Although chordomas are midline tumors, primarily intrasellar chordomas are extremely rare. In this report, the authors describe the case of a 68-year-old female with partial abducens nerve palsy in the right eye due to the intrasellar cystic tumor. After endonasal trans-sphenoidal surgery, intraoperative and histopathological findings confirmed the co-occurrence of an entirely intrasellar chordoma and pituitary adenoma. To our knowledge, the present case is the third reported case of an intrasellar chordoma with a pituitary adenoma.
Abducens Nerve Diseases ; Aged ; Chordoma* ; Female ; Humans ; Pathology ; Pituitary Neoplasms* ; Sella Turcica

Anaplastic large T-cell lymphoma (ALCL) encompasses different clinical entities that can be aggressive or localized. Scalp anaplastic lymphoma kinase (ALK)-negative ALCL is considered a localized lymphoma, and usually extends to the regional lymph nodes; intracranial invasion is rare. A 74-year-old woman was diagnosed with scalp ALK-negative ALCL, but did not exhibit invasion of the lymph nodes. Computed tomography and magnetic resonance imaging revealed intracranial masses with bony erosions. We treated the patient using CHOP chemotherapy and achieved short-term regression of the scalp and intracranial lesions. However, the patients ultimately died of pneumonia during the pancytopenic period. Therefore, caution must be exercised when treating scalp ALK-negative ALCL with intracranial invasion.
Aged ; Central Nervous System* ; Drug Therapy ; Female ; Humans ; Lymph Nodes ; Lymphoma ; Lymphoma, T-Cell* ; Magnetic Resonance Imaging ; Meningioma ; Neurofibromatoses ; Phosphotransferases ; Pneumonia ; Scalp ; T-Lymphocytes*

This report presents a case of fourth ventricle neurenteric cyst (NE cyst) mimicking hemangioblastoma, which developed in a 50-year-old woman. A tiny enhancing mural portion of the fourth ventricle in MRI suggested that the cyst was hemangioblastoma, but pathological evidence showed that the cyst was in fact NE cyst in the fourth ventricle. In order to make proper decision on to what extent of surgical resection should be done, considering every possibility in differential diagnosis might be helpful. This case reports an unusual pathology in 4th ventricle, considering the patient's age, and demonstrates that a rarer disease may share radiological features of a common disease.
Diagnosis, Differential ; Female ; Fourth Ventricle* ; Hemangioblastoma* ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Neural Tube Defects* ; Pathology

We report a rare case of subependymal giant cell astrocytoma (SEGA) associated with tumoral bleeding in a pediatric patient without tuberous sclerosis complex (TSC). A 10-year-old girl presented with a 2-week history of an increasingly aggravating headache. Brain magnetic resonance imaging revealed an approximately 3.6-cm, well-defined, heterogeneously enhancing mass with multistage hemorrhages on the right-sided foramen of Monro. The tumor was completely resected using a transcallosal approach. Intraoperatively, the mass presented as a gray-colored firm tumor associated with acute and subacute hemorrhages. The origin of the mass was identified as the ventricular septum adjacent to the foramen of Monro. A pathological analysis revealed pleomorphic multinucleated eosinophilic tumor cells with abundant cytoplasm. These cells showed positive staining for the glial fibrillary acidic protein and S100 protein. A diagnosis of SEGA was established. The patient recovered without any neurological symptoms. There was no evidence of TSC. The radiological follow-up showed no recurrence for 2 years. This was a case of SEGA with intratumoral hemorrhage, for which a favorable outcome was achieved, without any neurological deficit after tumoral resection.
Astrocytoma* ; Brain ; Cerebral Ventricles ; Child ; Cytoplasm ; Diagnosis ; Eosinophils ; Female ; Follow-Up Studies ; Glial Fibrillary Acidic Protein ; Headache ; Hemorrhage* ; Humans ; Magnetic Resonance Imaging ; Recurrence ; Tuberous Sclerosis ; Ventricular Septum

Toxoplasmic encephalitis (TE) is an opportunistic infection found in immunocompromised patients and TE related cerebral mass lesion is often reported in acquired immunodeficiency acquired immunodeficiency syndrome (AIDS) patients. However, incidence of TE related AIDS in Korea is still rare and is unfamiliar to neurosurgeons. Differential diagnosis is needed to rule out other brain lesions. A 39-year-old man visited the emergency room with rapid progressive left hemiparesis. Magnetic resonance imaging showed a ring-enhanced mass lesion in his right frontal lobe. Human immunodeficiency virus and Toxoplasma gondii immunoglobulin G were tested positive by a serologic test. We report here a rare case of patient with TE related AIDS.
Acquired Immunodeficiency Syndrome* ; Adult ; Brain ; Diagnosis, Differential ; Emergency Service, Hospital ; Encephalitis* ; Frontal Lobe ; HIV ; Humans ; Immunocompromised Host ; Immunoglobulin G ; Incidence ; Korea ; Magnetic Resonance Imaging ; Neurosurgeons ; Opportunistic Infections ; Paresis ; Serologic Tests ; Toxoplasma ; Toxoplasmosis

We report a case of primary central nervous system vasculitis (PCNSV) mimicking a cortical brain tumor. A 25-year-old woman presented with a 2-week history of headache and transient right hemiparesis. Brain magnetic resonance imaging (MRI) revealed a cortical-involving lesion on the left frontal lobe. The 6-cm sized lesion showed low signal intensity on T1-weighted images and high signal intensity on T2-weighted images. The lesion had continual linear enhancement on the subcortical white matter and leptomeninges. There was no evidence of hemorrhage on susceptibility-weighted images and no diffusion restriction on diffusion-weighted images. The regional cerebral blood volume was decreased on the MR perfusion images, and spectroscopy showed increased lactate and lipid peaks. The symptoms were aggravated by fever and seizures. Biopsy was performed to rule out tumorous or inflammatory lesions. Pathologically, lymphocytes were infiltrated on the vessels, and the arachnoid membrane was thickened with inflammatory cells. The patient did not have any underlying diseases, including immune disorders. After high-dose steroid administration, her symptoms improved. Two months later, brain MRI showed a reduction in the infiltration of the T2 hyperintensity lesion with subtle subcortical enhancement. We present a case of PCNSV involving the left frontal lobe, showing vasogenic edema, mass effect, and subcortical linear contrast enhancement without hemorrhage or infarction.
Adult ; Arachnoid ; Biopsy ; Blood Volume ; Brain Neoplasms* ; Brain* ; Central Nervous System ; Diffusion ; Edema ; Female ; Fever ; Frontal Lobe ; Headache ; Hemorrhage ; Humans ; Immune System Diseases ; Infarction ; Lactic Acid ; Lymphocytes ; Magnetic Resonance Imaging ; Membranes ; Paresis ; Perfusion ; Seizures ; Spectrum Analysis ; Steroids ; Vasculitis ; Vasculitis, Central Nervous System* ; White Matter

Over the course of five years, a total of ten cases were collected of glioma patients in whom a distant lesion at the fourth ventricle was noted. A ‘distant lesion’ was defined as a lesion with a normal appearing tissue bridge at imaging between the primary and secondary locations. Previous imaging of these patients was reviewed along with clinical history, course of therapy, and available histology. A review of the literature was performed with respect to present knowledge on patterns of glioma proliferation and dissemination. This case series is the first to describe the fourth ventricle as a location that may be prone to secondary lesions in glioma patients. Further investigation on this subject may yield deeper insights into the mechanisms by which glial tumors spread within the brain, with the hope of developing or improving therapeutic targets.
Brain ; Cerebrospinal Fluid ; Fourth Ventricle ; Glioblastoma ; Glioma* ; Hope ; Humans ; Magnetic Resonance Imaging ; Neoplasm Metastasis

BACKGROUND: This report aims to provide accurate nationwide epidemiologic data on primary brain and central nervous system (CNS) tumors in the Republic of Korea. We updated the data by analyzing primary brain and CNS tumors diagnosed in 2013 using the data from the national cancer incidence database. METHODS: Data on primary brain and CNS tumors diagnosed in 2013 were collected from the Korean Central Cancer Registry. Crude and age-standardized rates were calculated in terms of gender, age, and histological type. RESULTS: A total of 11,827 patients were diagnosed with primary brain and CNS tumors in 2013. Brain and CNS tumors occurred in females more often than in males (female:male, 1.70:1). The most common tumor was meningioma (37.3%). Pituitary tumors (18.0%), gliomas (12.7%), and nerve sheath tumors (12.3%) followed in incidence. Glioblastomas accounted for 41.8% of all gliomas. In children (<19 years), sellar region tumors (pituitary and craniopharyngioma), embryonal/primitive/medulloblastoma, and germ cell tumors were the most common tumors. CONCLUSION: This study should provide valuable information regarding the primary brain tumor epidemiology in Republic of Korea.
Brain ; Brain Neoplasms* ; Central Nervous System ; Child ; Epidemiology* ; Female ; Glioblastoma ; Glioma ; Humans ; Incidence ; Korea ; Male ; Meningioma ; Neoplasms, Germ Cell and Embryonal ; Nerve Sheath Neoplasms ; Pituitary Neoplasms ; Registries ; Republic of Korea*

Central neurocytoma (CN) typically presents as an intraventricular mass causing obstructive hydrocephalus. The first line of treatment is surgical resection with adjuvant conventional radiotherapy. Stereotactic radiosurgery (SRS) was proposed as an alternative therapy for CN because of its lower risk profile. The objective of this systematic analysis is to assess the efficacy of SRS for CN. A systematic analysis for CN treated with SRS was conducted in PubMed. Baseline patient characteristics and outcomes data were extracted. Heterogeneity and publication bias were also assessed. Univariate and multivariate linear regressions were used to test for correlations to the primary outcome: local control (LC). The estimated cumulative rate of LC was 92.2% (95% confidence interval: 86.5-95.7%, p<0.001). Mean follow-up time was 62.4 months (range 3-149 months). Heterogeneity and publication bias were insignificant. The univariate linear regression models for both mean tumor volume and mean dose were significantly correlated with improved LC (p<0.001). Our data suggests that SRS may be an effective and safe therapy for CN. However, the rarity of CN still limits the efficacy of a quantitative analysis. Future multi-institutional, randomized trials of CN patients should be considered to further elucidate this therapy.
Brain Neoplasms ; Follow-Up Studies ; Humans ; Hydrocephalus ; Linear Models ; Neurocytoma* ; Particle Accelerators ; Population Characteristics ; Publication Bias ; Radiosurgery* ; Radiotherapy ; Tumor Burden

Glioblastoma, the most common primary malignant brain tumor in adults, is highly aggressive and associated with a poor prognosis. Bevacizumab, a monoclonal antibody against the vascular endothelial growth factor receptor, has increasingly been used in the treatment of recurrent glioblastoma. It has achieved excellent rates of radiographic response, but most patients will progress after only a few months. Upon recurrence, tumors may not enhance, secondary to vascular normalization. We describe four patterns of radiographic progression commonly associated with Bevacizumab failure: 1) Distant enhancing tumor, 2) Local tumor progression without enhancement, 3) Diffuse gliomatosis-like infiltration, and 4) Local or multifocal progression, with enhancement. Some have noted an increased incidence of distant or diffuse disease upon recurrence, suggestive of a transition to a more aggressive phenotype, but a review of the literature suggests there is no conclusive evidence that Bevacizumab treatment is associated with an increased rate of distant or diffuse recurrence.
Adult ; Bevacizumab* ; Brain Neoplasms ; Glioblastoma ; Glioma* ; Humans ; Incidence ; Neuroimaging ; Phenotype ; Prognosis ; Receptors, Vascular Endothelial Growth Factor ; Recurrence ; Treatment Failure* ; Vascular Endothelial Growth Factor A