Primary central nervous system lymphoma (PCNSL) is an extranodal Non-Hodgkin's lymphoma that is confined to the brain, eyes, and/or leptomeninges without evidence of a systemic primary tumor. Although the tumor can affect all age groups, it is rare in childhood; thus, its incidence and prognosis in children have not been well defined and the best treatment strategy remains unclear. A nine-year old presented at our department with complaints of diplopia, dizziness, dysarthria, and right side hemiparesis. Magnetic resonance image suggested a diffuse brain stem glioma with infiltration into the right cerebellar peduncle. The patient was surgically treated by craniotomy and frameless stereotactic-guided biopsy, and unexpectedly, the histopathology of the mass was consistent with diffuse large B cell lymphoma, and immunohistochemical staining revealed positivity for CD20 and CD79a. Accordingly, we performed a staging work-up for systemic lymphoma, but no evidence of lymphoma elsewhere in the body was obtained. In addition, she had a negative serologic finding for human immunodeficient virus, which confirmed the histopathological diagnosis of PCNSL. She was treated by radiosurgery at 12 Gy and subsequent adjuvant combination chemotherapy based on high dose methotrexate. Unfortunately, 10 months after the tissue-based diagnosis, she succumbed due to an acute hydrocephalic crisis.
Biopsy ; Brain Stem* ; Brain* ; Central Nervous System* ; Child* ; Craniotomy ; Diagnosis ; Diplopia ; Dizziness ; Drug Therapy, Combination ; Dysarthria ; Glioma ; Humans ; Incidence ; Lymphoma* ; Lymphoma, B-Cell ; Lymphoma, Non-Hodgkin ; Methotrexate ; Paresis ; Prognosis ; Radiosurgery

OBJECTIVE: This study investigated whether pyrosequencing can be used to determine the methylation status of the MGMT promoter as a clinical biomarker using relatively old archival tissue samples of glioblastoma. We also examined other prognostic factors for survival of glioblastoma patients. METHODS: The available study set included formalin-fixed paraffin-embedded (FFPE) tissue from 104 patients at two institutes from 1997 to 2012, all of which were diagnosed histopathologically as glioblastoma. Clinicopathologic data were collected by review of medical records. For pyrosequencing analysis, the PyroMark Q96 CpG MGMT kit (Qiagen, Hilden, Germany) was used to detect the level of methylation at exon 1 positions 17-39 of the MGMT gene, which contains 5 CpGs. RESULTS: Methylation of the MGMT promoter was detected in 43 (41.3%) of 104 samples. The average percentage methylation was 14.0+/-16.8% overall and 39.0+/-14.7% for methylated cases. There was no significant pattern of linear increase or decrease according to the age of the FFPE block (p=0.687). In multivariate analysis, age, performance status, extent of surgery, method of adjuvant therapy, and methylation status estimated by pyrosequencing were independently associated with overall survival. Additionally, patients with a high level of methylation survived longer than those with low methylation (p=0.016). CONCLUSION: In this study, the status and extent of methylation of the MGMT promoter analyzed by pyrosequencing were associated with overall survival in glioblastoma patients. Pyrosequencing is a quantitative method that overcomes the problems of MSP and a simple technique for accurate analysis of DNA sequences.
Academies and Institutes ; Base Sequence ; Exons ; Glioblastoma* ; Humans ; Medical Records ; Methylation* ; Multivariate Analysis ; Prognosis

Chiari type I malformation(CM-I) is a congenital disorder recognized by caudal displacement of the cerebellar tonsils through the foramen magnum and into the cervical canal. Though bony anomalies associated with CM including platybasia, small posterior fossa and occipitalization, are relatively frequent, but the incidence rate associated with agenesis of posterior arch of atlas is very low. We report our experience of surgical treatment for patient who had CM-I with unilateral agenesis of posterior arch of atlas.
Arnold-Chiari Malformation ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Foramen Magnum ; Humans ; Incidence ; Palatine Tonsil ; Platybasia

OBJECTIVE: This study is designed to evaluate the clinical outcome, the safety and the effectiveness of the open sella methods(OSM) with intentionally staged transsphenoidal approach(TSA) for giant pituitary adenomas(GPA). METHODS: Eight patients with GPA were managed by the OSM with intentionally staged TSA. There were 5 nonfunctioning adenomas, 2 prolactin- secreting adenomas, and 1 growth hormone-secreting adenoma. Among them, 6 patients underwent two times of TSA, one patient underwent three times of TSA, and the other patient underwent two times of TSA followed by radiation therapy. The mean time interval between staged operations was 3.9 months except for one case. RESULTS: Seven out of the eight patients with GPA treated with the OSM with intentionally staged TSA showed that the tumors were completely removed on magnetic resonance imaging and that they were free from headache and visual problem suffered previously. Only one patient experienced severe complications including panhypopituitarism, cerebrospinal fluid rhinorrhea and permanent diabetes insipidus. CONCLUSION: With the surgical treatment for 8 cases of GPA, which extended to the suprasellar and parasellar area, we suggest that the OSM with intentionally staged TSA is a safe and effective method in management for GPA.
Adenoma ; Cerebrospinal Fluid Rhinorrhea ; Diabetes Insipidus ; Headache ; Humans ; Intention* ; Magnetic Resonance Imaging ; Pituitary Neoplasms*

OBJECTIVE: The aim of this study was to determine 30-day mortality and 6-month functional recovery rates in spontaneous intracerebral hemorrhage (S-ICH) patients undergoing hemodialysis treatment for end-stage renal disease (ESRD), and to compare the outcomes of these patients and S-ICH patients without ESRD. METHODS: The medical records of 1943 S-ICH patients from January 2000 to December 2011 were retrospectively analyzed with focus on demographic, radiological, and laboratory characteristics. RESULTS: A total of 1558 supratentorial S-ICH patients were included in the present study and 102 (6.5%) were ESRD patients. The 30-day mortality of the S-ICH patients with ESRD was 53.9%, and 29.4% achieved good functional recovery at 6 months post-S-ICH. Multivariate analysis showed that age, Glasgow Coma Scale (GCS) score, pupillary abnormality, ventricular extension of hemorrhage, hemorrhagic volume, hematoma enlargement, anemia, and treatment modality were independently associated with 30-day mortality in S-ICH patients with ESRD (p<0.05), and that GCS score, volume of hemorrhage, conservative treatment, and shorter hemodialysis duration was independently associated with good functional recovery at 6 months post-S-ICH in patients with ESRD (p<0.05). CONCLUSION: This retrospective study showed worse outcome after S-ICH in patients with ESRD than those without ESRD; 30-day mortality was four times higher and the functional recovery rate was significantly lower in S-ICH patients with ESRD than in S-ICH patients without ESRD.
Anemia ; Cerebral Hemorrhage* ; Glasgow Coma Scale ; Hematoma ; Hemorrhage ; Humans ; Kidney Failure, Chronic* ; Medical Records ; Multivariate Analysis ; Renal Dialysis ; Retrospective Studies

OBJECTIVE: The decision to adopt a conservative or surgical modality for a relatively small volume of spontaneous intracerebral hemorrhage (SICH) is difficult and often controversial, especially when consciousness is tolerable. The authors examined the results of stereotactic-guided evacuation of SICH for relatively small volumes with respect to functional outcome. METHODS: This prospective study was performed on 387 patients with SICH who underwent stereotactic-guided evacuation (n = 204, group A) or conservative treatment (n = 183, group B) during the past 8 years. The primary end-point was recovery of functional status, which was estimated using the Modified Barthel Index (MBI) and the modified Rankin Scale (mRS). RESULTS: All patients had a Glasgow coma scale (GCS) score of > or = 13 and unilateral hemiparesis of less than motor power grade 3. Group demographic characteristics and initial neurological statuses were similar. In all cases, the volume of SICH involved was < 30 cm3 and location was limited to basal ganglia and thalamus. At 6-month follow-ups, MBI was 90.9 in group A and 62.4 in group B (p < 0.05), and MRS was 1.2 in group A and 3.0 in group B (p < 0.05). Better motor function and stereotactic-guided evacuation had a significant effect on a functional recovery in regression analyses. CONCLUSION: Even in patients with a small volume of SICH, stereotactic-guided evacuation improved functional recovery in activities in daily life than conservative treatment did.
Basal Ganglia ; Cerebral Hemorrhage ; Consciousness ; Follow-Up Studies ; Glasgow Coma Scale ; Humans ; Neurosurgery ; Paresis ; Prospective Studies ; Recovery of Function ; Thalamus ; Treatment Outcome

Gliomas are the most frequently occurring primary brain tumors in adults. Although they exist in different malignant stages, including histologically benign forms and highly aggressive states, most gliomas are clinically challenging for neuro-oncologists because of their infiltrative growth patterns and inherent relapse tendency with increased malignancy. Once this disease reaches the glioblastoma multiforme stage, the prognosis of patients is dismal: median survival time is 15 months. Extensive genetic analyses of glial tumors have revealed a variety of deregulated genetic pathways involved in DNA repair, apoptosis, cell migration/adhesion, and cell cycle. Recently, it has become evident that epigenetic alterations may also be an important factor for glioma genesis. Of epigenetic marks, histone modification is a key mark that regulates gene expression and thus modulates a wide range of cellular processes. In this review, I discuss the neuro-oncological significance of altered histone modifications and modifiers in glioma patients while briefly overviewing the biological roles of histone modifications.
Acetylation ; Adult ; Apoptosis ; Brain Neoplasms ; Cell Cycle ; DNA Repair ; Epigenomics ; Gene Expression ; Glioblastoma ; Glioma* ; Histones* ; Humans ; Methylation ; Prognosis ; Recurrence

OBJECTIVE: Hypertensive intracerebral hemorrhage is a potentially life-threatening neurological deficit with the highest morbidity and mortality. In recent years, neuroendoscopy has been used to treat intracerebral hemorrhages (ICHs). However, the choice of neuroendoscopic surgery or craniotomy for patients with ICHs is controversial. The objective of this meta-analysis was to assess the efficacy of neuroendoscopic surgery compared to craniotomy in patients with supratentorial hypertensive ICH.MATERIALS AND METHODS: A systematic electronic search was performed using online electronic databases such as Pubmed, Embase, and Cochrane library updated on December 2017. The meta-analysis was performed by only including studies designed as randomized controlled trials.RESULTS: Three randomized controlled trials met our inclusion criteria. Pooled analysis of death showed that neuroendoscopic surgery decreased the rate of death compared to craniotomy (RR=0.58, 95% CI: 0.26–1.29; P=0.18). Pooled results of complications showed that neuroendoscopic surgery tended to have fewer complications than craniotomy had (RR=0.37, 95% CI: 0.28–0.49; P < 0.0001).CONCLUSION: Although the presenting analyses suggest that neuroendoscopic surgery should have fewer complications than craniotomy dose, it had no superior advantage in morbidity rate definitely. Therefore, it may be necessary for the neurosurgeons to select best optimal patients for individual treatment.
Cerebral Hemorrhage ; Craniotomy ; Humans ; Intracranial Hemorrhage, Hypertensive ; Mortality ; Neuroendoscopy ; Neurosurgeons

Meningiomas are the most common primary brain tumors in adults. The treatment of non-benign meningiomas remains a challenging task, and after the publication of the 2021 World Health Organization classification, the importance of molecular biological classification is emerging. In this article, we introduce the mechanisms of brain invasion in atypical meningioma and review the genetic factors involved along with epigenetic regulation. First, it is important to understand the three major steps for brain invasion of meningeal cells: 1) degradation of extracellular matrix by proteases, 2) promotion of tumor cell migration to resident cells by adhesion molecules, and 3) neovascularization and supporting cells by growth factors. Second, the genomic landscape of meningiomas should be analyzed by major categories, such as germline mutations in NF2 and somatic mutations in non-NF2 genes (TRAF7, KLF4, AKT1, SMO, and POLR2A). Finally, epigenetic alterations in meningiomas are being studied, with a focus on DNA methylation, histone modification, and RNA interference. Increasing knowledge of the molecular landscape of meningiomas has allowed the identification of prognostic and predictive markers that can guide therapeutic decision-making processes and the timing of follow-up.

BACKGROUND: The aim of this study was to determine whether pyrosequencing (PSQ) might be useful to achieve O6-methyl guanine methyltransferase (MGMT) promoter methylation using 1- to 13-year-old archival tissues as a clinical biomarker in routine practice. METHODS: The study included 141 formalin-fixed paraffin-embedded (FFPE) glial tumors from the archives of the Pathology Department from 1997-2010. RESULTS: The average percentage of methylation (MP) of the 141 cases was 14.0+/-16.8%, and methylated cases were 32.3+/-14.9%. The average MP of each year did not show a linear increasing or decreasing pattern according to the age of the FFPE block (p=0.771). The average MP of methylated glioblastomas was 35.8+/-14.7%, 31.8+/-15.5% for anaplastic astrocytomas, and 22.4+/-15.1% for astrocytoma. A tendency was observed toward an increasing pattern of average MP with World Health Organization (WHO) grade (p=0.063) in astrocytic tumors. A correlation was observed between average MP and WHO grade (p=0.038) and a bimodal distribution was observed between the methylated and unmethylated cases, using a 9% cut-off value (p<0.001). CONCLUSIONS: The results showed that a quantitative approach for MGMT promoter methylation yielded a 100% success rate for FFPE tissues from archives. PSQ can be used in a retrospective trial, but the cut-off value and calculation method should be further validated.
Adolescent ; Astrocytoma ; Brain ; Glioblastoma ; Glioma ; Guanine ; Humans ; Methylation ; Retrospective Studies ; World Health Organization