Radiologic Follow-up of Ruptured Arachnoid Cysts With or Without Hemorrhage: Five Case Reports and a Review of the Literature.

Arachnoid cysts are usually asymptomatic and discovered incidentally. However, cysts may occasionally rupture because of minor head trauma. We describe the radiologic follow-up of 5 patients with ruptured arachnoid cysts featuring spontaneous resolution, subdural hygroma formation, and cystic and subdural hemorrhage. From January 2004 through July 2020, 5 patients (1.3%) with ruptured arachnoid cysts were evaluated out of 388 patients with arachnoid cysts encountered at our institution at that time. The 5 patients were all male, and they ranged in age from 6-17 years (median, 12 years). The median duration of radiologic follow-up was 3.5 years (range, 2.3-10.1 years). All of the ruptured arachnoid cysts were overlying the temporal lobe with Galassi type II. The median cyst diameter was 4.9 cm (range, 4.4-8.9 cm). Four patients had a history of recent minor head trauma. There were no particular neurologic symptoms in their past medical history in all patients. In the follow-up, two patients' cysts resolved spontaneously without hemorrhage. One patient's cyst resolved post-burr-hole drainage for chronic subdural hemorrhage. Another patient, whose cyst led to a hemorrhage and chronic subdural hemorrhage, recovered following a craniotomy, hematoma removal, and cyst fenestration. Another patient, presenting with hygroma, cystic hemorrhage, and chronic subdural hemorrhage, was treated with burr-hole drainage. Three patients recovered postoperatively. Arachnoid cysts rarely rupture, and surgical intervention is required for some cases associated with hemorrhage. Postoperatively, all patients had good outcomes without complications in this series.

Multiple craniospinal tumors in a pediatric patient with neurofibromatosis type 2: a case report.

INTRODUCTION: Neurofibromatosis type 2 (NF-2) is an inherited disease, linked with abnormalities in the NF-2 gene, which is located on chromosome 22 and involved in merlin production. Many craniospinal tumors are common in individuals with NF-2. We present a case of NF-2 with the rapid symptomatic progression of multiple craniospinal tumors. CASE REPORT: A 12-year-old male complained of headache and hearing impairment in the right ear for 7 months. Brain magnetic resonance imaging (MRI) revealed a right frontal meningioma, bilateral vestibular and trigeminal schwannomas, and a brainstem tumor. He was diagnosed with NF-2 and underwent brain surgery and radiotherapy for chordoid meningioma. He complained of right leg motor weakness 5 months post-surgery. The spine MRI showed multiple heterogeneously enhanced masses spreading over the entire spinal cord. The symptomatic intradural extramedullary mass at the cervicothoracic area was removed and the histological finding was schwannoma. His leg motor weakness was relieved after surgery. At the 6-month follow-up, brain MRI revealed the progression of the vestibular schwannoma, trigeminal schwannoma, and brainstem tumor. The patient was treated with bevacizumab (5 mg/kg) every 2 weeks for 6 months. For 2 years, all of the craniospinal tumors were stable without neurological deterioration after the completion of chemotherapy. CONCLUSION: Meningiomas and schwannomas grow slowly in most patients with NF-2, but these multiple craniospinal tumors can show sudden rapid growth and manifest as neurological symptoms in a pediatric patient. These tumors could be controlled with local symptomatic and systemic bevacizumab treatments.

Staged operations for a hypervascular mixed germ cell tumor with growing teratoma syndrome: a case report.

INTRODUCTION: A mixed germ cell tumor with a teratoma component can become enlarged following chemotherapy, and such an event is diagnosed as growing teratoma syndrome. Removing large, hypervascular tumors including a tumor encased by developed vasculatures from the pineal region is challenging during a single operation. CASE REPORT: A 15-year-old male underwent chemotherapy for mixed germ cell tumors according to the KSPNO G082 protocol. This case of a mixed germ cell tumor with growing teratoma syndrome was recognized very early during chemotherapy. The tumor was completely removed during the staged operations. First, the anteriorly located tumor on the third ventricle was removed via the transcallosal interforniceal approach, and 1 month later, the occipital transtentorial approach was used for the pineal tumor with decreased vascularity. CONCLUSION: Performing staged operations could be recommended for large hypervascular pineal tumors, which can be safely removed during the second operation once vascularity has decreased.

The Tumor Control According to Radiation Dose of Gamma Knife Radiosurgery for Small and Medium-Sized Brain Metastases from Non-Small Cell Lung Cancer.

OBJECTIVE: The effectiveness of gamma knife radiosurgery (GKR) in the treatment of brain metastases is well established. The aim of this study was to evaluate the efficacy and safety of maximizing the radiation dose in GKR and the factors influencing tumor control in cases of small and medium-sized brain metastases from non-small cell lung cancer (NSCLC). METHODS: We analyzed 230 metastatic brain tumors less than 5 mL in volume in 146 patients with NSCLC who underwent GKR. The patients had no previous radiation therapy for brain metastases. The pathologies of the tumors were adenocarcinoma (n=207), squamous cell carcinoma (n=18), and others (n=5). The radiation doses were classified as 18, 20, 22, and 24 Gy, and based on the tumor volume, the tumors were categorized as follows : small-sized (less than 1 mL) and medium-sized (1-3 and 3-5 mL). The progression-free survival (PFS) of the individual 230 tumors and 146 brain metastases was evaluated after GKR depending on the pathology, Eastern Cooperative Oncology Group (ECOG) performance score (PS), tumor volume, radiation dose, and anti-cancer regimens. The radiotoxicity after GKR was also evaluated. RESULTS: After GKR, the restricted mean PFS of individual 230 tumors at 24 months was 15.6 months (14.0-17.1). In small-sized tumors, as the dose of radiation increased, the tumor control rates tended to increase (p=0.072). In medium-sized tumors, there was no statistically difference in PFS with an increase of radiation dose (p=0.783). On univariate analyses, a statistically significant increase in PFS was associated with adenocarcinomas (p=0.001), tumors with ECOG PS 0 (p=0.005), small-sized tumors (p=0.003), radiation dose of 24 Gy (p=0.014), synchronous lesions (p=0.002), and targeted therapy (p=0.004). On multivariate analyses, an improved PFS was seen with targeted therapy (hazard ratio, 0.356; 95% confidence interval, 0.150-0.842; p=0.019). After GKR, the restricted mean PFS of brain at 24 months was 9.8 months (8.5-11.1) in 146 patients, and the pattern of recurrence was mostly distant within the brain (66.4%). The small and medium-sized tumors treated with GKR showed radiotoxicitiy in five out of 230 tumors (2.2%), which were controlled with medical treatment. CONCLUSION: The small-sized tumors were effectively controlled without symptomatic radiation necrosis as the radiation dose was increased up to 24 Gy. The medium-sized tumors showed potential for symptomatic radiation necrosis without signifcant tumor control rate, when greater than 18 Gy. GKR combined targeted therapy improved the tumor control of GKR-treated tumors.

Differentiating Radiation Necrosis from Brain Tumor Using Hyperpolarized Carbon-13 MR Metabolic Imaging.

PURPOSE: Differentiation between radiation-induced necrosis and tumor recurrence is crucial to determine proper management strategies but continues to be one of the central challenges in neuro-oncology. We hypothesized that hyperpolarized 13C MRI, a unique technique to measure real-time in vivo metabolism, would distinguish radiation necrosis from tumor on the basis of cell-intrinsic metabolic differences. The purpose of this study was to explore the feasibility of using hyperpolarized [1-13C]pyruvate for differentiating radiation necrosis from brain tumors. PROCEDURES: Radiation necrosis was initiated by employing a CT-guided 80-Gy single-dose irradiation of a half cerebrum in mice (n = 7). Intracerebral tumor was modeled with two orthotopic mouse models: GL261 glioma (n = 6) and Lewis lung carcinoma (LLC) metastasis (n = 7). 13C 3D MR spectroscopic imaging data were acquired following hyperpolarized [1-13C]pyruvate injection approximately 89 and 14 days after treatment for irradiated and tumor-bearing mice, respectively. The ratio of lactate to pyruvate (Lac/Pyr), normalized lactate, and pyruvate in contrast-enhancing lesion was compared between the radiation-induced necrosis and brain tumors. Histopathological analysis was performed from resected brains. RESULTS: Conventional MRI exhibited typical radiographic features of radiation necrosis and brain tumor with large areas of contrast enhancement and T2 hyperintensity in all animals. Normalized lactate in radiation necrosis (0.10) was significantly lower than that in glioma (0.26, P = .004) and LLC metastatic tissue (0.25, P = .00007). Similarly, Lac/Pyr in radiation necrosis (0.18) was significantly lower than that in glioma (0.55, P = .00008) and LLC metastasis (0.46, P = .000008). These results were consistent with histological findings where tumor-bearing brains were highly cellular, while irradiated brains exhibited pathological markers consistent with reparative changes from radiation necrosis. CONCLUSION: Hyperpolarized 13C MR metabolic imaging of pyruvate is a noninvasive imaging method that differentiates between radiation necrosis and brain tumors, providing a groundwork for further clinical investigation and translation for the improved management of patients with brain tumors.

Dural-Based Primary Central Nervous System Lymphoma with Bone Invasion.

BACKGROUND: Primary central nervous system lymphoma (PCNSL) occurs frequently in a deep intraparenchymal location. It rarely occurs in the meninges, and bone invasion is uncommon. CASE DESCRIPTION: A 12-year-old boy was admitted to our hospital with a history of headache and seizures. Magnetic resonance imaging showed a 4-cm-sized dural-based mass with osteolytic bone change. Craniotomy and tumor removal were performed. Operative findings showed a dural-based hard tumor, including necrosis and hemorrhage, and the skull was focally destroyed by tumor cells. The tumor was completely removed. Pathologically, large atypical cells with pleomorphic nuclei and frequent mitoses were found. The tumor cells were immune-positive for CD30, epithelial cell antigen, and anaplastic lymphoma kinase. The final diagnosis was anaplastic lymphoma kinase-positive anaplastic large cell lymphoma (ALCL). There was no evidence of systemic cancer. The boy underwent chemotherapy following the Non-Hodgkin Lymphoma-Berlin-Frankfurt-Münster protocol. There was no recurrence after a 16-month chemotherapy-off period. CONCLUSIONS: ALCL is not a common type of PCNSL, and central nervous system ALCL frequently involves the dura and meninges compared with PCNSL in general. However, osteolytic bone lesions are rarely seen in ALCL. This case thus represents a rare case of dural-based ALCL with bone invasion.

Intracranial Inflammatory Pseudotumor Associated with Idiopathic Hypertrophic Pachymeningitis Mimicking Malignant Tumor or High-Grade Meningioma.

BACKGROUND: Idiopathic hypertrophic pachymeningitis (IHP) is a rare clinical disease characterized by inflammatory fibrosis, which causes diffuse thickening of the dura mater. The inflammatory fibrosis will be locally invasive in nature but will be characterized by a benign histological appearance, known as an inflammatory pseudotumor. We present the case of a patient with an infiltrative lesion involving the right frontal convexity diagnosed as IHP, which had been suspected to be a malignant tumor or high-grade meningioma from the preoperative radiological findings. CASE DESCRIPTION: A 59-year-old man was admitted to our hospital because of a temporary loss of consciousness. Contrast-enhanced magnetic resonance imaging (MRI) of the brain showed a 30-mm × 12-mm mass located in the right frontal convexity. The lesion had an isointensity to high-intensity signal with moderate periregional edema on T2-weighted MRI, and homogeneous enhancement, including a necrotic portion with a long dural tail along the right frontal convexity, after contrast-enhanced MRI. Thus, our preliminary diagnosis was high-grade meningioma or a malignant tumor. We decided to surgically remove the tumor. Intraoperatively, the lesion appeared as a dural-based yellowish mass with partial infiltration of the cortex. Histopathological examination of the lesion revealed thickened meninges with marked fibroinflammatory changes. The inflammatory changes extended into the underlying brain parenchyma and were centered in the perivascular spaces. The lesion showed abundant lymphoplasmacytic infiltration with fibrosis. Immunohistochemistry revealed mixed T and B lymphocytes and plasma cells. Only a small number of IgG4-positive cells were identified. From these findings, we finally concluded that the diagnosis was IHP. The patient did not receive any further steroid therapy, because the patient had no evidence of systemic autoimmune disease. A follow-up brain MRI scan was performed 6 month after surgery, which revealed no recurrence of the lesion. CONCLUSIONS: Surgical treatment can be the first treatment option when the lesion is not localized to a critical portion of the brain. Thus, it might be possible to arrive at a definitive diagnosis histologically and determine additional treatment strategies. Also, if the surgeons are confident that the IHP has been removed completely, additional steroid therapy might not be necessary.

Multiple Brain Biopsies for Epstein-Barr Virus-Positive Diffuse Large B-Cell Lymphoma with Extensive Necrosis in a Posttransplant Patient.

BACKGROUND: Epstein-Barr virus (EBV)-positive diffuse large B-cell lymphoma (DLBCL) in an immunocompromised patient with organ transplantation demonstrated unusual brain magnetic resonance imaging (MRI) findings. Recognition of EBV-positive DLBCL by radiologists on MRI may prevent unnecessary neurosurgical resection, and it could be important to obtain viable cells for accurate diagnosis on stereotactic biopsy because of extensive necrosis. CASE DESCRIPTION: A 62-year-old woman presented to the emergency department with left hemiparesis grade III and dysarthria lasting for 3 weeks. She underwent kidney transplantation in 2007 and was taking immunosuppressants and had hypothyroidism. Brain MRI showed a 3.8-cm peripheral enhancing lesion with extensive central necrosis in association with marked perilesional edema. The irregular ringlike enhancing lesion showed diffusion restriction and mildly increased regional cerebral blood volume in the rim portion of the mass. 11C-Methionine positron emission tomography revealed slightly increased uptake in the peripheral lesion. The provisional diagnosis was a high-grade glioma. Stereotactic multiple biopsies were performed for the central necrotic area and peripheral enhancing lesion. The nonenhancing areas showed only necrotic material, without viable cells, and the enhancing portion showed viable cells for an accurate diagnosis in a frozen biopsy specimen. The pathologic diagnosis was EBV-positive DLBCL with extensive necrosis. Positron emission tomography of the chest, abdomen, pelvis, and neck soft tissues ruled out systemic diseases. She underwent whole-brain radiotherapy at a dose of 30.6 Gy. Eight months later, her neurologic symptoms had improved, with a stable brain lesion and improved perilesional edema. CONCLUSIONS: We report an immunocompromised patient with EBV-positive DLBCL, which showed atypical MRI findings, including extensive necrosis. Multiple biopsies were required for final diagnosis.

The Application of Magnetic Resonance Imaging-Deformed 11C-Methionine-Positron Emission Tomography Images in Stereotactic Radiosurgery.

BACKGROUND: Although 11C-methionine positron emission tomography (MET-PET) images can be fused with magnetic resonance (MR) images using planning software for gamma knife radiosurgery (GKR), the stereotactic information has limited value in patients with recurrent malignant brain tumor due to the difference in imaging protocols between MET-PET and MR images. The aim of this study was to evaluate the clinical application of MR imaging (MRI)-deformed MET-PET images in GKR using a deformable registration tool. METHODS: We examined the enhanced MR stereotactic images, MET-PET and MRI-deformed MET-PET images without stereotactic information for 12 newly developed metastatic brain tumors. MET-PET and MRI-deformed MET-PET images were co-registered with the MR stereotactic images using radiosurgery planning software. Visual analysis was performed to determine whether the MET-PET and MR images matched better after using the deformable registration tool. In addition, the matching volume between MR and MET-PET images was compared before and after applying this tool. The matching volume was calculated as the metabolic tumor volume on the MET-PET images, including the MR-enhanced volume. The matching percentage was calculated as the matching volume divided by the MR-enhanced volume, multiplied by 100. RESULTS: Visual analysis revealed that the MRI-deformed MET-PET images provided the same axial plane as that of the MR images, with the same window level, enabling easy identification of the tumor with the radiosurgery planning software. The mean matching percentage of the MET-PET/MR fusion images was 61.1% (range 24.7-94.7) and that of the MRI-deformed MET-PET/MR fusion images was 63.4% (range 20.8-94.3). No significant difference was found in the matching percentage between the two types of fusion images (p = 0.754). CONCLUSIONS: The MRI-deformed MET-PET images enable utilization of the functional information when planning a treatment in GKR without significant volume change.

Tumor control of third ventricular central neurocytoma after gamma knife radiosurgery in an elderly patient: A case report and literature review.

RATIONALE: Central neurocytoma is rare benign tumor that occurs in high probability in young adults in the lateral ventricle. Herein, we report an unusual case of an elderly woman who was diagnosed with central neurocytoma isolated to the third ventricle. This deeply located tumor was effectively treated using gamma knife radiosurgery (GKR). PATIENT CONCERNS: A 79-year-old woman was admitted to hospital with gait disturbance and cognitive dysfunction. DIAGNOSIS: Brain magnetic resonance imaging (MRI) revealed a homogenously enhancing multilobulated mass in the posterior third ventricle measuring 1.8 cm in size. The tumor was diagnosed as a central neurocytoma isolated to the third ventricle. INTERVENTIONS: Neuronavigation-guided endoscopic third ventriculostomy and biopsy were performed. One week following surgery, GKR was performed using a prescribed dose of 14 Gy with 50% isodose lines, and a target volume of 1.62 cc. OUTCOMES: Three months after GKR, brain MRI revealed a decrease in the size (to 1.4 cm) of the multilobulated strong enhancing mass in the posterior third ventricle, and the patient's symptom of confusion was improved. LESSONS: Previous studies have reported that tumors in unusual locations, such as those isolated to the third ventricle, are different according to age, either in young children or elderly individuals. Although complete surgical resection is an effective treatment for central neurocytoma, it is often difficult to approach these tumors through surgery. GKR could, therefore, be an alternative primary treatment option for deeply located central neurocytomas in elderly patients.

Disseminated juvenile xanthogranuloma occurring after treatment of Langerhans cell histiocytosis: a case report.

CASE PRESENTATION: An 11-year-old boy presented with a complaint of a painful temporal mass. Brain magnetic resonance imaging (MRI) showed a 3-cm-sized, homogeneously enhancing mass in the greater wing of the left sphenoid bone, which was diagnosed as Langerhans cell histiocytosis (LCH). Chemotherapy with vincristine and prednisolone was performed for 1 year. After 1 year and 11 months off treatment, he developed symptoms such as polydipsia and polyuria. Brain MRI showed thickening of the pituitary stalk with enhancement, suggestive of LCH involvement, and no recurrence in the sphenoid bone. After 4 years and 4 months off treatment, he developed multiple, subcutaneous, asymptomatic, and yellowish variable-sized papules on his face, posterior neck, and back, which were pathologically diagnosed as juvenile xanthogranuloma (JXG). Brain MRI revealed multifocal enhancing skull lesions in the left parietal, right frontal, and left occipital bones, which were also diagnosed as JXG. After 5 years and 8 months off treatment, the number of variable-sized skin lesions was increased without changes in the lesions in the skull and pituitary stalk. CONCLUSION: We report a case of disseminated JXG occurring after treatment of LCH. These clinical co-presentations suggested a close relationship between their pathogenesis.

Visual outcome after endoscopic third ventriculostomy for hydrocephalus.

PURPOSE: Hydrocephalus-related symptoms are mostly improved after successful endoscopic third ventriculostomy (ETV). However, visual symptoms can be different. This study was focused on visual symptoms. We analyzed the magnetic resonance images (MRI) of the orbit and visual outcomes. METHODS: From August 2006 to November 2016, 50 patients with hydrocephalus underwent ETV. The male-to-female ratio was 33:17, and the median age was 61 years (range, 5-74 years). There were 18 pediatric and 32 adult patients. Abnormal orbital MRI findings included prominent subarachnoid space around the optic nerves and vertical tortuosity of the optic nerves. We retrospectively analyzed clinical symptoms, causes of hydrocephalus, ETV success score (ETVSS), ETV success rate, ETV complications, orbital MRI findings, and visual impairment score (VIS). RESULTS: The median duration of follow-up was 59 months (range, 3-113 months). The most common symptoms were headache, vomiting, and gait disturbance. Visual symptoms were found in 6 patients (12%). The most common causes of hydrocephalus were posterior fossa tumor in 13 patients, pineal tumor in 12, aqueductal stenosis in 8, thalamic malignant glioma in 7, and tectal glioma in 4. ETVSS was 70 in 3 patients, 80 in 34 patients, and 90 in 13 patients. ETV success rate was 80%. ETVSS 70 showed the trend in short-term survival compared to ETVSS 90 and 80. ETV complications included epidural hematoma requiring operation in one patient, transient hemiparesis in two patients, and infection in two patients. Preoperative abnormal orbital MRI findings were found in 18 patients and postoperative findings in 7 patients. Four of six patients with visual symptoms had abnormal MR findings. Three patients did not show VIS improvement, including two with severe visual symptoms. CONCLUSIONS: Patients with severe visual impairment were found to have bad outcomes. The visual symptoms related with increased intracranial pressure should be carefully monitored and controlled to improve outcomes.

Ependymomas: Prognostic Factors and Outcome Analysis in a Retrospective Series of 33 Patients.

BACKGROUND: The purpose of this study was to evaluate the prognostic factors and outcomes in patients with ependymoma to management plans. METHODS: Between 1997 and 2013, 33 patients with 25 ependymomas (WHO grade II) and eight anaplastic ependymomas (WHO grade III) were pathologically diagnosed. Six were pediatric patients (mean age, 6.15 years; range, 1.3-11 years), while 27 were adults (mean age, 47.5 years; range, 19-70 years). Of those, there were 12 adult patients with totally resected ependymomas without anaplastic pathology and adjuvant treatment. Prognostic factors were assessed in ependymoma patients. Prognostic factors were studied using Kaplan-Meier estimates in subgroups. RESULTS: For six pediatric patients, the progression-free survival (PFS) was 43.7±13.5 months, and the overall survival (OS) was 58.1±13.7 months. For 27 adult patients, the PFS was 125.6±14.3 months, and the OS was 151.2±12.5 months. Age demonstrated a statistically significant effect on PFS (p=0.03) and OS (p=0.03). In adult ependymomas, the extent of tumor removal significantly affected PFS (p=0.03) and trended towards an effect on OS (p=0.06). Out of 12 patients with totally resected ependymomas without anaplastic pathology and adjuvant treatment, one patient showed tumor recurrence during follow-up (mean, 93.5 months; range, 27.9-162.7 months). CONCLUSION: Adult patients with ependymomas were found to have better survival rates compared to pediatric patients. We suggest that totally resected adult ependymomas without anaplastic pathology could be observed without any adjuvant treatment, regardless of the tumor location.

Disease progression patterns of bevacizumab responders with recurrent malignant gliomas.

Tumor progression in patients with recurrent malignant glioma who respond to bevacizumab (BEV) is difficult to assess. The current study reviewed the clinical and radiological results of patients following a BEV-based chemotherapy regimen, and evaluated disease progression patterns in patients who responded to BEV therapy. From August 2011 to November 2015, 24 patients (18 glioblastoma cases and 6 anaplastic astrocytoma cases) were treated with BEV-based chemotherapy. In total, 6 patients were treated with BEV alone and 18 patients were treated with BEV combined with irinotecan. The male-female ratio was 10:14, and the median age was 47.5 years (range, 29-69). Patient performance status (PS) was classified using the Eastern Cooperative Oncology Group PS scores as follows: PS 1 (n=3), PS 2 (n=9), PS 3 (n=9) and PS 4 (n=3). Treatment-associated complications were also analyzed according to the National Cancer Institute Common Terminology Criteria for Adverse Events version 4.0. Treatment responses were estimated using the Response Assessment in Neuro-Oncology Criteria. Progression-free survival (PFS) following treatment, patterns of disease progression and overall survival following treatment failure were also analyzed. The median PFS was 2.8 months (range, 0.6-10.1). In total, 2 patients did not continue treatment due to rectal bleeding and severe hematologic toxicity. Amongst the BEV responders (n=16, 72.7%), there was clinical deterioration without significant radiological progression in 2 patients (n=2, 12.5%). Radiological progression of non-enhancing lesions without enhancement flare-ups was observed in 6 patients (42.9%). A total of 3 of those lesions were diffuse and 3 were focal. Increased lesion enhancement was observed in 8 patients (57.1%). Of the non-responders (n=6, 27.3%), diffuse enlargement of non-enhancing lesions was detected in 2 patients and an increase in lesion enhancement occurred in 4 patients. BEV complete responders (n=3) radiologically progressed with enlarged T2/fluid attenuation inversion recovery lesions without enhancement, followed by enhancement flare-ups. Following BEV treatment failure, 8 patients received a number of adjuvant treatments and the overall survival was 4.5 months (range, 0.4-34.0). Clinical symptoms and radiological alterations of non-enhancing lesions must be evaluated in order to assess tumor progression in the BEV responders, particularly in patients who have achieved complete remission.

Primary Central Nervous System Vasculitis Mimicking a Cortical Brain Tumor: A Case Report.

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.

Subependymal Giant Cell Astrocytoma Presenting with Tumoral Bleeding: A Case Report.

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.

Predictive factors for surgical outcome in anterior clinoidal meningiomas: Analysis of 59 consecutive surgically treated cases.

Despite the advances in the microsurgical technique and anatomical understanding of the anterior and middle skull base, anterior clinoidal meningiomas are still challenging lesions to resect completely and safely due to their intimate relationship with vital neurovascular structures. We report predictive factors for tumor recurrence and postoperative complications based on surgical outcome of patients with anterior clinoidal meningiomas treated at our institution.Fifty-nine consecutive patients with anterior clinoidal meningioma who were surgically treated between March, 1993, and July, 2015, were reviewed retrospectively. For microsurgical tumor removal, orbitocranial or orbitozygomatic (78.0%), extended pterional (15.3%) and subfrontal approach (6.8%) were performed.The median follow-up duration was 54.1 months. Gross total resection (GTR, Simpson's grade I or II) was achieved in 38 patients (64.4%). The overall recurrence rate (new lesion in GTR cases and re-growth in non-GTR cases) was 18.6%. GTR (Hazard ratio [HR] 0.014, 95% confidence interval [CI] 0.001-0.256; P = .004), absence of internal feeder (HR 0.058, 95% CI 0.004-0.759; P = .030) and benign pathology (WHO grade I, HR 0.056, 95% CI 0.005-0.674; P = .023) were independent prognostic factors for recurrence-free. Fourteen patients (23.7%) developed permanent complications. The most common complication was cranial nerve injury (n = 6; 10.2%), followed by postoperative hemorrhage/infarction, hydrocephalus and infection. Larger size (≥ 40 mm) was significant as an independent predictive factor for permanent complication (HR 0.139, 95% CI 0.030-0.653; P = .012). Old age (≥60 years, P = .056) and peritumoral edema (thickness ≥ 5 mm, P = .303) did not reach statistical significance in multivariate analysis.In surgical resection of anterior clinoidal meningiomas, various clinicoradiological factors were related with resection degree, complication, and progression rate. Although our results showed acceptable resection degree and morbidity, mortality, and recurrence rate, compared to the results of past, anterior clinoidal meningioma remain as neurosurgical challenges because of their close contact to critical vascular and neural structures.

Meningiomas with Rhabdoid or Papillary Components : Prognosis and Comparison with Anaplastic Meningiomas.

Papillary and rhabdoid meningiomas are pathologically World Health Organization (WHO) grade III. Any correlation between clinical prognosis and pathologic component is not clear. We analyzed the prognoses of patients with meningiomas with a rhabdoid or papillary component compared to those of patients with anaplastic meningiomas. From 1994 to June 2013, 14 anaplastic meningiomas, 6 meningiomas with a rhabdoid component, and 5 meningiomas with papillary component were pathologically diagnosed. We analyzed magnetic resonance imaging (MRI) findings, extent of removal, adjuvant treatment, progression-free survival (PFS), overall survival (OS), and pathologic features of 14 anaplastic meningiomas (group A), 5 meningiomas with a predominant (≥50%) papillary or rhabdoid component (group B1), and 6 meningiomas without a predominant (<50%) rhabdoid or papillary component (group B2). Homogeneous enhancement on MRI was associated with improved PFS compared to heterogeneous enhancement (p=0.025). Depending on pathology, the mean PFS was 134.9±31.6 months for group A, 46.6±13.4 months for group B1, and 118.7±19.2 months for group B2. The mean OS was 138.5±24.6 months for group A and 59.7±16.8 months for group B1. All recurrent tumors were of the previously diagnosed pathology, except for one tumor from group B1, which recurred as an atypical meningioma without a papillary component. Group B1 tumors showed a more aggressive behavior than group B2 tumors. In group B2 cases, the pathologic findings of non-rhabdoid/papillary portion could be considered for further adjuvant treatment.

Progressive Multiple Cavernous Angiomas after Radiotherapy in a Pediatric Patient with Medulloblastoma: A Case Report.

BACKGROUND: We report a case of progressive multiple cavernous angiomas. PATIENT AND METHOD: A 16-year-old boy presented with a 2-month history of headache and dizziness. Six years earlier, he underwent surgery for cerebellar medulloblastoma and subsequent chemoradiotherapy according to the M-051 protocol of the Korean Society of Pediatric Neuro-Oncology. Follow-up brain magnetic resonance imaging (MRI) revealed a tiny hemorrhage on the cerebellum 3 months after 23.4-Gy craniospinal and 32.4-Gy boost radiotherapy. RESULT: The multiple hemorrhagic lesions had progressively developed on the whole brain without any symptoms for 6 years. On admission, MRI revealed a 1.5-cm enlarged mass with subacute hemorrhage on the right frontal area. The mass was totally removed and diagnosed as cavernous angioma. However, 5 months later, the patient complained of a headache. MRI revealed 1.4- and 0.7-cm enlarged masses on the left frontal and right temporal areas with internal hemorrhage, respectively. The left frontal mass was totally removed, and the histopathological finding was suggestive of cavernous angioma. CONCLUSION: This case showed early-developed multiple hemorrhagic lesions after radiotherapy, which had been progressive and were associated with some symptomatic cavernous angiomas. Pediatric patients with brain radiotherapy should undergo radiological check-up to identify vascular lesions, especially symptomatic patients.

Pediatric brainstem abscess with hemorrhage mimicking diffuse intrinsic pontine glioma: a case report.

PURPOSE: We report a rare case of brainstem abscess with hemorrhage mimicking diffuse intrinsic pontine glioma (DIPG). METHODS: A 7-month-old baby girl presented with lethargy and poor oral feeding. She had the mild fever for 1 month. Brain computed tomography revealed the hypodense lesion in the pons. Brain magnetic resonance images (MRI) showed around 1.4-cm-sized rim-enhanced mass with perilesional edema and internal hemorrhage in the pons. The cerebral blood volume was increased in the rim-enhanced area. The provisional diagnosis was DIPG, but the mass did not show the expansile mass with encasement of the basilar artery on the ventral pons. RESULTS: The biopsy was done via the floor of the fourth ventricle, and the pathologic findings showed the many inflammatory cells and CD68-immunopositive macrophage which were compatible with abscess. The antibiotics with ceftriaxone and metronidazole were administrated for 11 weeks, and the follow-up MRI showed the slightly small enhanced lesion without central necrotic area. Three years later, follow-up MRI revealed the encephalomalacic change and atrophy of the pons. She had the stable neurologic deficit of left facial palsy and right hemiparesis. CONCLUSION: The biopsy could be necessary for pontine lesions without typical radiologic findings of DIPG.