• Journal of Korean Neurosurgical Society
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• v.55 no.6
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• pp.362-364
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• 2014

Extra-axial medulloblastoma is a rare phenomenon. We report a case in a 5-year-old boy who presented with nausea, vomiting, and gait disturbance. He was treated with total removal of the tumor. This is the first case of an extra-axially located medulloblastoma occurring in the cerebellar hemisphere posteriolateral to the cerebellopontine angle in Korea. Although the extra-axial occurrence of medulloblastoma is rare, it should be considered in the differential diagnosis of extra-axial lesions of the posterior fossa in children.

• Molecules and Cells
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• v.37 no.5
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• pp.357-364
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• 2014

Medulloblastoma, the most common malignant brain tumor in children, is a disease whose mechanisms are now beginning to be uncovered by high-throughput studies of somatic mutations, mRNA expression patterns, and epigenetic profiles of patient tumors. One emerging theme from studies that sequenced the tumor genomes of large cohorts of medulloblastoma patients is frequent mutation of RNA binding proteins. Proteins which bind multiple RNA targets can act as master regulators of gene expression at the post-transcriptional level to co-ordinate cellular processes and alter the phenotype of the cell. Identification of the target genes of RNA binding proteins may highlight essential pathways of medulloblastomagenesis that cannot be detected by study of transcriptomics alone. Furthermore, a subset of RNA binding proteins are attractive drug targets. For example, compounds that are under development as anti-viral targets due to their ability to inhibit RNA helicases could also be tested in novel approaches to medulloblastoma therapy by targeting key RNA binding proteins. In this review, we discuss a number of RNA binding proteins, including Musashi1 (MSI1), DEAD (Asp-Glu-Ala-Asp) box helicase 3 X-linked (DDX3X), DDX31, and cell division cycle and apoptosis regulator 1 (CCAR1), which play potentially critical roles in the growth and/or maintenance of medulloblastoma.

• Journal of Korean Neurosurgical Society
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• v.61 no.3
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• pp.292-301
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• 2018

Medulloblastoma is the most common malignant brain tumor of childhood and remains a major cause of cancer related mortality in children. Significant scientific advancements have transformed the understanding of medulloblastoma, leading to the recognition of four distinct clinical and molecular subgroups, namely wingless (WNT), sonic hedgehog, group 3, and group 4. Subgroup classification combined with the recognition of subgroup specific molecular alterations has also led to major changes in risk stratification of medulloblastoma patients and these changes have begun to alter clinical trial design, in which the newly recognized subgroups are being incorporated as individualized treatment arms. Despite these recent advancements, identification of effective targeted therapies remains a challenge for several reasons. First, significant molecular heterogeneity exists within the four subgroups, meaning this classification system alone may not be sufficient to predict response to a particular therapy. Second, the majority of novel agents are currently tested at the time of recurrence, after which significant selective pressures have been exerted by radiation and chemotherapy. Recent studies demonstrate selection of tumor sub-clones that exhibit genetic divergence from the primary tumor, exist within metastatic and recurrent tumor populations. Therefore, tumor resampling at the time of recurrence may become necessary to accurately select patients for personalized therapy.

• Journal of Korean Neurosurgical Society
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• v.58 no.6
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• pp.557-559
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• 2015

Medulloblastoma is a common pediatric tumor typically diagnosed before the age of fifteen. Initial therapy includes surgical resection and radiation of the entire neuro-axis. Recurrence is common and typically occurs within 2 years of initial diagnosis. Those fitting Collin's Law is considered tumor-free. We report a case of single supratentorial recurrence 13 years after initial diagnosis. Here we present a 22 year old male presenting 13 years after initial diagnosis with isolated septum pellucidum recurrence. He underwent complete resection of the tumor. Medulloblastoma is a common in the pediatric population. Late recurrence to the ventricular system is uncommon. Long term follow-up is recommended in these patients.

• Journal of Yeungnam Medical Science
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• v.24 no.2
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• pp.311-314
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• 2007

The extraaxial presentation of a medulloblastoma is rare. This article describes the case of a 12-year-old boy who presented with severe headache, nausea, and vomiting. The tumor developed in the left tentorium; it was misdiagnosed as a meningioma based on the radiology examination. We review the literature and discuss the atypical presentation of medulloblastoma.

• Journal of Korean Neurosurgical Society
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• v.50 no.1
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• pp.51-53
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• 2011

We present a rare case of medulloblastoma which presented with unilateral sudden sensorineural hearing loss as an initial symptom. A 19-year-old man was admitted to our hospital with a chief complaint of dizziness and facial numbness on the right side. His illness had begun two years previously with sudden hearing loss on the right side, for which he had been treated as an idiopathic sudden hearing loss. Magnetic resonance imaging demonstrated abnormal signals located mainly in the right middle cerebellar peduncle. We performed partial resection of the tumor by suboccipital craniotomy. The histopathological diagnosis was medulloblastoma. Intrinsic brain tumor is an extremely rare cause of sudden sensorineural hearing loss and is therefore easily overlooked as was in the present case. The present case highlights not only the need to evaluate patients with sudden sensorineural hearing loss by magnetic resonance imaging but also the importance of paying attention to intrinsic lesions involving the brainstem. Although this condition like the presented case might be rare, intrinsic brain tumor should be considered as a potential cause of sudden sensorineural hearing loss, as it may be easily missed leading to a delay in appropriate treatment.

• 한국데이터정보과학회:학술대회논문집
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• 2005.10a
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• pp.153-159
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• 2005

Microarray gene expression technology has applications that could refine diagnosis and therapeutic monitoring as well as improve disease prevention through risk assessment and early detection. Especially, microarray expression data can provide important information regarding specific genes related with metastasis through an appropriate analysis. Various methods for clustering analysis microarray data have been introduced so far. We used twostep clustering fot ascertain metastasis related gene through t-test. Through t-test between two groups for two publicly available medulloblastoma microarray data sets, we intended to find significant gene for metastasis. The paper describes the process in detail showing how the process is applied to clustering analysis and t-test for microarray datasets and how the metastasis-associated genes are explorated.

• Journal of Korean Neurosurgical Society
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• v.41 no.6
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• pp.403-407
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• 2007

The authors encountered a case of simultaneous radiation-induced multiple meningiomas and ventriculoperitoneal [VP] shunt-related pneumocephalus. A 35-year-old man, who had undergone surgery for medulloblastoma 21 years previously and subsequently received high dose craniospinal irradiation with adjuvant chemotherapy and later underwent a VP shunt because of hydrocephalus, presented with a severe headache and weakness of both lower extremities. Computed tomography showed an air pocket lesion in the left temporal lobe and a large amount of pneumocephalus with a bony defect of the left tegmen tympani. In addition, a 3 cm sized well enhancing mass was noted in the in the right middle cranial fossa and additional small enhancing nodule in the left frontal pole. He was treated by left temporal craniotomy and repair of the bony and dural defects of the left tegmentum tympanum through extradural and intradural approaches, respectively. Afterwards, he underwent right temporal craniotomy and gross total removal of a rapidly growing right middle fossa mass and a left frontal mass. The histological examination was consistent with atypical meningioma, WHO grade II. In conclusion, physicians have to consider the serious long term complications of high dose radiation therapy and VP shunt placement and need to perform the neuroradiologic follow-up after such treatments for several decades.

• Journal of Korean Neurosurgical Society
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• v.61 no.3
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• pp.402-406
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• 2018

In contrast to many of the malignant tumors that occur in the central nervous system in adults, the management, responses to therapy, and future perspectives of children with malignant lesions of the brain hold considerable promise. Within the past 5 years, remarkable progress has been made with our understanding of the basic biology of the molecular genetics of several pediatric malignant brain tumors including medulloblastoma, ependymoma, atypical teratoid rhabdoid tumour, and high grade glioma/diffuse intrinsic pontine glioma. The recent literature in pediatric neuro-oncology was reviewed, and a summary of the major findings are presented. Meaningful sub-classifications of these tumors have arisen, placing children into discrete categories of disease with requirements for targeted therapy. While the mainstay of therapy these past 30 years has been a combination of central nervous system irradiation and conventional chemotherapy, now with the advent of high resolution genetic mapping, targeted therapies have emerged, and less emphasis is being placed on craniospinal irradiation. In this article, the present and future perspective of pediatric brain malignancy are reviewed in detail. The progress that has been made offers significant hope for the future for patients with these tumours.

• Asian Pacific Journal of Cancer Prevention
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• v.15 no.16
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• pp.6781-6785
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• 2014

Purpose: This study used receiver operating characteristic curves to analyze Surveillance, Epidemiology and End Results (SEER) medulloblastoma (MB) and primitive neuroectodermal tumor (PNET) outcome data. The aim of this study was to identify and optimize predictive outcome models. Materials and Methods: Patients diagnosed from 1973 to 2009 were selected for analysis of socio-economic, staging and treatment factors available in the SEER database for MB and PNET. For the risk modeling, each factor was fitted by a generalized linear model to predict the outcome (brain cancer specific death, yes/no). The area under the receiver operating characteristic curve (ROC) was computed. Similar strata were combined to construct the most parsimonious models. A Monte Carlo algorithm was used to estimate the modeling errors. Results: There were 3,702 patients included in this study. The mean follow up time (S.D.) was 73.7 (86.2) months. Some 40% of the patients were female and the mean (S.D.) age was 16.5 (16.6) years. There were more adult MB/PNET patients listed from SEER data than pediatric and young adult patients. Only 12% of patients were staged. The SEER staging has the highest ROC (S.D.) area of 0.55 (0.05) among the factors tested. We simplified the 3-layered risk levels (local, regional, distant) to a simpler non-metastatic (I and II) versus metastatic (III) model. The ROC area (S.D.) of the 2-tiered model was 0.57 (0.04). Conclusions: ROC analysis optimized the most predictive SEER staging model. The high under staging rate may have prevented patients from selecting definitive radiotherapy after surgery.