Primary central nervous system lymphoma (PCNSL) is a rare extranodal lymphomas. Histopathologically, PCNSLs typically show patchy, poorly demarcated, and angiocentric proliferation different from other systemic lymphomas. From these perivascular cuffs, lymphoma cells invade neural parenchyma or subarchnoid spaces resembling encephalitis. Generally, diffusion of large B-cell lymphomas (DLBCL) constitute most PCNSLs. The analysis regarding phenotypes of marker expressions of DLBCL in the central nervous system indicates that they might be distinct lymphomas that originate from a late germinal center to an early postgerminal center. Regarding the growth and infiltraion of PCNSL, they are dependent on neoangiogenesis. Although PCNSLs may not require sufficient neoangiogenesis at the start of PCNSLs, it may instead require a higher rate of neoangiogenesis as they infiltrate and destroy the brain parenchyma.Understanding the role of the chemokine network in the neoangiogenesis and proliferation of PCNSLs clarifies the above characteristics of PCNSLs. As one of the pathogenesis of PCNSLs, Epstein-Barr virus (EBV) has been recently implicated in patients with or without immunodeficiency. Particularly, EBV-positive PCNSL in immunocompetent hosts may be related to immunological deterioration derived from the aging processes.This review will focus on the above characteristic of histopathology, the mechanism of neoangiogenesis, and pathogenesis of PCNSLs.

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We describe the clinicopathological features of the central nervous system （CNS） embryonal tumors mainly Atypical Teratoid / Rhabdoid Tumors （AT/RTs）, Medulloblastomas （MB） and primitive neuroectodermal tumors （PNETs）.The subtypes of MB are included such as desmoplastic/nodular MB, MB with extensive nodularity, anaplastic MB, and large cell MB. Definition of MB is a malignant invasive embryonal tumor of the cerebellum with preferential manifestation in children, predominantly neuronal differentiation, and an inherent tendency to metastasize via CSF pathways. Recent prognosis of the patients with MB has been improved by advanced chemotherapy and radiotherapy. PNET is a heterogeneous group of tumors occurring predominantly in children and adolescents. They may arise in the cerebral hemispheres, brain stem, or spinal cord, and are composed of undifferentiated or poorly differentiated neuroepithelial cells which may display divergent differentiation along neuronal, astorocytic and ependymal line. CNS/supratentorial PNET is an embryonal tumor composed of undifferentiated or poorly differentiated neuroepithelial cells. Tumors with only neuronal differentiation are termed cerebral neuroblastomas or, if ganglion cells are also present, cerebral ganglionneuroblastomas.Recent prognosis of the patients with PNET has been also improved by advanced chemotherapy and radiotherapy like MB.AT/RT is defined as a highly malignant CNS tumor predominantly manifesting in young children, typically containing rhabdoid cells, often with primitive neuroectodermal cells and with divergent differentiation along epithelial, mesenchymal, neuronal or glial lines; associated with inactivation of the INI1/hSNF5 gene in virtually all cases. As the tumor entity is new and extremely malignant tumor, prognosis of the patients with AT/RT is still poor.

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Surgery for tuberculum meningiomas is still challenging to obtain good functional outcomes. There are various surgical approaches advocated, because each approach has both advantages and disadvantages. We retrospectively analyzed the consecutive 35 cases of tuberculum sellae meningiomas that were mainly operated via modified frontobasal interhemispheric approach （IHA） during the last 7 years. Complete tumor resection was achieved in all but 3 cases with severe adhesion to the neural tissue. Out of 26 cases with preoperative visual impairment, 24 cases （92%） showed functional improvement. Postoperative anosmia was seen in 3 cases （12%） out of 27 IHA procedures. Our operative procedures are described in detail, and crucial points, including the strategy and the important operative techniques, are discussed.

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In this paper, we have examined the current status of brain metastases of lung cancer which indicating highest incidence of brain metastasis, including its clinical characteristics and the therapeutic outcomes. Of the 1335 patients who underwent resection of lung cancer in our hospital between 2005 and 2010, we examined the 63 cases who had the brain metastases. Brain metastases occur in three years after lung resection in all cases, the median time was 13.5 months. Initial treatment for brain metastases were as follows:17 patients (27.9%) of craniotomy plus whole brain radiotherapy (WBRT), 39 (63.9%) of stereotacticradiosurgery (SRS), 2 (3.3%) of WBRT alone, and 3 (4.9%) of chemotherapy alone. At the time in September 2012, survival patients were 26 cases, and patients who died were 32 cases. The cause of death was the original cancer in 27 cases, and was central nervous system (CNS) death in 5 cases. Four (80%) of the CNS death were meningeal carcinomatosis. The median survival time (MST) of the SRS group was 38 months, whereas MST of craniotomy + WBRT group was 20 months. However, between these two groups no statistically significant difference is observed. Since 2008, we have encountered the 27 cases of the meningeal carcinomatosis which had been confirmed by examination of cerebrospinal fluid cells during the course of lung cancer. MRI findings of meningeal carcinomatosis were frequently disappeared by administration of Erlotinib. The average survival time of Erlotinib administration group (15 cases) was 315 days, whereas the average survival time of Erlotinib untreated group (12 cases) was 59 days. Between these two groups statistically significant difference is observed. Already, efforts of MRI screening for brain metastases have been made in Thoracic Surgery, early detection of brain metastases is possible. It is thought to increase the importance of stereotactic radiotherapy. On the other hand, a potent molecular target for meningeal carcinomatosis is emerging and the effective chemotherapy will be expected.

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In recent years, advances in the surgical techniques and technologies of glioma removal have driven new paradigms in surgical strategies. We first evaluate the relationship between extensive glioma removal and prognosis and then we introduced novel techniques and technologies to maximize the removal rate and minimal complication rate. These are functional mapping and monitoring under awake craniotomy, intraoperative MRI, and photodynamic diagnosis. These technologies compose of “ information-guided surgery” in which surgeons make a decision not by their own experience but by various kinds of objective and solid information, such as anatomical, functional, and histological information respectively.

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The classification and definition of the vascular tumors in central nervous system is not clear, but which tumors are accepted as pathological entities showing many blood vessels among the tumor cells.Vascular tumors are listed as hemangioma,hemangioblastoma, hemangiopericytoma, hemangioendothelioma, angiomatous meningioma, atypical meningioma, anaplastic meningioma, which tumors are made a different diagnosis on MRI, light microscopy, and electron microscopy.The correct diagnosis among the meningeal tumors is necessary at pre and post operative stage for neurosurgeons, because of the good therapy.

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The patient was a 43-year-old female who had been conscious of exophthalmos of her right eye ball for 4years. She admitted to Department of Neurosurgery of our hospital.The CT scan revealed remarkable thickening with abnormal and irregular, ground glass shadow of right sphenoid bone. Fibrous dysplasia was considered as diagnosis of the bone lesion in the first impression, although the CT scan showed neither apparent nidus nor typical feature to fibrous dysplasia. Then, the pathological right sphenoid bone was resected piecemeal on the operation. The cut surface of resected fragments appeared to be gritty like a sand-paper. As histopathologically, flat arrangement of small round cells including whorled pattern around capillaries was observed and the tumor cells were positive for EMA on the immunohistochemical study, histopathological diagnosis of meningothelial meningioma was made. As meningioma was not present in other areas than right sphenoid bone, the present case was diagnosed as a rare case of primary intra-osseous meningioma. Review of the CT scan films demonstrates “inward and outward fluff” in the periphery of right sphenoid bone that is characteristic feature to intra-osseous meningioma.

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Glioblastoma is the most common and malignant primary brain tumor. Factors such as age, Karnofsky performance score （KPS）, and extent of resection have been shown to be prognostic for survival in newly diagnosed malignant glioma patients. O6-methylguanine DNA-methyltransferase （MGMT） promoter methylation status is also a prognostic factor in newly diagnosed glioblastoma patients. IDH1 mutation detected by direct sequencing or by immunochemistry of its more frequent form has been reported to be a strong and independent prognostic factor in gliomas whatever the grade. 1p/19q deletions are prognostic factors in oligodendroglial tumors and predict better survival after both chemotherapy and radiotherapy. Expression of immunohistochemical staining for α-Internexin was reported to correlate with 1p/19q deletions.We have two glioblastoma long term survivors and these cases showed similar clinical and radiological presentation with other glioblastoma cases who relapsed and survived less than a year. We investigated immunohistochemical staining for IDH1-R132H, MGMT and α-Internexin in these two cases and all stainings were negative. We should have more attention for other prognostic factors in glioblastoma long term survivors.

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Multiple myeloma（MM） is a systemic plasmacytosis, with a poor prognosis, which generally causes death within an average of two to three years because of infection and renal failure. Males in their seventh decade are the most likely to develop the disease, and comprise about 10% of all cases of hematopoietic system malignant neoplasms^[5]. The characteristic findings of MM that can be used for a diagnosis include the frequent appearance of “punched-out” lesions, such as, skull lesions anemia and increased plasmacytoma（>10%） in the bone marrow. However, the positive rate of various examinations is 70〜90% , and all findings may not be all present. According to the study reported by Okuda et al of 30 patients who harbored a huge mass in their skull, although the average age was 61 years old （36-62）, which was similar to MM, there were eight males and 22 females, and this elderly female dominance was not typical of MM. That prognosis is uneven, but generally poor. Approximately half of all patients died, and the one-year survival rate for such patients was around 50%（4）. In the cases able to undergo tumor removal, the prognosis at 1〜2 years tends to be better than the nonsurgical cases. When these tumors, especially those in the convexity area, can be removed, a surgical strategy is recommended. In non-surgical cases and for postoperative treatment, radiation and chemotherapy are recommended, because these can provide at least temporary responses. However, the treatment often requires two to six, months to induce tumor disappearance, so some neurological deficits may take a long time to resolve, or may never resolve completely （4）. However, despite the better prognosis following surgery, an operation is considered to be contraindicated when there is cerebral spinal fluid dissemination. Although case 1 in this paper was a very rare SPB arising from a centered sphenoid bone, no bone marrow abnormalities were found in a systemic examination. Because solitary SMM was diagnosed in case1, radiation and chemotherapy were added. Because case 2 was diagnosed to have a pure SPB, additional therapies were not t added. Both of our cases were solitary cases. Though they are both still alive and have had a good course strict observation especially in case 2, will be needed.

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[Object] Pathological investigation of adult embryonal tumor [Material and Method] The patient was 38 years-old male. He had the symptom of relatively high grade fever and headache. After the continuation of these symptoms for about 2 weeks, speech disturbance occurred. He consulted other hospital and received MRI. MRI showed mass lesion in the left frontal lobe which was low intensity on T1WI, and high intensity on T2WI and showed ring enhancement on Gd enhanced study. He was referred to our hospital. His symptom on admission was right central type facial palsy, right hemiparesis (4/5), and non-fluent aphasia.We performed biopsy and then performed subtotal resection because of worsening of the symptoms. After the operation he received cerebrospinal irradiation therapy and chemotherapy.Pathological findings suggested highly malignant neuroepithelial (embryonal) tumor including CSN PNET. [Comments in meeting] Poor typical pathological findings of CSN PNET were shown. Pathological diagnosis was embryonal tumor. Further investigation including markers of germ cell tumor was recommended

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This case is difficult diagnosis on MRI and morphology. MRI shows the extrinsic tumor in the cerebellopontine angle and light microscopy seems to be malignant glial tumor. Large cells were stained with cytokeratin, EMA which was diagnosed with AT/RT by multistainig.INI-1 stainig showed positive, which result is interested. Another interested point is the age of adult.

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