Intraventricular Glioblastomas

doi:10.1016/j.wneu.2015.12.079

World Neurosurgery

Volume 88, April 2016, Pages 126-131

https://doi.org/10.1016/j.wneu.2015.12.079 Get rights and content

Background and Importance

Although glioblastoma is the most common primary brain tumor, primary intraventricular locations are extremely rare; only 21 cases have been reported to date.

Methods

A retrospectively acquired database of all intracranial glioblastomas treated in 2 different neurosurgical departments during the last 10 years was queried. Patients with histologically proven intraventricular glioblastomas were included in the study.

Results

Eight patients were identified as having a histologically confirmed intraventricular glioblastoma. Patient age at diagnosis ranged from 6 to 74 years (mean 29.6 years) and the male/female ratio was 5:3. Increased intracranial pressure due to hydrocephalus was the main cause of the clinical manifestations. The tumor was located within the lateral ventricle in 6 cases and the anterior third ventricle in 2 others. Gross total tumor excision was achieved in 3 patients, whereas the surgical resection was subtotal in 4 cases and a surgical biopsy was performed in 1 patient. Postoperative adjuvant therapies were administered in 5 patients. Median survival time was 32.1 months, and 3 patients were alive at the end of study. All of them had isocitrate dehydrogenase−mutated tumors.

Conclusions

Intraventricular glioblastoma is extremely rare and can affect younger individuals including children. This malignant tumor should be included in the differential diagnosis of intraventricular lesions, especially in the lateral ventricles. Radical surgical resection can be associated with remarkable disease-free survival, especially in isocitrate dehydrogenase−mutated tumors. Because recurrence virtually is unavoidable, long-term follow-up is mandatory.

Introduction

Glioblastoma multiforme (GBM) is the most common primary brain tumor, representing approximately 25% of all adult intracranial tumors¹ and typically occurring during the sixth or seventh decade of life. Although the tumor can arise anywhere in the central nervous system (CNS), the frontotemporal region of the cerebral cortex is preferentially affected, encompassing nearly two thirds of cases.² Primary intraventricular locations are extremely rare. To the best of our knowledge, only 21 cases have been documented to date. We present 8 cases of histologically proven intraventricular glioblastomas treated during the last 10 years and briefly review the relevant literature.

Section snippets

Materials and Methods

After obtaining approval for the study from their institutional ethics committees, we analyzed retrospectively the clinical charts, operative conditions, and outcome of 8 patients diagnosed with intraventricular glioblastoma treated between 2005 and 2014 at the Department of Neurosurgery of The Tunisian National Institute of Neurology, Tunis, Tunisia and the Department of Neurosurgery of Fattouma Bourguiba University Hospital, Monastir, Tunisia. For diagnostic accuracy, all identified cases

Results

The sample consisted of 5 male and 3 female patients between 6 and 74 years of age (mean 29.6 years), representing approximately 0.07% of all glioblastomas treated during that period (8/1148). Patient details are summarized in Table 1. Signs of increased intracranial pressure were observed in almost all cases (7/8; 87.5%). Other presenting symptoms included mental disturbance or a Medical Research Council Scale 2/5 motor deficit (Cases 1 and 2). Seizure was a presenting symptom in only 1 case

Discussion

GBM is the most common and most aggressive malignant primary brain tumor in humans, accounting for approximately 25% of all intracranial tumors and 50% of all glial tumors in adults.¹ Although these tumors can arise anywhere in the CNS, the cerebral cortex of the frontal and the temporal lobes comprise nearly two thirds of cases.² GBMs arising within the intraventricular system are very rare.

We performed a PubMed search using the keywords “intraventricular glioblastoma” and “intraventricular

Conclusions

GBM usually shows rapid progression and recurrence after treatment; however, intraventricular GBMs might have a better prognosis, especially in children, and the clinical awareness of this rare entity is helpful in formulating the complete differential diagnosis. Gross total resection, when feasible, should be the goal and can be associated with extended disease-free survival, especially in case of IDH-mutated tumors. A multimodal treatment strategy combining surgery plus radiation−chemotherapy

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Cited by (17)

Pathology of intraventricular tumors
2019, Comprehensive Overview of Modern Surgical Approaches to Intrinsic Brain Tumors
Intraventricular tumors are rare and comprise a variety of histological entities. They may be found throughout the ventricular system, which is comprised broadly of the lateral ventricles, third ventricle, cerebral aqueduct, and fourth ventricle. Given the widespread and varied topography of the ventricular system, its presence in the supratentorial and infratentorial compartments, as well as its diverse cellular constituency, it is not surprising that tumors arising from this complex are quite heterogeneous in location, clinical consequence, and biological behavior. The diverse nature of intraventricular tumors makes their classification more challenging than the standard intra-axial versus extra-axial categorization applied to many central nervous system lesions.
Survival of Ventricular and Periventricular High-Grade Gliomas: A Surveillance, Epidemiology, and End Results Program–Based Study
2018, World Neurosurgery
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.
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.
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.
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.
Adult Brain Tumors and Pseudotumors: Interesting (Bizarre) Cases
2016, Neuroimaging Clinics of North America
Citation Excerpt :
Imaging features of IVGB are similar to those located in extraventricular locations. On CT they present as mixed density masses with contrast enhancement of the non-necrotic areas.68 On MRI, T1WIs shows an infiltrative mass with irregular borders and mixed signal intensity.
Case Study of a Complex Neurovascular Disorder: Choroidal Arteriovenous Malformation
2024, Medicina (Lithuania)
Management and Molecular Characterization of Intraventricular Glioblastoma: A Single-Institution Case Series
2023, International Journal of Molecular Sciences
Machine learning model to identify prognostic factors in glioblastoma: a SEER-based analysis
2023, Pamukkale Medical Journal

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: Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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Original ArticleIntraventricular Glioblastomas

Background and Importance

Methods

Results

Conclusions

Introduction

Section snippets

Materials and Methods

Results

Discussion

Conclusions

Surg Neurol

Clin Neurol Neurosurg

Neurocirugia (Astur)

Eur J Radiol

Intraventricular glioblastoma multiforme with previous history of intracerebral hemorrhage: a case report

J Korean Neurosurg Soc

Intraventricular glioblastoma multiforme with the pneumographic characteristics of intraventricular epidermoids; a case report with a critical analysis

Radiology

Intraventricular tumours: syndrome of the trigone

Can Med Assoc J

Third ventricular glioblastoma multiforme: case report

Neurosurg Rev

Well-circumscribed, minimally enhancing glioblastoma multiforme of the trigone: a case report and review of the literature

AJNR Am J Neuroradiol

Intraventricular glioblastoma: a paediatric case report

Br J Neurosurg

Glioblastoma multiforme of the lateral ventricle: report of nine cases

Br J Neurosurg

Original Article
Intraventricular Glioblastomas