Language：Japanese   Publisher：新潟医学会  

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Language：Japanese   Publisher：Japan Society for Laser Surgery and Medicine  

We assessed demographics, progression free survival (PFS), overall survival (OS), and adverse effects of photodynamic therapy (PDT) for malignant brain tumors at the Department of Neurosurgery, Niigata University from July 2018 to June 2021. Median PFS was 14 months and median OS was not reached in 9 newly-diagnosed glioblastoma patients undergoing PDT during initial surgery. Death was observed in only one patient who showed early, distant recurrence. Main adverse effects, all transient, included photosensitivity in 1 case, reversible FLAIR hyperintensity in 3 cases, and depressive state in 5 cases. Depressive state due to long light shielding times were observed and effort to reduce shielding times should be made. We share our initial PDT experience by introducing specific cases, and also our preclinical experiments for near-infrared photoimmunotherapy (NIR-PIT), which we consider to be the next-generation, intraoperative treatment for malignant brain tumors. NIR-PIT targets cancer cell-specific surface antigens, and treating with near-infrared light destroys the cell membrane of tumor cells. We succeeded in treating glioblastoma cell lines by NIR-PIT, using an antibody against the specific surface antigen X.

DOI： 10.2530/jslsm.jslsm-44_0024

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Other Link： https://search.jamas.or.jp/default/link?pub_year=2023&ichushi_jid=J01213&link_issn=&doc_id=20230803380003&doc_link_id=10.2530%2Fjslsm.jslsm-44_0024&url=https%3A%2F%2Fdoi.org%2F10.2530%2Fjslsm.jslsm-44_0024&type=J-STAGE&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00007_2.gif

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Cerebellopontine angle (CPA) lipoma-associated hemifacial spasm (HFS) is rare. As the removal of CPA lipomas has a high risk of worsening the neurological symptoms, surgical exploration is warranted only in selected patients. Preoperative identification of the lipoma affected site of the facial nerve, and offending artery are crucial for patient selection and successful microvascular decompression (MVD). OBSERVATIONS: Presurgical simulation using three-dimensional (3D) multifusion imaging showed a tiny CPA lipoma wedged between the facial and auditory nerves, as well as an affected facial nerve by the anterior inferior cerebellar artery (AICA) at the cisternal segment. Although a recurrent perforating artery from the AICA anchored the AICA to the lipoma, successful MVD was achieved without lipoma removal. LESSONS: The presurgical simulation using 3D multifusion imaging could identify the CPA lipoma, affected site of the facial nerve, and offending artery. It was helpful for patient selection and successful MVD.

DOI： 10.3171/CASE2318

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Neurenteric cyst (NC) shows benign histopathology and rarely demonstrate malignant transformation. We herein describe a case of NC that exhibited malignant transformation. A 65-year-old female presented with gait disturbance due to compression by a cystic mass on the dorsal surface of the medulla oblongata. Partial resection was performed twice, leading to improvement of her symptoms. Two years after the second surgery, gadolinium-perfused T1-weighted magnetic resonance imaging revealed an invasive lesion with contrast enhancement at the trigone of the left lateral ventricle for which partial resection followed by radiotherapy was performed. However, mass regrowth was observed, with the patient eventually succumbing to her disease 11 months after her third surgery. Histopathological analyses of the first and second surgical specimens identified pseudostratified cuboidal epithelial cells, with no nuclear or cellular atypia resembling gastrointestinal mucosa, lining the inner surface of the cystic wall. Based on these findings the lesion was diagnosed as NC. The third surgical specimen exhibited apparent malignant features of the epithelial cells with elongated and hyperchromatic nuclei, several mitotic figures, small necrotic foci, and a patternless or sheet-like arrangement. Based on these findings, the lesion was diagnosed as NC with malignant transformation. Next-generation sequencing revealed KRAS p.G12D mutation in all specimens. Additionally, the third surgical specimen harbored the following 12 de novo gene alterations: ARID1A loss, BAP1 p.F170L, CDKN1B loss, CDKN2A loss, CDKN2B loss, FLCN loss, PTCH1 loss, PTEN loss, PTPRD loss, SUFU loss, TP53 loss, and TSC1 loss. The aforementioned results suggest that KRAS mutation is associated with the development of the NC, and that the additional gene alterations contribute to malignant transformation of the NC.

DOI： 10.1111/neup.12822

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Language：Japanese   Publisher：日本脳腫瘍病理学会  

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Multimodal therapy including surgery, radiation treatment, and temozolomide (TMZ) is performed on glioblastoma (GBM). However, the prognosis is still poor and there is an urgent need to develop effective treatments to improve survival. Molecular biological analysis was conducted to examine the signal activation patterns in GBM specimens and remains an open problem. Advanced macrolides, such as azithromycin, reduce the phosphorylation of p70 ribosomal protein S6 kinase (p70S6K), a downstream mammalian target of rapamycin (mTOR) effector, and suppress the proliferation of T-cells. We focused on its unique profile and screened for the antitumor activity of approved macrolide antibiotics. Clindamycin (CLD) reduced the viability of GBM cells in vitro. We assessed the effects of the candidate macrolide on the mTOR pathway through Western blotting. CLD attenuated p70S6K phosphorylation in a dose-dependent manner. These effects on GBM cells were enhanced by co-treatment with TMZ. Furthermore, CLD inhibited the expression of the O6-methylguanine-DNA methyltransferase (MGMT) protein in cultured cells. In the mouse xenograft model, CLD and TMZ co-administration significantly suppressed the tumor growth and markedly decreased the number of Ki-67 (clone MIB-1)-positive cells within the tumor. These results suggest that CLD suppressed GBM cell growth by inhibiting mTOR signaling. Moreover, CLD and TMZ showed promising synergistic antitumor activity.

DOI： 10.3390/cancers14030770

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We have previously published a study on the reliable detection of 2-hydroxyglutarate (2HG) in lower-grade gliomas by magnetic resonance spectroscopy (MRS). In this short article, we re-evaluated five glioma cases originally assessed as isocitrate dehydrogenase (IDH) wildtype, which showed a high accumulation of 2HG, and were thought to be false-positives. A new primer was used for the detection of IDH2 mutation by Sanger sequencing. Adequate tissue for DNA analysis was available in 4 out of 5 cases. We found rare IDH2 mutations in two cases, with IDH2 R172W mutation in one case and IDH2 R172K mutation in another case. Both cases had very small mutant peaks, suggesting that the tumor volume was low in the tumor samples. Thus, the specificity of MRS for detecting IDH1/2 mutations was higher (81.3%) than that originally reported (72.2%). The detection of 2HG by MRS can aid in the diagnosis of rare, non-IDH1-R132H IDH1 and IDH2 mutations in gliomas.

DOI： 10.3390/diagnostics11112129

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Language：Japanese   Publisher：日本脳腫瘍病理学会  

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Language：Japanese   Publisher：日本脳腫瘍病理学会  

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Language：Japanese   Publisher：(NPO)日本脳神経血管内治療学会  

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Language：Japanese  

We experienced a case of an accidental infantile acute subdural hematoma caused by household minor head trauma(Nakamura type I intracranial hemorrhage)with postoperative hemispheric hypodensity lesion(Big Black Brain)whose pathophysiology was analyzed using perfusion MRI. A ten-month-old boy was admitted to our hospital in a comatose state. His mother revealed that the boy suffered a fall from a sofa bed. A CT scan indicated massive acute subdural hematoma in the left cerebral hemisphere. Emergency craniotomy and hematoma evacuation were performed. On postoperative day 3, CT revealed hemispheric hypodensity, and the boy suffered from status epilepticus. MRI on the following day showed widespread white matter hyperintensity in diffusion-weighted images, and MRA demonstrated dilation of the middle cerebral artery. Perfusion MRI using the dynamic susceptibility contrast method revealed a marked increase in cerebral blood flow in the left hemisphere. These abnormal MRI and MRA findings disappeared on postoperative day 13. Status epilepticus also improved upon administration of multi-antiepileptic drugs. Fundoscopy findings on postoperative day 3 showed small bilateral petechial or brush retinal hemorrhages. However, whole-body examination did not show any problems, and was consistent with the mother's account. Thus, we judged non-abusive head trauma. Although follow-up MRI showed diffuse atrophy of the left cerebral hemisphere, the boy aged well without obvious paresis or verbal developmental delay as judged by a follow-up more than a year later. Based on these results, we speculated that hyperperfusion caused by dilation of the cerebral artery was related to the postoperative hemispheric hypodensity, namely "Big Black Brain".

DOI： 10.11477/mf.1436204281

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Authorship：Lead author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：The Japanese Society for Neuroendovascular Therapy  

OBJECTIVE: For curative Onyx embolization of dural arteriovenous fistulas (dAVF) with multiple feeders, it is essential to select the optimal target artery as well as to control the blood flow at the fistula point. We report a case of tentorial dAVF (TdAVF) treated by Onyx embolization under flow control using balloon catheters. CASE PRESENTATION: A 66-year-old male was admitted to our hospital for treatment of TdAVF detected incidentally by MRI, which revealed a dilated and tortuous vein around the cerebellum. Cerebral angiography demonstrated a TdAVF, fed mainly by bilateral middle meningeal arteries (MMA) and bilateral occipital arteries (OA), with the fistula point at the torcular and venous drainage to the two superior vermian veins (SVVs). Onyx 18 was injected from the low-flow feeder of the MMA under flow control by occluding the high-flow feeder of the OA using balloon catheters, obliterating the arteriovenous shunt. CONCLUSION: In treatment of TdAVF involving low- and high-flow feeders, Onyx embolization via the low-flow feeder with temporary balloon occlusion of other high-flow feeders is a useful method. This technique makes it easier for Onyx to penetrate the fistula point.

DOI： 10.5797/jnet.tn.2019-0053

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Language：Japanese   Publisher：(NPO)日本脳神経血管内治療学会  

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We have previously reported that reliable detection of 2-hydroxyglutarate (2HG) in isocitrate dehydrogenase (IDH)-mutant WHO grade 2 and 3 gliomas is possible utilizing 3.0-T single-voxel magnetic resonance spectroscopy (SVMRS). We set out to determine whether the same method could be applied to detect 2HG in IDH-mutant glioblastoma. Forty-four patients harboring glioblastoma underwent pre-operative MRS evaluation to detect 2HG and other metabolites. Presence of IDH-mutations was determined by IDH1 R132H immunohistochemical analysis and DNA sequencing of surgically obtained tissues. Six out of 44 (13.6%) glioblastomas were IDH-mutant. IDH-mutant glioblastoma exhibited significantly higher accumulation of 2HG (median 3.191 vs. 0.000 mM, p < 0.0001, Mann-Whitney test). A cutoff of 2HG = 0.897 mM achieved high sensitivity (100.0%) and specificity (92.59%) in determining IDH-mutation in glioblastoma. Glioblastoma with high 2HG accumulation did not have significantly longer overall survival than glioblastoma with low 2HG accumulation (p = 0.107, log-rank test). Non-invasive and reliable detection of 2HG in IDH-mutant glioblastoma was possible by 3.0-T SVMRS.

DOI： 10.1007/s10143-017-0908-y

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Cerebral venous sinus thrombosis (CVST) is an uncommon form of stroke, and mortality of the acute phase is high. We report the clinical, postmortem 3 T-MRI, and autopsy features of a patient, 20-year-old Japanese woman, with CVST who died shortly after starting to use low-dose estrogen combined hormonal contraceptives (CHCs). A postmortem 3 T-MRI study with our originally developed system revealed abnormal intensities suggestive of thrombi extending throughout the straight sinus and left sigmoid sinus. At autopsy, in accordance with the images, we performed careful preparations of the sinuses. Histological examination revealed an organizing white thrombus occupying the lumen of the left sigmoid sinus, and an acute, red thrombus in the lumen of the left transverse, straight, and tentorial sinuses, and vein of Galen, indicating that the thrombus had developed first in the left sigmoid sinus, then extended retrogradely to the more proximal portion of the sinus system, reaching the vein of Galen. The features of the present CVST patient appear to be informative, when encountering CHC users with neurological symptoms, even in those who begun to use low-dose estrogen CHCs only recently.

DOI： 10.1016/j.ehpc.2016.01.002

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：WILEY  

Glioblastoma (GBM), one of the most malignant human cancers, frequently recurs despite multimodal treatment with surgery and chemo/radiotherapies. GBM-initiating cells (GICs) are the likely cell-of-origin in recurrences, as they proliferate indefinitely, form tumors in vivo, and are resistant to chemo/radiotherapies. It is therefore crucial to find chemicals that specifically kill GICs. We established temozolomide (the standard medicine for GBM)-resistant GICs (GICRs) and used the cells for chemical screening. Here, we identified 1-(3-C-ethynyl-β-d-ribopentofuranosyl) uracil (EUrd) as a selective drug for targeting GICRs. EUrd induced the death in GICRs more effectively than their parental GICs, while it was less toxic to normal neural stem cells. We demonstrate that the cytotoxic effect of EUrd on GICRs partly depended on the increased expression of uridine-cytidine kinase-like 1 (UCKL1) and the decreased one of 5'-nucleotidase cytosolic III (NT5C3), which regulate uridine-monophosphate synthesis positively and negatively respectively. Together, these findings suggest that EUrd can be used as a new therapeutic drug for GBM with the expression of surrogate markers UCKL1 and NT5C3. Stem Cells 2016;34:2016-2025.

DOI： 10.1002/stem.2380

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Language：Japanese   Publisher：日本脳腫瘍病理学会  

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Language：Japanese   Publishing type：Research paper (bulletin of university, research institution)  

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Other Link： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2016&ichushi_jid=J00990&link_issn=&doc_id=20160803070013&doc_link_id=%2Fdg3nigta%2F2016%2Fs13003%2F005%2F0210-0211%26dl%3D0&url=http%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fdg3nigta%2F2016%2Fs13003%2F005%2F0210-0211%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

Authorship：Lead author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：WILEY-BLACKWELL  

Genetic and epigenetic status, including mutations of isocitrate dehydrogenase (IDH) and TP53 and methylation of O(6) -methylguanine-DNA methyltransferase (MGMT), are associated with the development of various types of glioma and are useful for prognostication. Here, using routinely available histology sections from 312 patients with diffuse gliomas, we performed immunohistochemistry using antibodies specific for IDH1 mutation, MGMT methylation status, and aberrant p53 expression to evaluate the possible prognostic significance of these features. With regard to overall survival (OS), univariate analysis indicated that an IDH1-positive profile in patients with glioblastoma (GBM), anaplastic astrocytoma (AA), anaplastic oligoastrocytoma and oligodendroglioma, or a MGMT-negative profile in patients with GBM and AA were significantly associated with a favorable outcome. Multivariate analysis revealed that both profiles were independent factors influencing prognosis. The OS of patients with IDH1-positive/MGMT-negative profiles was significantly longer than that of patients with negative/negative and negative/positive profiles. A p53 profile was not an independent prognostic factor. However, for GBM/AA patients with IDH1-negative/MGMT-negative profiles, p53 overexpression was significantly associated with an unfavorable outcome. Thus, the immunohistochemical profiles of IDH1 and MGMT are of considerable significance in gliomas, and a combination of IDH1, MGMT and p53 profiles may be useful for prognostication of GBM/AA.

DOI： 10.1111/neup.12196

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BACKGROUND: The supplementary motor area (SMA) makes multiple reciprocal connections to many areas of the cerebral cortices, such as the primary motor cortex (PMC), anterior cingulate cortex, and various regions in the parietal somatosensory cortex. In patients with SMA seizures, epileptic discharges from the SMA rapidly propagate to the PMC. We sought to determine whether near-infrared spectroscopy (NIRS) is able to intraoperatively display hemodynamic changes in epileptic network activities between the SMA and the PMC. CASE DESCRIPTIONS: In a 60-year-old male with SMA seizures, we intraoperatively delivered a 500 Hz, 5-train stimulation to the medial cortical surface and measured the resulting hemodynamic changes in the PMC by calculating the oxyhemoglobin (HbO2) and deoxyhemoglobin (HbR) concentration changes during stimulation. No hemodynamic changes in the lateral cortex were observed during stimulation of the medial surface corresponding to the foot motor areas. In contrast, both HbO2 and HbR increased in the lateral cortex corresponding to the hand motor areas when the seizure onset zone was stimulated. In the premotor cortex and the lateral cortex corresponding to the trunk motor areas, hemodynamic changes showed a pattern of increased HbO2 with decreased HbR. CONCLUSIONS: This is the first reported study using intraoperative NIRS to characterize the epileptic network activities between the SMA and PMC. Our intraoperative NIRS procedure may thus be useful in monitoring the activities of cortico-cortical neural pathways such as the language system.

DOI： 10.4103/2152-7806.153872

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：BIOMED CENTRAL LTD  

INTRODUCTION: Previous magnetic resonance spectroscopy (MRS) and mass spectroscopy studies have shown accumulation of 2-hydroxyglutarate (2HG) in mutant isocitrate dehydrogenase (IDH) gliomas. IDH mutation is known to be a powerful positive prognostic marker in malignant gliomas. Hence, 2HG accumulation in gliomas was assumed to be a positive prognostic factor in gliomas, but this has not yet been proven. Here, we analyzed 52 patients harboring World Health Organization (WHO) grade II and III gliomas utilizing 3.0-tesla MRS. RESULTS: Mutant IDH gliomas showed significantly higher accumulation of 2HG (median 5.077 vs. 0.000, p =0.0002, Mann-Whitney test). 2HG was detectable in all mutant IDH gliomas, whereas in 10 out of 27 (37.0%) wild-type IDH gliomas, 2HG was below the detectable range (2HG =0) (p =0.0003, chi-squared test). Screening for IDH mutation by 2HG analysis was highly sensitive (cutoff 2HG =1.489 mM, sensitivity 100.0%, specificity 72.2%). Gliomas with high 2HG accumulation had better overall survival than gliomas with low 2HG accumulation (p =0.0401, Kaplan-Meier analysis). DISCUSSION: 2HG accumulation detected by 3.0-tesla MRS not only correlates well with IDH status, but also positively correlates with survival in WHO grade II and III gliomas.

DOI： 10.1186/s40478-014-0158-y

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本神経学会  

症例は76歳女性で、10年間のアルツハイマー病の既往があり、2012年に亜急性の認知機能低下と右側不全麻痺を同時発症した。脳MRIで脳梗塞を疑う病変が認められ、抗血小板療法を開始した。その後のフォローアップMRIで微小脳出血を伴うび漫性白質病変を認めたため、今回の入院となった。深部腱反射は両側ともに活発に認められた。ミニメンタルステート検査で重度の認知機能障害を認めた。FLAIR画像では、主に左半球で皮質灰白質を一部含む皮質下白質部に高信号域を広範に認めた。意識障害および右側不全麻痺が増悪して侵害刺激に反応しなくなったため、メチルプレドニゾロンを処方したところ、徐々に意識を回復し、不全麻痺も改善した。フォローアップMRIでは、異常所見の一部が消失していた。

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：WILEY-BLACKWELL  

Gliomatosis cerebri is a rare diffuse glioma that is neither mass-forming nor necrotic, and does not disrupt existing structures. Gliomatosis occurring in the cerebellum is known as gliomatosis cerebelli, and only three such cases examined by biopsy have been reported. Here we describe the first autopsy findings of a patient who was diagnosed as having gliomatosis in the cerebellum. Neuropathological examination identified the tumor cells as being positive for glial fibrillary acidic protein, vimentin and nestin, with atypical nuclei that were cashew-nut- or dishcloth-gourd-shaped. These tumor cells were dense in the right cerebellum, but also spread broadly throughout the brain including the left cerebrum and optic nerve. Mitotic figures were frequently seen in the cerebellum, brain stem and cerebrum. Scherer's secondary structures were evident not only in the cerebellum but also the cerebrum. No necrosis, microvascular proliferation or destruction of anatomical structures was detected in the whole brain. Differences in the origin of the tumors of the gliomatoses cerbri and cerebelli suggests these tumors are different types of brain tumors. Thus the findings support that the gliomatosis cerebelli is a novel type of brain tumor classification. Furthermore, by the similarities of the histological features among the tumors, it appears appropriate to establish a novel category of "gliomatosis encephali" which includes both gliomatosis cerebri and gliomatosis cerebelli.

DOI： 10.1111/neup.12083

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Authorship：Lead author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：WILEY-BLACKWELL  

DOI： 10.1111/neup.12072

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：WILEY-BLACKWELL  

Medulloblastoma (MB) is a malignant cerebellar tumor arising in children, and its ontogenesis is regulated by Sonic Hedgehog (Shh) signaling. No data are available regarding the correlation between expression of Gli3, a protein lying downstream of Shh, and neuronal differentiation of MB cells, or the prognostic significance of these features. We re-evaluated the histopathological features of surgical specimens of MB taken from 32 patients, and defined 15 of them as MB with neuronal differentiation (ND), three as MB with both glial and neuronal differentiation (GD), and 14 as differentiation-free (DF) MB. Gli3-immunoreactivity (IR) was evident as a clear circular stain outlining the nuclei of the tumor cells. The difference in the frequency of IR between the ND+GD (94.4%) and DF (0%) groups was significant (P < 0.001). The tumor cells with ND showed IR for both Gli3 and neuronal nuclei. Ultrastructurally, Gli3-IR was observed at the nuclear membrane. The overall survival and event-free survival rates of the patients in the ND group were significantly higher than those in the other groups. The expression profile of Gli3 is of considerable significance, and the association of ND with this feature may be prognostically favorable in patients with MB.

DOI： 10.1111/neup.12052

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Malignant peripheral nerve sheath tumors (MPNSTs) arising from cranial nerves are rare and usually affect adults. Here we report the clinicopathologic features of a young adult patient with a trigeminal nerve MPNST, in whom another tumor involving the oculomotor nerve on the contralateral side was evident. The patient, an 18-year-old woman, had suffered recurrent paroxysmal sharp stabbing pain over her cheek and forehead on the right side for 1 month. A brain MRI study disclosed a mass, 35 mm in diameter, in the right Meckel's cave, and another mass, 10 mm in diameter, involving the intracranial portion of the left oculomotor nerve. Following gadolinium administration, the former and latter tumors exhibited strong and weak enhancement, respectively. The patient had no clinical stigmata characteristic of neurofibromatosis type 1. Following a tentative diagnosis of schwannoma, total resection of the trigeminal nerve tumor was performed. Histologically, the tumor consisted of highly cellular, spindle-shaped cells arranged in a fascicular pattern, with occasional mitotic figures, nuclear pleomorphism and necrosis. Immunohistochemically, the tumor cells showed variable intensities and frequencies of reactivity for S-100 protein, myelin basic protein, CD34, podoplanin and p53, but no reactivity for Smarcb1. Thus, the tumor exhibited features of MPNST. This case appears to provide information that is useful for accurate diagnosis and surgical planning in patients with bilateral or multiple cranial nerve tumors.

DOI： 10.1111/neup.12004

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Authorship：Lead author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：WILEY-BLACKWELL  

Primary central nervous system lymphoma (PCNSL) expressing T-cell markers is rare, among which nasal-type extranodal NK/T-cell lymphoma is an extremely rare subtype associated with Epstein-Barr virus (EBV) infection. Here we report the clinicopathologic features of a case of EBV-associated PCNSL showing a cytotoxic T-cell phenotype. The patient, a 73-year-old woman, presented with rapidly progressive mental deterioration. Brain MRI revealed multiple lesions with swelling in the bilateral cerebral hemispheres, which were hypointense on T1-weighted images, hyperintense on T2-weighted and fluid-attenuated inversion recovery images, and slightly hyperintense on diffusion-weighted images. Biopsy specimens from the temporal region showed many medium-sized anaplastic lymphocytic cells with perivascular and angio-invasive patterns in the cortex. Immunohistochemically, the cells were positive for CD3, CD8, T-cell-restricted intracellular antigen-1 (TIA-1), granzyme B and perforin, but negative for CD56 and CD20. In situ hybridization revealed EBV-encoded RNAs in the tumor cell nuclei. A rearrangement study showed T-cell receptor γ-chain gene rearrangement with a clonal appearance. The patient died 6 months after surgery, and a general autopsy revealed no lymphoma cells outside the brain. These cellular profiles are inconsistent with those of extranodal NK/T-cell lymphoma, and have not been previously described. This case appears to represent an unusual CNS manifestation of EBV-associated T-cell lymphoma.

DOI： 10.1111/neup.12005

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Language：Japanese   Publishing type：Research paper (scientific journal)  

OBJECTIVE: To determine whether motor evoked potentials(MEPs)provide reliable monitoring of the motor system during resection of gliomas in or adjacent to the motor cortex or pyramidal tract. MATERIALS AND METHODS: MEP recording was performed during 64 operations in 55 patients harboring gliomas. Intraoperative MEP findings were classified into 3 groups:Group A was defined as having no significant MEP changes, Group B as having reversible MEP changes(?50% amplitude decrease or loss), and Group C as having irreversible changes. Postoperative motor function was evaluated according to the presence/absence of deterioration immediately after surgery and 1 month later, as compared to preoperative motor status RESULTS: Immediately after surgery, 13 of 39(33%)patients in Group A, 6 of 17(35%)in Group B, and 7 of 8(88%)in Group C experienced deterioration of motor function. One month after surgery, 4 of 39(10%)patients in Group A, 3 of 17(18%)in Group B, and 4 of 8(50%)showed deterioration of motor function. Both immediately(χ2=8.3, p<0.05)and 1 month(χ2=6.9, p<0.05)after surgery, MEP alterations correlated significantly with postoperative deterioration of motor function. Despite MEPs being stable throughout surgery(Group A), there were some patients with deterioration of motor function initially appearing to represent false negative monitoring. However, these deteriorations were confirmed to have been caused by secondary hemorrhage, venous return dysfunction, postoperative convulsion, or resection of the supplementary motor area. CONCLUSIONS: MEP monitoring provides reliable information on the motor system during glioma surgery. Although false negative MEP results may exist in some patients, most data were not influenced by intraoperative manipulation but rather were attributable to secondary postoperative events.

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DOI： 10.11477/mf.1436101949

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The authors report a rare case involving the occurrence of metachronous pure germinomas long after treatment of a mixed germ cell tumor (GCT) categorized as having a poor prognosis. A neurohypophysial germinoma occurred 4 years and 6 months after the initial treatment of a mixed pineal GCT containing a yolk sac tumor and a germinoma. Furthermore, intramedullary germinomas occurred 21 years after the initial treatment of the mixed GCT and 15 years after the second treatment of the neurohypophysial germinoma. The neurohypophysial germinoma was not confirmed histopathologically, but the intramedullary germinoma was histopathologically diagnosed as a pure germinoma. Serum α-fetoprotein levels at the second neurohypophysial and third intramedullary occurrences of the germinomas were less than 10 ng/ml. Therefore, no yolk sac components seemed to be contained in the tumors. The second neurohypophysial and third intramedullary germinomas might be recurrences of the germinoma component of the pineal mixed GCT, which consisted of a yolk sac tumor and a germinoma. However, it seems very unlikely that only the germinoma, categorized in the good prognosis group, would be the only one to recur. Hence, it seems plausible that both the second and the third occurrences of pure germinoma were de novo metachronous GCTs arising after the pineal mixed GCT was cured. The authors' case indicates the possibility of multicentric GCTs in the CNS.

DOI： 10.3171/2012.9.PEDS12151

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The efficacy and toxicity of high-dose methotrexate (HD-MTX)-based chemotherapy were retrospectively reviewed in patients with primary central nervous system lymphoma (PCNSL). All immunocompetent patients with histologically or radiographically diagnosed PCNSL treated between 2006 and 2012 at Niigata University Hospital were enrolled. Thirty-eight patients with a diagnosis of PCNSL were treated with one of two regimens during different time periods. During the first period, from 2006 to 2009, three 3-week cycles of MPV (MTX + procarbazine + vincristine) were administered (MPV3 group). In the second period, from 2010 to 2012, five 2-week cycles of MTX were administered (MTX5 group). High-dose cytarabine was used in both groups following HD-MTX-based chemotherapy. Whole-brain radiotherapy was used for patients who did not attain a complete response (CR) based on magnetic resonance images. In the MPV3 group, 20 out of 23 patients (87%) completed the planned treatment. The CR rate after chemotherapy was 30%, and 57% after radiation therapy. Thirteen out of 15 patients (87%) in the MTX5 group completed the planned treatment. The CR rates after chemotherapy and radiation therapy were 53% and 93%, respectively. Renal dysfunction was assessed by measuring creatinine clearance rates, which were very similar in both groups. In terms of hematologic toxicity and other adverse reactions, there was no significant difference between the two groups. In conclusion, dose-dense MTX chemotherapy improved outcome with acceptable toxicity compared with the treatment schedule for three cycles of MPV treatment.

DOI： 10.2176/nmc.oa2013-0195

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Language：Japanese   Publisher：(株)メディカ出版  

症例は33歳男性で、進行性の頭痛と失語症状の増悪を主訴とした。外傷などの既往はなく、頭部MRIで左前頭葉実質内を占拠する嚢胞様腫瘤性病変を認め、腫瘤内部はT1・T2強調画像ともに低信号を呈し空気成分を疑った。腫瘤下端には前頭蓋底部に隣接して造影効果を示す小結節が存在し、CTで骨化所見も認めた。前篩骨洞上壁は菲薄化し、小結節病変が脳内への空気混入と関連している可能性があった。緊張型脳実質内気脳症の診断で左前頭開頭により結節の摘出と硬膜の修復を行った。小結節剥離後の骨面には複数の小孔を認め、硬膜再建には自家骨膜を使用し、前頭蓋底骨面にも有茎側頭筋膜弁を敷き込み小孔を完全被覆した。病理所見で結節の軟性部表面と骨化組織表面には線毛を有した重層扁平上皮および基底膜を認め、副鼻腔粘膜由来の組織と診断した。術後頭痛は速やかに消失し、術後6ヵ月の画像検査で頭蓋内に空気貯留は認めず、術後2年経過して髄液鼻漏は認めていない。

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Language：Japanese   Publisher：(一社)日本臨床神経生理学会  

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Language：Japanese   Publisher：(NPO)日本レーザー医学会  

2018年7月に当科に光線力学療法(photodynamic therapy:PDT)を導入してから2021年6月まで,当科でPDTを施行した悪性脳腫瘍症例の臨床的特徴および無増悪生存期間(progression free survival:PFS),全生存期間(overall survival:OS),主な合併症について検討した.初発膠芽腫9例におけるPFSの中央値は14ヵ月,OS中央値は未達であった.死亡例は早期に遠隔再発を来した1例のみであった.主な合併症は光過敏症が1例,脳表に可逆性のFLAIR高信号が3例,術後うつ状態が5例に認め,いずれも一過性であった.術後の長期間遮光管理が原因と思われるうつ症状には,早期遮光解除などの工夫が必要と思われた.実際の症例を提示し我々のPDT初期治療経験を紹介し,また,次世代治療と考える近赤外光線免疫療法(near-infrared photoimmunotherapy:NIR-PIT)の研究に関しても紹介する.NIR-PITは,癌細胞の表面抗原を標識とし,近赤外線照射により生じた熱エネルギーにより腫瘍細胞の細胞膜を破壊する画期的な治療法である.今回,我々は膠芽腫細胞株に特異的な表面抗原Xに対する抗体を用いたNIR-PITを行い,殺細胞効果を確認した.(著者抄録)

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Other Link： https://search.jamas.or.jp/default/link?pub_year=2023&ichushi_jid=J01213&link_issn=&doc_id=20230803380003&doc_link_id=10.2530%2Fjslsm.jslsm-44_0024&url=https%3A%2F%2Fdoi.org%2F10.2530%2Fjslsm.jslsm-44_0024&type=J-STAGE&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00007_3.gif

Language：Japanese   Publisher：信州医学会  

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Language：Japanese   Publisher：信州医学会  

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Language：Japanese   Publisher：長野赤十字病院  

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Language：Japanese   Publisher：新潟医学会  

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Other Link： http://hdl.handle.net/10191/49554

Language：Japanese   Publisher：北関東医学会  

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Other Link： https://gunma-u.repo.nii.ac.jp/records/8605

Language：Japanese   Publisher：日本脳神経CI学会  

2-ヒドロキシグルタル酸(2HG)は正常組織では殆ど検出されないが、IDH変異を有するグリオーマでは蓄積する。IDH変異を有するグリオーマ症例で、3T-single voxel Magnetic Resonance Spectroscopy(3T-SVMRS)を用いて、2HGを検出できることを報告した。その内容を、1)IDH変異と2HG、2)IDH変異を有するグリオーマの特異なbiology、3)3T-SVMRSの撮像条件、4)SVMRSによる2HGの検出、5)2HGの測定の臨床的意義、の5項目に分けて解説した。

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Language：Japanese   Publisher：(株)インナービジョン  

新潟大学脳研究所は、脳神経外科と神経内科の臨床2科、神経病理部門、そして統合脳機能研究センターなどを有している。同センターの中田力名誉教授(当時・教授/センター長)の発案により実現した、3T MRIを用いた死後画像解析システムが稼働している。われわれのポリシーは、死亡時画像診断(以下、Ai)施行後直ちに病理解剖を行い、画像情報と組織所見を統合して病態理解につなげようとするものである。Aiを剖検の代替手段として行おうとするものではない。本稿では、神経疾患を対象として当研究所が取り組んでいる死後画像解析システムと病理解剖について紹介する。(著者抄録)

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Language：Japanese   Publisher：日本脳腫瘍病理学会  

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Language：Japanese   Publisher：新潟医学会  

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Other Link： http://search.jamas.or.jp/link/ui/2015187840

Language：English   Publishing type：Research paper, summary (international conference)   Publisher：LIPPINCOTT WILLIAMS & WILKINS  

Web of Science

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Language：Japanese   Publisher：(株)インナービジョン  

脳腫瘍の画像診断は、(1)存在・局在診断(腫瘍性病変の検出と部位・進展範囲の把握)、(2)質的(腫瘍の組織型と悪性度)診断推定と鑑別診断、(3)術前情報取得、(4)術後評価、(5)治療効果判定のいずれにも大きく関与する。CTは、(1)(2)における腫瘍の石灰化の検出、(3)での頭蓋骨描出などにおいて有用であるが、MRI情報はすべての過程において重要である。本稿では、神経膠腫の(2)(3)における当施設でのMRI撮像について、症例を提示して示す。なお、3T MRI装置を用いたMRスペクトロスコピー(MRS)、arterial spin labeling(ASL)法による灌流MRI、拡散テンソル画像(DTI)、three dimensional anisotropy contrast(3D-AC)法による神経線維描出は、本学脳研究所統合脳機能研究センターの協力を得て行っている。(著者抄録)

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Other Link： http://search.jamas.or.jp/link/ui/2014333897

Language：Japanese   Publisher：新潟医学会  

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Other Link： http://search.jamas.or.jp/link/ui/2014333870

Language：Japanese   Publisher：新潟医学会  

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Other Link： http://search.jamas.or.jp/link/ui/2014230016

Language：Japanese   Publisher：新潟県医師会  

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Language：Japanese   Publisher：北関東医学会  

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Other Link： http://search.jamas.or.jp/link/ui/2014043378

Language：Japanese   Publisher：日本脳腫瘍病理学会  

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Language：Japanese   Publisher：新潟医学会  

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Other Link： http://search.jamas.or.jp/link/ui/2013333129

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Other Link： http://hdl.handle.net/10191/31528

Language：Japanese   Publisher：日本脳腫瘍病理学会  

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Language：English   Publisher：日本脳腫瘍病理学会  

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Language：Japanese   Publisher：新潟医学会  

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Other Link： http://hdl.handle.net/10191/29282

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Other Link： http://hdl.handle.net/10191/29269

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Other Link： http://hdl.handle.net/10191/29110

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Other Link： http://hdl.handle.net/10191/28909

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Other Link： http://hdl.handle.net/10191/28830

Language：English   Publishing type：Research paper, summary (international conference)   Publisher：JAPAN ENDOCRINE SOC  

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Other Link： http://hdl.handle.net/10191/28492

Language：Japanese   Publisher：社団法人日本循環器学会  

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