular central neurocytomas and extraventricular neurocytomas.

Similar histology and clinical behavior of both intraventricular central neurocytomas (CNs) and extraventricular neurocytomas (EVNs) may argue against the idea that EVNs were the distinct entity to distinguish from CNs in the 2007 World Health Organization classification. To explore respective characteristics and compare similarities and differences in CNs and EVNs, relevant clinical, radiological, operative and pathological data of 49 patients (35 CNs and 14 EVNs) in the Department of Neurosurgery at our hospital from 2005 to 2012 was reviewed and some comparisons between CNs and EVNs were conducted. The factors affecting posttreatment recurrence of CNs and EVNs were assessed by Cox regression analysis. In comparison, CNs showed a more typical clinical manifestation, and radiological and histopathological features, while EVNs demonstrated more malignant biological behavior, with higher MIB-1 index (p = 0.006), higher rate of atypia (p = 0.042), higher recurrence rate (p = 0.028), and shorter time to recurrence (p = 0.049). Subtotal resection was associated with higher rates of recurrence in both CNs (hazard ratio [HR] 6.16, p = 0.046) and EVNs (HR 5.26, p = 0.045), and atypia was also associated with a higher recurrence rate in CNs (HR 5.03, p = 0.042). CNs were thus easier to diagnose than EVNs, with typical clinical, radiological, and histopathological features, while the latter were more likely to show malignant biological behavior associated with atypia and recurrence. Total surgical resection is the optimal treatment choice for both CNs and EVNs, and patients with either CN or EVN with typical and/or totally resected lesions showed favorable clinical outcomes.

Rosette-forming glioneuronal tumour (RGNT) of the fourth ventricle: a highly aggressive case.

BACKGROUND: The rosette-forming glioneuronal tumour (RGNT) is a rarely encountered tumour that has been included as a new entity in the 2007 edition of the "World Health Organization (WHO) Classification of Tumours of the Central Nervous System". We describe a rather unusual case of multifocal cerebellar RGNT, located in the spinal cord and displaying leptomeningeal spread. CLINICAL PRESENTATION: Twenty-four-year-old male with history of long-lasting headaches. A magnetic resonance scan revealed three heterogeneous lesions located within both cerebellar hemispheres and the left cerebellopontine angle, in addition to a spinal cord lesion at the level of the cervical region, and images of leptomeningeal spread. Interventions were performed in two stages; these involved resection of two cerebellar lesions, with a histopathological diagnosis of RGNT with atypical microvascular proliferation and focal necrosis. Although these tumours appear to be benign, our case debuted in an aggressive form, both from the radiological point of view and with respect to its histopathological characteristics. For this reason, the patient received adjuvant therapy with chemotherapy and radiotherapy. CONCLUSIONS: Experience of RGNT is limited. The prognostic significance of the histological findings of vascular proliferation and necrosis is still unknown. The clinical improvement in our patient endorses our decision to perform aggressive treatment.

Lateral ventricle haemangioblastoma: the role of perfusion scanning and embolisation in diagnosis and management.

We present the case of a 24-year-old man who presented with vertigo and right-sided weakness. Subsequent imaging demonstrated a lateral ventricle haemangioblastoma. This is the first case ever to be treated with surgical excision augmented by preoperative endovascular embolisation, as illustrated with perfusion CT scanning performed pre-embolisation and postembolisation. We present the case followed by a summary of the previously published cases and a discussion of the advantages of perfusion scanning and endovascular embolisation in these highly vascular (and therefore potentially dangerous) lesions.