Cerebellar Glioblastoma Multiforme Research Articles

OLIG2 expression level could be used as an independent prognostic factor for patients with cerebellar Glioblastoma (cGBM)

ObjectivesThe incidence of cerebellar Glioblastoma Multiforme (cGBM) is rare. Database like TCGA have not distinguish cGBM from GBM, our knowledge on cGBM gene expression characteristics is limited. The expression status of Oligodendrocyte Lineage Transcription factor 2 (OLIG2) and its clinical significance in cGBM is still unclear. MethodsThe clinical data and tissue specimens of 73 cGBM patients were retrospectively studied. The association between OLIG2 expression level and the demographic characteristics of cGBM patients was identified by the Chi-Square test. The survival curves were drawn by Kaplan-Meier analysis. The independent prognostic factors was calculated according to Cox regression analysis. ResultsThe OLIG2 high expression was observed in about 57.5% (42/73) of the cGBM patients. Patients with high OLIG2 expression levels had a higher alive ratio at the end of follow-up (alive ratio: 70.6% vs. 29.4%, p = 0.04). The median survival time was 21 months and 13 months for high and low expression of OLIG2 (p < 0 .05). Univariate analysis and Multivariate analysis indicated that EOR (HR = 3.89, 95% CI 1.23‒12.26, p = 0.02), low OLIG2 expression (HR = 5.26, 95% CI 1.13‒24.59, p = 0.04), and without adjuvant therapy (HR = 4.95, 95% CI 1.22‒20.00, p = 0.03) were independent risk factors for the OS of cGBM patients. ConclusionHigh expression level of OLIG2 could be used as an independent favorable prognosis indicator in cGBM patients and be recognized as a characteristic biomarker of cGBM.

Cerebellar glioblastoma multiforme in an adult patient with neurofibromatosis type 1: an extremely rare report with review of literature

BackgroundNeurofibromatosis type 1 (NF1) is a multisystem genetic disorder with autosomal dominant inheritance which predisposes the affected individuals to increased risk of developing certain benign and malignant central nervous system (CNS) tumors. NF1 patients are most notably prone to develop low-grade optic pathway, brainstem, and cerebellar astrocytoma. Current literature suggests that brain tumors in patients with NF1 tend to be less aggressive compared to sporadic ones. Glioblastoma multiforme (GBM) is a high-grade glioma which is relatively rare in patients with NF1 and is most commonly seen in supratentorial regions of the brain.Case presentationA 33-year-old patient was admitted in neurosurgery ward with acute hydrocephalus caused by a cerebellar mass lesion. On primary assessment, the patient was diagnosed with NF1. He was followed for 2 months and underwent surgical resection of the mass due to worsening symptoms. The pathology report revealed the malignant nature of the lesion. Patient received adjuvant chemo-radiotherapy with diagnosis of cerebellar GBM. Up to 19 months following surgery, he had gained a relatively well ability to walk and talk again.

Cerebellar glioblastoma in an NF1 patient. Is it surgical debulking really necessary?

Introduction: Neurofibromatosis type 1 is an autosomal dominant tumour syndrome with an increased risk of developing central nervous system neoplasms, mostly benign low-grade gliomas involving the optic pathway and the brainstem. High-grade astrocytomas or glioblastoma multiforme (GBM) are rare. Cerebellar GBMs are rarer still, only seven cases NF1 patients have been reported Case description: We report a case of a cerebellar GBM in a 25-year-old male with NF1 who survived 18 months from the initial diagnosis without surgical debulking but only chemo and radiotherapy.Conclusion: A literature review found that the best outcomes were achieved in NF1 patients who didn’t undergo gross surgical resection. Possible reasons and future directions are discussed.

Primary glioblastoma of the cerebellar vermis: A case report.

Cerebellar glioblastoma is a rare adult tumor. The accurate diagnosis of cerebellar glioblastoma is important for establishing a suitable therapeutic schedule. However, it is occasionally difficult to diagnosis these tumors. Clinical presentation, computed tomography (CT) and magnetic resonance imaging can provide useful information, but they may not lead to a definitive diagnosis. Positron emission tomography/computed tomography (PET/CT) may provide a novel way of forming a differential diagnoses. The lesions of glioblastoma multiforme (GBM) rarely occur in the cerebellum, with prior studies reporting that only 0.4-3.4% of all GBM tumors occur here. In the current study, a case of primary cerebellar glioblastoma is presented and the physiopathology, clinical presentation, diagnosis, differential diagnosis, treatment and general outcome of this disease are discussed. A 61-year-old female presented with nausea, vomiting, balance problems and cerebellar signs. Cranial magnetic resonance imaging (MRI) and positron emission tomography/computed tomography (PET/CT) examination demonstrated one regular contour of a mass lesion in the cerebellar vermis. Following surgery, glioblastoma was histologically confirmed. The outcome of the patient was favorable after 18 months of follow-up. Cerebellar GBM should be considered in the differential diagnosis of a cerebellar mass lesion, and PET/CT may provide a novel identification method for different cerebellar mass lesions.

Cerebellar giant cell glioblastoma multiforme in an adult

ABSTRACTCerebellar glioblastoma multiforme (GBM) is a rare tumor that accounts for only 1% of all cases of GBM and its giant cell variant is even much rarely encountered in adults. A case of cerebellar giant cell GBM managed at our institution reporting its clinical presentation, radiological and histological findings, and treatment instituted is described. In conjunction, a literature review, including particular issues, clinical data, advances in imaging studies, pathological characteristics, treatment options, and the behavior of such malignant tumor is presented. It is very important for the neurosurgeon to make the differential diagnosis between the cerebellar GBM, and other diseases such as metastasis, anaplastic astrocytomas, and cerebellar infarct because their treatment modalities, prognosis, and outcome are different.

Outcome and prognostic factors in adult cerebellar glioblastoma

Cerebellar glioblastoma multiforme (GBM) occurs rarely in adults, accounting for 0.4–3.4% of all GBM. Current studies have all involved small patient numbers, limiting the clear identification of prognostic factors. Additionally, while few studies have compared cerebellar GBM to their supratentorial counterparts, there is conflicting data regarding their relative prognosis. To better characterize outcome and identify patient and treatment factors which affect survival, the authors analyzed cases of adult cerebellar GBM from the Surveillance, Epidemiology, and End Results database. A total of 247 adult patients with cerebellar GBM were identified, accounting for 0.67% of all adult GBM. Patients with cerebellar GBM were significantly younger than those with supratentorial tumors (56.6 versus 61.8years, p<0.0001), but a larger percentage of patients with supratentorial GBM were Caucasian (91.7% versus 85.0%, p<0.0001). Overall median survival did not differ between those with cerebellar and supratentorial GBM (7 versus 8months, p=0.24), with similar rates of long-term (greater than 2years) survival (13.4% versus 10.6%, p=0.21). Multivariate analysis revealed age greater than 40years (hazard ratio [HR]: 2.20; 95% confidence interval [CI]: 1.47–3.28; p=0.0001) to be associated with worse patient survival, while the use of radiotherapy (HR: 0.33; 95% CI: 0.24–0.47; p<0.0001) and surgical resection (HR: 0.66; 95% CI: 0.45–0.96; p=0.028) were seen to be independent favorable prognostic factors. In conclusion, patients with cerebellar GBM have an overall poor prognosis, with radiotherapy and surgical resection significantly improving survival. As with supratentorial GBM, older age is a poor prognostic factor. The lack of differences between supratentorial and cerebellar GBM with respect to overall survival and prognostic factors suggests these tumors to be biologically similar.

Cerebellar glioblastoma multiforme: a retrospective study of 28 patients at a single institution

Cerebellar glioblastoma multiforme (CGBM) is rare and its treatment is ill defined. To elucidate prognostic factors, we performed a single institutional review of the largest series to date of CGBM. The West China Hospital database was reviewed from 2007 to June 2011, and a total of 28 CGBM patients were collected. Median age of patients was 50 years old, whereas median Karnofsky Performance Status was 80 (range, 30–100). Brainstem invasion was observed in 9 patients. Sixteen patients received total resection and 12 patients received subtotal resection. Postoperative Gamma Knife radiosurgery (GKRS) was administered to 22 patients. After operation, 16 patients received concurrent GKRS and chemotherapy, and 6 patients received chemotherapy after GKRS. In addition, 4 patients only received chemotherapy, and 2 patients did not receive either GKRS or chemotherapy due to various reasons. Median follow-up period was 13.7 months (range, 5.2–28.1 months). Median overall survival (OS) of 28 patients was 14.3 months and median progression-free survival (PFS) was 9.4 months. Univariate log-rank analysis showed that OS and PFS were significantly related to brainstem invasion (p = 0.03, p = 0.04, respectively), extent of resection (p = 0.02, p = 0.04, respectively) and GKRS (p = 0.01, p = 0.02, respectively) of GBM. Multivariate analysis revealed that OS and PFS were also significantly associated with brainstem invasion (p = 0.007, p = 0.014, respectively), extent of resection (p = 0.032, p = 0.045, respectively) and GKRS (p = 0.031, p = 0.046, respectively) of CGBM. According to our study, brainstem invasion, extent of resection and GKRS were major prognostic factors for survival. Combination of postoperative GKRS and chemotherapy had an improved prognosis, and it may be a feasible postoperative adjuvant treatment of CGBM.

Comparison of Survival Between Cerebellar and Supratentorial Glioblastoma Patients

Cerebellar glioblastoma multiforme (cGBM) is rare, and although there is a general belief that these tumors have a worse prognosis than supratentorial GBM (sGBM), few studies have been published to support this belief. To investigate the effect of cerebellar location on survival through a case-control design comparing overall survival time of cGBM and sGBM patients. The Surveillance, Epidemiology, and End Results (SEER) registry was used to identify 132 patients with cGBM (1973-2008). Each cGBM patient was matched with an sGBM patient from among 20,848 sGBM patients on the basis of age, extent of resection, decade of diagnosis, and radiation therapy using propensity score matching. Within the cGBM, 37% were older than 65 years of age, 62% were men, and 87% were white. Most patients underwent surgery and radiation (74%), whereas only 26% underwent surgical resection only. The median survival time for the cGBM and sGBM matched cohort was 8 months; however, the survival distributions differed (log-rank P = .04). Survival time for cGBM vs sGBM at 2 years was 21.5% vs 8.0%, and 12.7% vs 5.3% at 3 years. Multivariate analysis of survival among cGBM patients showed that younger age (P < .0001) and having radiation therapy (P < .0001) were significantly associated with reduced hazard of mortality. Among all patients, multivariate analysis showed that tumor location (P = .03), age (P < .0001), tumor size (P = .009), radiation (P < .0001), and resection (P < .0001) were associated with survival time in the unmatched cohort. Median survival time for cGBM and sGBM patients was 8 months, but cGBM patients had a survival time advantage as the study progressed. These findings suggest that cGBM patients should be treated as aggressively as sGBM patients with surgical resection and radiation therapy.

A retrospective study of primary cerebellar glioblastoma multiforme in adults

Primary cerebellar glioblastoma multiforme (GBM) is a rare tumour in adults that accounts for less than 1% of all patients with GBM. In view of their rarity, the pathogenesis and prognosis of cerebellar GBM are not yet completely understood. The aim of this study was to retrospectively analyse patients with primary cerebellar GBM treated in our institute over a period of 10years. Data from the case records of five adult patients with cerebellar GBM was evaluated and their outcome was assessed. We observed local failure in patients who reported back with recurrence. The presence of brainstem infiltration was a significant factor influencing progression-free survival. The overall prognosis was worse than for patients with supratentorial GBM. In view of their rarity, a meta-analysis is required to assess the pathogenesis and prognostic factors affecting overall survival in patients with cerebellar GBM.

Primary glioblastoma of the cerebellum in a 19-year-old woman: a case report

IntroductionCerebellar glioblastoma is an uncommon adult lesion. The pathogeny and prognosis of cerebellar glioblastoma are still incompletely elucidated.Case presentationWe report the case of a 19-year-old Moroccan woman. A neurologic examination revealed the presence of cerebellar signs. A diagnosis of cerebellar glioblastoma is rarely made before surgery. An encephalic computer tomography scan and magnetic resonance imaging increased the accuracy of defining the nature of the lesion. Magnetic resonance imaging showed a heterogeneously enhancing mass in the vermis and the right cerebellar hemisphere. After surgery, glioblastoma was histologically confirmed. Post-operative radiotherapy followed. The outcome of our patient was favorable after one year of follow-up.ConclusionsWe report an unusual case of cerebellar gliobastoma. Cerebellar glioblastoma multiforme should be considered in the differential diagnosis of a cerebellar mass lesion.

The Rare Cancer Network: Achievements from 1993 to 2012

The Rare Cancer Network (RCN), founded in 1993, performs research involving rare tumors that are not common enough to be the focus of prospective study. Over 55 studies have either been completed or are in progress.The aim of the paper is to present an overview of the 30 studies done through the RCN to date, organized by disease site. Five studies focus on breast pathology, including sarcoma, lymphoma, phyllodes tumor, adenoid cystic carcinoma, and ductal carcinoma in situ in young women. Three studies on prostate cancer address prostatic small cell carcinoma and adenocarcinoma of young and elderly patients. Six studies on head and neck cancers include orbital and intraocular lymphoma, mucosal melanoma, pediatric nasopharyngeal carcinoma, olfactory neuroblastoma, and mucosa-associated lymphoid tissue lymphoma of the salivary glands. There were 4 central nervous system studies on patients with cerebellar glioblastoma multiforme, atypical and malignant meningioma, spinal epidural lymphoma and myxopapillary ependymoma. Outside of these disease sites, there is a wide variety of other studies on tumors ranging from uterine leiomyosarcoma to giant cell tumors of the bone. The studies done by the RCN represent a wide range of rare pathologies that were previously only studied in small series or case reports. With further growth of the RCN and collaboration between members our ability to analyze rare tumors will increase and result in better understanding of their behavior and ultimately help direct research that may improve patient outcomes.

Pediatric Cerebellar Hemorrhagic Glioblastoma Multiforme

We report the case of an 11 year old boy who presented with nausea, vomiting and ataxia. He was evaluated with computed tomography (CT) and magnetic resonance imaging (MRI). Imaging demonstrated minimal enhancement and hemorrhage of a cerebellar mass. Cerebellar glioblastoma multiforme (GBM) is extremely rare in the cerebellum at any age but especially in children. The atypical findings of minimal enhancement, cerebellar location and hemorrhagic presentation combine to make the prospective diagnosis of GBM a difficult one. This rare combination of findings has not been previously reported.

Cerebellar glioblastomas: pathophysiology, clinical presentation and management

Glioblastoma multiforme usually affects the cerebral hemispheres with the peak age of onset in the sixth or seventh decade, while cerebellar glioblastoma multiforme is a rare tumour especially in younger patients. Most result from de-differentiation from low grade astrocytoma (secondary glioblastoma) or can develop de novo (primary glioblastoma). Primary glioblastomas develop in older patients while secondary glioblastomas develop in younger patients and contain TP53 mutations as the earliest detectable change. We report a 28 year old patient with primary multi-focal cerebellar glioblastoma multiforme and review the pathophysiology, clinical presentation, diagnosis and treatment of cerebellar glioblastomas.

POSTERIOR FOSSA TUMOR IN A 12 YEAR‐OLD BOY

Cerebellar glioblastoma multiforme (GBM) has rarely been reported in children. We report a case of a 12-year-old child complaining of right upper limb tremor, loss of the normal capacity to modulate fine voluntary movements with right hand and headache, lasting for over a month. Radiological studies (CT and MRI) revealed a lesion of the right cerebellar hemisphere. The tumor was surgically excised and the histological examination revealed the presence of a GBM. The differential diagnosis of the lesions in the posterior fossa should include GBM. A gross total resection should be always attempted in order to achieve a better clinical outcome, although nearly all of these tumors recur.

Cerebellar glioblastoma mutiforme in an adult

Eventhough supratentorial glioblastoma multiforme (GBM) is the most frequent primary brain tumor in adults, its localization in the cerebellum is extremely rare1. It is very important for the neurosurgeon to make the differential diagnosis between the cerebellar GBM, and other diseases like metastasis, anaplasic astrocytomas, and cerebellar infarct because their treatment modalities, prognosis, and outcome are different2. Clinical manifestations of these diseases are very similar, so making an appropriate diagnosis is fundamental for the management of each of them3 […] [...]

Glioblastoma Multiforme of the Cerebellum: Report of Two Cases

Glioblastoma multiforme (GBM) usually arises in the supratentorial region, and rarely occurs in the cerebellum. Cerebellar GBM was first described in 1928 by Carmichael, and only 105 cases have since been reported in the literature. Only one case has ever been reported in Asia. Herein, we report two additional cases of cerebellar GBM occurring in the Asian area. Both patients had a history of dizziness, unsteady gait, and obvious cerebellar signs. Tumor excision along with postoperative posterior fossa irradiation was performed in each case. We discuss the clinical symptoms, radiological features, surgical findings, and radiotherapeutic strategies, and then review the literature.

Cerebellar Glioblastoma Multiforme in an Adult

Primary cerebellar glioblastoma multiforme (GBM) is a rare tumor in adults that accounts for just 1% of all cases of GBM. Due to their rarity, cerebellar GBMs are not yet completely understood about the pathogenesis and the prognosis. Here, we present a case of GBM in a 69-year-old man. Neurologic examination revealed the presence of cerebellar signs. Magnetic resonance imaging (MRI) showed a 4.5 x 3.6 cm-sized, ill-defined, heterogeneously enhancing mass in the left cerebellum and two patchy hyperintense lesions in the right cerebellum with minimal enhancement. After operation, glioblastoma was histologically confirmed. Postoperative radiotherapy with concomitant and adjuvant temozolomide chemotherapy was subsequently followed. Here, a case of unusual GBM in the cerebellum is reported with review of literature regarding the pathogenesis, the differential diagnosis and prognosis. There was no evidence of recurrence during postoperative one year. This patient showed a good prognosis in spite of the multiple lesions.

Tumor do sistema nervoso central secundário a radioterapia e quimioterapia.

A 22 year-old-man with acute lymphoblastic leukaemia had received prophylactic cranial irradiation and intrathecal chemotherapy. Eighteen years later a cerebellar glioblastoma multiforme was diagnosed. The authors speculate about the possibility that this tumor may have been radiation and/or chemotherapy induced. Improvement in neuroimaging techniques, in particular magnetic resonance imaging, has helped characterize Central Nervous System abnormalities, namely secondary brain tumours.

Outcome and prognostic factors in cerebellar glioblastoma multiforme in adults: A retrospective study from the Rare Cancer Network

The aim of this study was to assess the outcome in patients with cerebellar glioblastoma (GBM) treated in 15 institutions of the Rare Cancer Network. Data from a series of 45 adult patients with cerebellar GBM were collected in a retrospective multicenter study. Median age was 50.3 years. Brainstem invasion was observed in 9 (20%) patients. Radiotherapy (RT) was administered to 36 patients (with concomitant chemotherapy, 7 patients). Adjuvant chemotherapy after RT was administered in 8 patients. Median RT dose was 59.4 Gy. Median follow-up was 7.2 months (range, 3.4-39.0). The 1-year and 2-year actuarial overall survival rate was 37.8% and 14.7%, respectively, and was significantly influenced by salvage treatment (p = 0.048), tumor volume (p = 0.044), extent of neurosurgical resection (p = 0.019), brainstem invasion (p = 0.0013), additional treatment after surgery (p < 0.001), and completion of the initial treatment (p < 0.001) on univariate analysis. All patients experienced local progression: 8 and 22 had progression with and without a distant failure, respectively. The 1- and 2-year actuarial progression free survival was 25% and 10.7%, respectively, and was significantly influenced by brainstem invasion (p = 0.002), additional treatment after surgery (p = 0.0016), and completion of the initial treatment (p < 0.001). On multivariate analysis, survival was negatively influenced by the extent of surgery (p = 0.03) and brainstem invasion (p = 0.02). In this multicenter retrospective study, the observed pattern of failure was local in all cases, but approximately 1 patient of 4 presented with an extracerebellar component. Brainstem invasion was observed in a substantial number of patients and was an adverse prognostic factor.

Cerebellar glioblastoma multiforme in an adult

Cerebellar glioblastoma multiforme (GBM) is a rare tumor. This is the third case published in Brazilian literature and, the last one has been described more than 15 years ago. The aggressive behavior of GBM prompts for fast treatment, which can be hampered by the fact that the diagnosis of GBM requires a high degree of suspicion. We describe a case of GBM in a 46 years old man. In conjunction, we present a literature review including particular issues, clinical data, advances in imaging studies, pathological characteristics, treatment options and the behavior of such malignant tumor.