Child ; Child Welfare ; Humans ; Medulloblastoma ; drug therapy ; epidemiology ; surgery ; Mortality ; Radiotherapy ; Singapore ; epidemiology ; Treatment Outcome

With improvement of computerized tomography(CT), stereotaxic surgery has been applied for neurosurgery ; aspiration of deep-seated brain lesions, radiotherapy using a small radiogenic sources, and evacuation of hematoma. And so, a prototype Brown-Roberts-Wells(BRW) CT stereotaxic system has been avaible to us. We report its utilization in evaluation and management of less accessible brain lesions. Surgical exploration was undertaken in 25 patients with various less accessible brain lesions using BRW system : 9 superficial lesions including the motor cortex and multiple lesions, 5 deep-seated lesions, 3 parasellar lesions, 8 brain stem lesions. Histologically, there are 5 gliomas, 4 metastatic cancers, 8 hematomas, 3 brain abscesses, 1 medulloblastoma, 1 malignant melanoma, 1 cryptic AVM, and 2 undiagnosed cases. There are two complications : 1 intratumoral bleeing, 1 rebleeding. The diagnostic rate is 92%. There is no mortality in this series. This BRW CT-guidance stereotaxic approach for less accessible lesions have following advantages ; 1) accurate, simple and safe ; 2) performed under local anesthesia ; 3) less traumatic and low cost ; 4) easily diagnosed ; 5) relatively no limitation in lesion sites.
Anesthesia, Local ; Brain Abscess ; Brain Stem ; Brain* ; Glioma ; Hematoma ; Humans ; Medulloblastoma ; Melanoma ; Mortality ; Motor Cortex ; Neurosurgery ; Radiotherapy

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.
Arm ; Brain Neoplasms ; Child ; Classification ; Computational Biology ; Drug Therapy ; Hedgehogs ; Humans ; Medulloblastoma ; Mortality ; Neurosurgery ; Pediatrics ; Population Characteristics ; Recurrence

We analyzed the 43 cases of the posterior fossa tumors at the Busan Paik Hospital, Inje Medical College from January 1980 to December 1986. The posterior fossa tumors included 12 cases of cerebellar astrocytoma, 10 cases of acoustic neurinoma, 5 cases of medulloblastoma, 5 cases of brain stem glioma, 2 cases of meningioma, 2 cases of hemangioblastoma, 2 cases of tuberculoma, 1 case of glioblastoma multiforme, 1 case of oliodendroglioma, 1 case of rhabdomyosarcoma, 1 case of glomus jugulare tumor and 1 case of arteriovenous malformation. The posterior fossa tumors were occupied on the cerebellar hemisphere in 18 cases, the cerebellar vermis in 8 cases, the cerebellar pontine angle in 12 cases and the brain stem in 5 cases. The 25 cases out of the 43 cases of the posterior fossa tumors were accompanied with hydrocephalus. The 10 cases received the radiation therapy and/or the chemotherapy after operation. After treatment of the posterior fossa tumors, 52.6% favorable outcome, 34.2% unfavorable outcome and 13.2% mortality were estimated.
Arteriovenous Malformations ; Astrocytoma ; Brain Stem ; Busan ; Drug Therapy ; Glioblastoma ; Glioma ; Glomus Jugulare Tumor ; Hemangioblastoma ; Hydrocephalus ; Infratentorial Neoplasms* ; Medulloblastoma ; Meningioma ; Mortality ; Neuroma, Acoustic ; Rhabdomyosarcoma ; Tuberculoma

The authors reviewed one hundred and twenty cases of cerebellopontine angle(CPA) tumors that were operated upon at the department of neurosurgery of Seoul National University Hospital between 1977 and 1987. The pathologic diagnosis of the tumors were acoustic neurinoma(66%), meningioma(13%), epidermoid(5%) and trigeminal neurinoma(5%). The age incidence was most frequent in the 5th and the 6th decades and 6.7% of cases occurred at the pediatric ages. Acoustic neurinomas were frequent in the 5th decade and on admission more than half of patients were poor clinical grades with large tumors more than 3cm in diameter(78.5%). There was good correlation between the clinical grade and tumor size on admission. All of the acoustic tumors were operated through the suboccipital transmeatal approach and total removal was possible in 73% with 5% of motality rate. Facial nerve was preserved in 62% of total removal-cases and the size of tumor was the important factor for the total removal of tumor with preservation of facial nerve. Cerebellopontine angle meningioma comprised 13% of all CPA tumors and incidence of male to female ratio was 2:13. Operations were performed either through retromastoid suboccipital approach or combined supra-infratentorial approach. Total temoval was possible in 67% without mortality. Six trigeminal neurinomas were located:one in the middle fossa, one in the posterior fossa and the other four cases appearing as dumbbell shape. Total removal was possible in two cases and subtotal removal in four cases and the outcome was rather good in all cases. Pediatric CPA tumors were two each of medulloblastomas and ependymomas and one each of astrocytoma, primitive neuroectodermal tumor(PNET) and trigeminal neurinoma, Masson's hemagioendothelioma. Total removal was possible in four cases and the outcome was good in all cases.
Acoustics ; Astrocytoma ; Cerebellopontine Angle* ; Diagnosis ; Ependymoma ; Facial Nerve ; Female ; Humans ; Incidence ; Male ; Medulloblastoma ; Meningioma ; Mortality ; Neural Plate ; Neurilemmoma ; Neuroma, Acoustic* ; Neurosurgery ; Seoul

Medulloblastoma is considered one of the most threatening malignant brain tumors with an extremely high mortality rate in children. In the medulloblastoma, there are several genes and mutations found to work in an unregulated manner that works together to push the cells into a cancerous state. With the discovery of non-coding RNAs such as microRNAs (miRNAs), it has been shown that a different layer of gene regulations may be disrupted which would cause cancer. This fact led scientists to put their focus on the role of miRNAs in cancer. A mature miRNA contains a seed sequence which gives the miRNA to identify and attach to the interest mRNA; this attachment may lead degradation of mRNA or suppress of translation of the mRNA. The expression of miRNAs in medulloblastoma shows that some of these non-coding RNAs are overexpressed (OncomiRs) which help cells to proliferate and keep their stemness features. On the other hand, there are other forms of these miRNAs which normally inhibit cell proliferation and promote cell differentiation (tumor suppressor). These are down-regulated during cancer progression. In this systematic review, we attempted to gather several important studies on miRNAs’ role in medulloblastoma tumors and the importance of these non-coding RNAs in the future study of cancer.
Brain Neoplasms ; Cell Differentiation ; Cell Proliferation ; Child ; Genes, Tumor Suppressor ; Hand ; Humans ; Medulloblastoma ; MicroRNAs ; Mortality ; Oncogenes ; RNA, Messenger ; RNA, Untranslated ; Social Control, Formal

Medulloblastoma accounts for 20 to 25+ACU- of all intracranial neoplasms in children. The significance of the presence of isochromosome 17q (i(17q)), proliferative potential, apoptotic activity, and expression of c-erbB-2, bd-2, and p53 proteins in predicting long-term survival of patients with medulloblastomas was investigated. Twenty children were divided into two groups (favorable and poor outcome groups). Ten children with favorable outcome (FO) were disease-free during the follow-up period (median: 61.5 months). The other ten children with poor outcome (PO) died of disease progression, having a median survival of 18 months. Fluorescent in situ hybridization (FISH) for i(17q), terminal deoxynucleotidyl transferase-mediated dUTP-biotin nick end labeling (TUNEL), and immunohistochemistry for Ki-67, c-erbB-2, bcl-2, and p53 proteins was performed in these patients. Nine out of 17 children showed i(17q). There was no difference in the rate of positive i(17q) between the FO and PO groups. The presence of i(17q) was not significantly related to biological factors that we investigated. Unlike the prominent presence of the proliferative potential and p53 expression in children with PO, apoptotic activity and expression of c-erbB-2 and bcl-2 had no correlation with the outcome.
Adolescence ; Apoptosis ; Brain Neoplasms/pathology ; Brain Neoplasms/mortality ; Brain Neoplasms/genetics+ACo- ; Cell Division ; Child ; Child, Preschool ; Chromosomes, Human, Pair 17/ultrastructure+ACo- ; Chromosomes, Human, Pair 17/genetics ; Comparative Study ; Disease-Free Survival ; Female ; Follow-Up Studies ; Genes, bcl-2+ACo- ; Genes, erbB-2+ACo- ; Genes, p53+ACo- ; Human ; In Situ Hybridization, Fluorescence ; In Situ Nick-End Labeling ; Infant ; Ki-67 Antigen/analysis ; Male ; Medulloblastoma/pathology ; Medulloblastoma/mortality ; Medulloblastoma/genetics+ACo- ; Neoplasm Proteins/analysis ; Prognosis ; Retrospective Studies ; Survival Analysis ; Treatment Outcome

Medulloblastoma accounts for 20 to 25+ACU- of all intracranial neoplasms in children. The significance of the presence of isochromosome 17q (i(17q)), proliferative potential, apoptotic activity, and expression of c-erbB-2, bd-2, and p53 proteins in predicting long-term survival of patients with medulloblastomas was investigated. Twenty children were divided into two groups (favorable and poor outcome groups). Ten children with favorable outcome (FO) were disease-free during the follow-up period (median: 61.5 months). The other ten children with poor outcome (PO) died of disease progression, having a median survival of 18 months. Fluorescent in situ hybridization (FISH) for i(17q), terminal deoxynucleotidyl transferase-mediated dUTP-biotin nick end labeling (TUNEL), and immunohistochemistry for Ki-67, c-erbB-2, bcl-2, and p53 proteins was performed in these patients. Nine out of 17 children showed i(17q). There was no difference in the rate of positive i(17q) between the FO and PO groups. The presence of i(17q) was not significantly related to biological factors that we investigated. Unlike the prominent presence of the proliferative potential and p53 expression in children with PO, apoptotic activity and expression of c-erbB-2 and bcl-2 had no correlation with the outcome.
Adolescence ; Apoptosis ; Brain Neoplasms/pathology ; Brain Neoplasms/mortality ; Brain Neoplasms/genetics+ACo- ; Cell Division ; Child ; Child, Preschool ; Chromosomes, Human, Pair 17/ultrastructure+ACo- ; Chromosomes, Human, Pair 17/genetics ; Comparative Study ; Disease-Free Survival ; Female ; Follow-Up Studies ; Genes, bcl-2+ACo- ; Genes, erbB-2+ACo- ; Genes, p53+ACo- ; Human ; In Situ Hybridization, Fluorescence ; In Situ Nick-End Labeling ; Infant ; Ki-67 Antigen/analysis ; Male ; Medulloblastoma/pathology ; Medulloblastoma/mortality ; Medulloblastoma/genetics+ACo- ; Neoplasm Proteins/analysis ; Prognosis ; Retrospective Studies ; Survival Analysis ; Treatment Outcome

Medulloblastoma, once a tumor with a dismal prognosis, is one of the most common primary brain tumors of childhood. As the methods of treatment have been continuously refined, the outcome has improved remarkably during the last few decades. The outcome of 78 medulloblastoma patients, which were managed from 1972 to 1992 at the Department of Neurosurgery of Seoul National University Hospital, were analyzed to calculate the 3-year and 5-year survival rates (3yS and 5yS). Of those, 52 cases which were treated after July 1982 were studied 1) to calculate the 3yS and 5yS, 2) to figure out the prognostic factors of survival, and 3) to investigate the role of adjuvant chemotherapy ('8-drugs-in-a-day' protocol: CCNU, cisplatin, vincristine, hydroxyurea, procarbazine, cytosine arabinoside, methylprednisolone and cyclophosphamide). The 3yS and 5yS of the 78 patients were 57.4% and 47.3%, respectively. Of the 52 patients treated after July 1982, the 3yS and 5yS were 67.8% and 64.1%, respectively. The latest recurrence was at 56 months after surgery. All the recurrences were within the risk period of Collins' rule. Of the prognostic factors studied by univariate analysis (age, sex, Chang's classification T- and M-stages, extent of surgical removal, and chemotherapy), Chang's classification M-stage and sex were the statistically significant factors (p = 0.028 and 0.024 respectively). On multivariate analysis, only the M-stage was statistically significant (p = 0.004). Adjuvant chemotherapy had different influences in different patient groups. Only in the 'poor risk' group, did adjuvant chemotherapy have a strong tendency to better outcome (p = 0.069). Further data collection and analysis will lead to better treatment modalities and better outcome for this most common primary malignant brain tumor in childhood.
Adolescent ; Adult ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use ; Cerebellar Neoplasms/*drug therapy/mortality/radiotherapy ; Chemotherapy, Adjuvant ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Medulloblastoma/*drug therapy/mortality/radiotherapy ; Middle Aged ; Neoplasm Recurrence, Local ; Prognosis ; Survival Rate ; Treatment Outcome

OBJECTIVETo study the potential relationship between the expressions of c-jun and c-fos oncogenes and the prognosis of medulloblastoma.

METHODSThe specimens from 70 cases of medulloblastoma of the posterior fossa and 10 cases of normal cerebellar tissues were collected to determine c-jun and c-fos expressions by immunohistochemical staining in formalin fixed paraffin-embedded sections.

RESULTS(1) It showed that c-fos and c-jun protein expression was negative in 10 normal cerebellar tissue, while positive c-fos, c-jun immunoreactivity was found in 70 medulloblastoma specimens. The positive rate of c-jun and c-fos was 80% and 77%, respectively. There was high expression of c-jun and c-fos protein in medulloblastoma tissues. (2) There were positive correlations and strong co-operativity between c-jun and c-fos expression (r = 0.493, P < 0.01). (3) Correlative analysis indicated that expression of c-jun, c-fos were significantly correlated with survival time (c-jun: r = -0.447, P < 0.01; c-fos: r = -0.590, P < 0.01). The higher the expression level of c-jun and c-fos protein was, the worse the prognosis was in medulloblastoma patients.

CONCLUSIONSHigh expression of c-jun and c-fos protein could be noted in medulloblastoma tissues. The two transcription factors show positive correlation and strong co-existence between c-jun and c-fos expressions. The expression levels of c-jun as well as c-fos are negatively correlated with the mortality rate and life expectancy of patients with medulloblastoma. In addition, the co-expression of c-jun and c-fos could serve as an indicator for judging the prognosis of medulloblastoma.

Adolescent ; Adult ; Child ; Child, Preschool ; Female ; Humans ; Immunohistochemistry ; Infant ; Male ; Medulloblastoma ; metabolism ; mortality ; pathology ; Proto-Oncogene Proteins c-fos ; analysis ; Proto-Oncogene Proteins c-jun ; analysis ; Survival Analysis ; Survival Rate