[A Case of Radiation Necrosis in the Right Occipital Lobe Accompanied with Massive Hemorrhage:Histopathological Analysis].

Radiation necrosis with massive hemorrhage is a rare complication of radiotherapy. We report the case of a male patient who had undergone radiotherapy therapy 18 years earlier and presently underwent gamma knife radiosurgery for a metastatic brain tumor in his right occipital lobe. The patient showed aberrant behavior with left homonymous hemianopsia and a gradual deterioration of cognitive function after radiotherapy. A CT scan showed the presence of an intracerebral hematoma over the right occipital lobe with mass effect, and small spotty enhancements on the lesion when enhanced on gadolinium contrast-enhanced MRI. Intraoperative findings revealed necrosis of the occipital surface and a hematoma in the occipital lobe. Pathological findings showed damage to the walls of the sinusoidal capillaries and vitreous degeneration of the inner membrane with a spongiform hemangioma. After surgery, the cerebral edema resolved, and the patient's clinical symptoms improved. The cause of the radiation necrosis and bleeding in this patient was assumed to be due to the breakdown of the congested walls of the sinusoidal capillary vessels.

[Calvarial Intraosseous Schwannoma of the Frontoparietal Bone: A Case Report].

Calvarial intraosseous schwannoma is very rare bone tumor. Herein we report the case of a 24-year-old woman with an intraosseous schwannoma of the right frontoparietal bone. The patient had a minor head trauma caused by a traffic accident. The patient was examined by a brain computed tomography (CT) and accidentally found a calvarial bone tumor. On local examination, there was no scalp swelling and tenderness. Magnetic resonance imaging (MRI) showed isointensity of the tumor within the brain cortex on T1-weighted image (WI) and high intensity on T2-WI. On the diffusion-weighted images, the isointensity of the tumor was again depicted. The mass was strongly enhanced by gadolinium administration. Subsequently, the tumor was totally removed and was yellowish, well demarcated, and situated in the diploë intraoperatively. The inner table was widely erosive and there was a mild adhesion between the mass and the dura mater. The pathological diagnosis was an intraosseous schwannoma. We reviewed previous reports and discussed.

[Surgical Outcome of Acute and Subacute Subdural Hematoma with Endoscopic Surgery].

Acute subdural hematoma (ASDH) and subacute subdural hematoma(SASDH)evacuations are commonly performed through a large craniotomy or with external decompression surgery to avoid secondary brain injury. In the field of head trauma, minimally invasive surgeries performed with neuroendoscopy were recently reported. We report 12 patients with ASDH( n=9) and SASDH (n=3)w ho underwent endoscopic hematoma evacuation via a small craniotomy between November 2013 and May 2015. All patients were over 65 years of age(mean age, 78.8 years[range, 65-91 years]) and had subdural hematomas without extensive contusion. The mean preoperative Glasgow Coma Scale(GCS)score was 8.75 (range, 4-13). In three patients, we observed the bleeding point and substantially coagulated it. Decompression in all patients was adequate after surgery. Patients with a preoperative GCS score of 4-6 showed poor outcomes, whereas those with a score >9 showed relatively good outcomes. We performed the operations safely in patients who were on antithrombotic therapy or had a systemic bleeding tendency pre-injury. Endoscopic hematoma evacuation via a small craniotomy is a safe and minimally invasive procedure in patients older than 65 years with comorbidities.

[A case of ganglioglioma extended to the lateral ventricle and associated with neurofibromatosis type 1].

We encountered a rare case of intraventricular ganglioglioma associated with neurofibromatosis type 1. A 42-year-old woman presented with a feeling of heaviness of the head and dizziness. She was diagnosed with neurofibromatosis type 1 because she had multiple subcutaneous neurofibromas and café au lait spots. On admission, she deteriorated slightly(Japan Coma Scale 1)and suffered from cognitive dysfunction and right hemiparesis. A computed tomography(CT)scan showed that she had an obstructed hydrocephalus with a long and circular mass lesion, 2cm in diameter, in the anterior horn of the left lateral ventricle. The mass showed low signal intensity(SI)on the T1-weighted image(WI), heterogeneous high SI on the T2-WI, and dense enhancement on a Gd-DTPA contrast MRI, extending from the head of the left caudate nucleus to the lateral ventricle. The patient underwent an urgent operation via an anterior transcallosal approach because of an obstructed hydrocephalus. The tumor was removed in its entirety, including its origin at the caudate head. The pathological diagnosis was a ganglioglioma grade 1 according with the classification of the World Health Organization. Here we describe this case and discuss the rare association between gangliogliomas and neurofibromatosis type 1.

Carotid Endarterectomy for Pseudo-occlusion of the Cervical Internal Carotid Artery.

OBJECT: This study described clinicopathological characteristics of pseudo-occlusion (PO) of the internal carotid artery (ICA) with regards to the pathological mechanism and the benefit of carotid endarterectomy (CEA). METHODS: We retrospectively reviewed 17 PO patients who underwent CEA. Clinical presentation, angiographic findings, surgical outcomes and plaque components obtained from CEA were investigated. RESULTS: PO plaques had more fibrous and two different pathological features, including total occlusion with recanalization and severe stenosis. Plaques of the total occlusion with recanalization (8 patients) were composed of thrombotic total occlusion and lumen recanalization by large neovascular channels, whereas those with severe stenosis (9 patients) were fibrous or fibroatheromatous plaque with severe stenosis of the original lumen. Of all the patients who underwent a carotid angiogram 2 weeks after surgery, 16 were successfully treated, but one showed complete occlusion of the ICA. At the follow-up period, two patients showed restenosis of the ICA. Three patients with complete occlusion or restenosis had histologically fibrous sclerotic plaques. CONCLUSION: Patients with PO had more fibrous plaques and two different histological features, including total occlusion with recanalization or severe stenosis. The plaque histology may be related to the pathogenesis and the surgical outcome.

Ruptured distal anterior inferior cerebellar artery aneurysm years after stereotactic radiosurgery for vestibular schwannoma: A case report and literature review.

Background: Aneurysmal formation after stereotactic radiosurgery (SRS) for vestibular schwannoma (VS) is a rare complication. Its characteristics and the best treatment strategies remain controversial, and the clinical course is especially unknown because reported aneurysms are first incidentally detected, or aneurysmal rupture occurs suddenly, and they are treated immediately. Case Description: A 68-year-old man who underwent SRS for VS 20 years ago presented with subarachnoid hemorrhage (SAH) due to rupture of a radiation-induced fusiform anterior inferior cerebellar artery aneurysm. He was treated with parent artery occlusion, resulting in a modified Rankin scale grade 2. This report illustrates the first case of detected aneurysm formation before rupture with retrospective magnetic resonance imaging evaluation. Conclusion: We describe the possible risk of rapid progression and rupture of aneurysms, focusing on the interval from SRS to aneurysmal formation. The period of formation of SRS-induced aneurysms is suspected to vary from years to decades regardless of radiation doses; however, aneurysms estimated as pseudoaneurysms have an extremely high risk of rupture within a few years, even when small in size. If aneurysms are discovered unruptured, there are some advantages in not only the prevention of poor prognosis due to SAH but also in the availability of optional therapeutic strategies using revascularization. Long-term annual follow-up, including vessel examination, is warranted not only to assess tumor status but also for early detection of any vascular lesions.

Clinicopathological characteristics in patients presenting with acute onset of symptoms caused by Rathke's cleft cysts.

BACKGROUND: Symptomatic Rathke's cleft cyst is usually accompanied by a long history of headache, visual disturbance, and hypopituitarism; however, rare cases present with acute onset and the clinical features in such cases remain uncertain. We report herein the clinical features of Rathke's cleft cyst with acute onset and discuss the clinical significance. METHOD: In this study, we defined acute onset as the clinical course with clinical symptoms within a 7-day history. From among 35 cases of symptomatic Rathke's cleft cyst that were pathologically diagnosed at Fukuoka University Hospital between 1990 and 2009, five cases presented with acute onset. The symptoms, endocrinological findings, MR image findings, and pathological findings of these cases were analyzed retrospectively. FINDINGS: Mean age was 56.8 years. Initial symptoms included headache (n = 3), general malaise (n = 2), polyuria (n = 2), and fever (n = 1). MR imaging revealed an intrasellar cystic lesion with suprasellar extension in all cases and showed rim enhancement in three cases. All cases were treated by transsphenoidal surgery. Pathological findings included hemorrhage (n = 2), hypophysitis (n = 2), and abscess formation in the cyst (n = 1). Postoperatively, all symptoms, except for hypopituitarism, improved in all cases. CONCLUSIONS: Rathke's cleft cysts sometimes present with acute onset, and the presentation is consistent with the features of pituitary apoplexy caused by pituitary adenoma. Although pituitary apoplexy due to hemorrhage, inflammation, or infection due to an underlying Rathke's cleft cyst is difficult to diagnose pre-operatively, Rathke's cleft cyst should be included in the differential diagnosis, and early surgical treatment is needed, as for pituitary apoplexy caused by pituitary adenoma.

[A case of intramedullary cervical metastasis from cerebellar anaplastic oligodendroglioma without typical MR appearance for CSF dissemination].

We report a case of intramedullary cervical metastasis from cerebellar anaplastic oligodendroglioma without any MR appearance for CSF dissemination or recurrence in the primary tumor site. The case was a 73-year-old female who underwent tumor resection for cerebellar anaplastic oligodendroglioma following irradiation and TMZ chemotherapy. One year and a half later, a small nodular lesion developed at the temporal lobe. While treatment with TMZ was restarted during the course, another intramedullary cervical lesion produced additionally without any CSF dissemination or recurrence at the primary site. The histological examination obtained in spinal surgery revealed evidence of similar features consistent with a previous cerebellar tumor with anaplastic oligodendroglioma. Although CSF dissemination by malignant glioma with leptomeningeal enhancement is relatively well recognized at their terminal stage, cases with intramedullary cervical metastasis without any leptomeningeal enhancement have only been rarely described. We discussed the mechanisms of intramedullary cervical metastasis from intracranial malignant glioma.

Efficacy of temozolomide treatment in patients with high-grade glioma.

BACKGROUND: Numerous studies have reported the clinical efficacy of temozolomide (TMZ) treatment for high-grade glioma, but information on Japanese populations has been limited. This study assessed the safety and early outcomes of TMZ treatment, with or without combination therapy. PATIENTS AND METHODS: The subjects comprised ten patients with high-grade glioma [glioblastoma multiforme (GBM), n=3, gliosarcoma (GS), n=1, anaplastic oligodendroglioma (AO), n=3, anaplastic mixed oligoastrocytoma (AOA), n=1, and anaplastic ependymoma (AE), n=2]. All the patients were initially treated with conventional radiotherapy following surgical resection with or without adjuvant chemotherapy. As second- or third-line chemotherapy, patients received TMZ for recurrence or tumor progression. As combination therapy, the local administration of tumor necrosis factor-alpha and the addition of carboplatin and etoposide were included for three patients during the course of oral TMZ treatment. RESULTS: Partial response (PR) to TMZ therapy was achieved by four out of the ten patients (objective response rate, 40%), while three patients displayed stable disease (SD) and three showed disease progression (PD). One of the patients receiving combination therapy has continued to show shrinkage of the relapsed tumor. Despite prior radio- and chemotherapy, most patients experienced only grade 1-2 hematotoxicity that was well-controlled by conservative therapy. CONCLUSION: TMZ chemotherapy is effective for the treatment of high-grade glioma in some patients without serious toxicity. Assessing the true efficacy of TMZ will require a larger study with comparison of long-term outcomes between other agents or combined therapeutic modalities.

Management of pilomyxoid astrocytomas: our experience.

BACKGROUND: Pilomyxoid astrocytoma (PMA) shows a higher rate of recurrence and cerebrospinal fluid (CSF) dissemination than does pilocytic astrocytoma (PA). In this article, we discuss the treatment of PMA. MATERIALS AND METHODS: Between 1992 and 2007, the authors treated 5 patients. Two of these were male, three female. Their ages ranged from 3 months to 11 years. RESULTS: Three patients showed CSF dissemination on the initial radiographic examination. All patients received chemotherapy; the most commonly used combination drugs were cisplatin (CDDP)/carboplatin (CBDCA) and etoposide. When these drugs were unsuccessful, they were changed or other drugs added to the combination. After chemotherapy, four patients showed remarkable tumor regression. Nevertheless, one patient died 22 months after initial diagnosis, due to tumor progression. CONCLUSION: While our series was limited to a small number of patients, we have a positive impression of the value of chemotherapy. Even if initial chemotherapy is ineffective, we recommend continued CDDP/CBDCA-based chemotherapy with new drug combinations.

Survival and late effects on development of patients with infantile brain tumor.

BACKGROUND: Most infants with brain tumor may have a poor prognosis. The aim of the present study was to retrospectively analyze the survival and outcome with regard to mental and physical development in 11 subjects with brain tumor; these tumors were diagnosed when the patients were under 1 year of age. METHODS: The histological diagnoses of these tumors were astrocytoma, n = 3; pineocytoma, n = 2; teratoma, n = 1; ependymoma, n = 1; atypical teratoid/rhabdoid tumor, n = 1; glioblastoma, n = 1; medulloblastoma, n = 1; and choroid plexus papilloma, n = 1. Surgical resection was performed in eight patients, and adjuvant chemotherapy was administered to all except one patient with choroid plexus papilloma. Radiotherapy was additionally performed for four of the 10 chemotherapy patients. RESULTS: Six patients survived. Among the surviving patients, five were under no treatment for 50-167 months after the diagnosis (median duration, 89 months), while one received chemotherapy for 20 months. Five patients exhibited mental retardation, and one patient experienced normal development after surgical removal of his choroid plexus papilloma. Diencephalic syndrome developed in one patient with pilomyxoid astrocytoma that necessitated hormone replacement therapy, and bodyweight over +2 SD was observed in two patients. The remaining five patients died 11-111 months after diagnosis (median duration, 24 months). CONCLUSION: The prognosis of infantile brain tumor with regard to mortality and developmental outcome remains poor. Furthermore, survivors require comprehensive medical and social support for an extended period.

Metastatic adenocarcinoma in the brain: magnetic resonance imaging with pathological correlations to mucin content.

BACKGROUND: Hypointense signal appearance of metastatic adenocarcinoma on T2-weighted imaging (T2-WI) has been infrequently documented. The purpose of this report was to evaluate the degree to which mucin content affects signal manifestations on conventional MR imaging. PATIENTS AND METHODS: This series of 24 cases with intracerebral metastatic adenocarcinoma was assessed retrospectively, focusing on the association between hypointense appearance on T2-WI and intratumoral mucin content. RESULTS: Among the 24 metastatic adenocarcinomas, intratumoral mucin was histopathologically confirmed in 8 lesions. Of these, 4 masses were demonstrated as hyperintense signal on T2-WI. The other 4 masses were depicted as isointensity. No cases were identified with hypointense signals in mucin-containing metastatic adenocarcinoma. Conversely, only 2 metastatic tumors originating from the stomach exhibited hypointense signal lesions on T2-WI. No histological or magnetic resonance imaging evidence of blood products or other forms of iron were identified as causes of the hypointense appearance on T2-WI. CONCLUSION: This hypointense signal may simply reflect the relatively shorter T2 relaxation time of the primary tissue from which metastases arose. Intratumoral mucin itself may be considered to demonstrate the masses as hyper- or isointense signals in the brain.

Congenital medulloblastoma with atypical MRI appearance.

A 13-day-old female infant was admitted with hydrocephalus that had been diagnosed on prenatal ultrasound at 33 weeks' gestation. She was delivered by Caesarean section at 34 weeks with an Apgar score of 10. On admission, she weighed 2,103 g. The head circumference was 32.3 cm, and the fontanelle was tense. T(1)- and T(2)-weighted MR images revealed an isointense mass occupying the fourth ventricle with multiple cysts in the vermis. The mass was not enhanced after gadolinium administration. CT showed no definite calcification in the lesion. Preoperatively, vermian tumors, including medulloblastoma, ependymoma, astrocytoma, and hamartomas, were considered in the differential diagnosis. Hamartoma was strongly suspected due to the lack of enhancement on MRI. After a suboccipital midline craniotomy, subtotal resection of a soft grayish tumor with areas of hematoma was carried out. The pathological diagnosis was medulloblastoma. Despite chemotherapy, CSF dissemination resulted in death at 11 months. We report this case of congenital medulloblastoma with atypical MRI findings and discuss the clinical characteristics of this lesion.

Complication caused by use of fibrin glue in vessel transposition for trigeminal neuralgia.

A 64-year-old man underwent microvascular decompression of the left superior cerebellar artery (SCA) for left trigeminal neuralgia (TN) using a sling of Teflon tape fixed to the tentorium with fibrin glue. The TN disappeared immediately after surgery, but recurred unusually rapidly at 2 weeks later at the same intensity as before. Second surgery revealed the SCA was suspended from the tentorium, but the trigeminal nerve was stretched and displaced superolaterally because of adhesion to the superior petrosal vein. The adhesion was thought to involve the fibrin glue used during the sling retraction procedure. The nerve was meticulously dissected from the adhesion, and the trigeminal nerve was placed in the correct position. The postoperative course was uneventful, and the TN disappeared completely. We recommend that the smallest amount of the fibrin glue possible be used to avoid adhesion to the surrounding neurovascular elements.

[Case of idiopathic hypertrophic pachymeningitis presenting with hyperostosis].

We report a case of idiopathic hypertrophic pachymeningitis presenting with cranial hyperostosis. A 64-year-old man had suffered from pulsating headache during the last 3 months. CT showed bony thickening of the sphenoid ridge, and MRI with Gd-DTPA revealed a linear or nodular enhanced mass along the left sphenoid ridge, extending to the anterior and middle cranial fossae and cavernous sinus. Preoperative steroid therapy resulted in improved clinical symptoms and shrinkage of the enhanced lesion. To clarify the nature of the lesion, biopsy was performed. Granulation tissue infiltrated by lymphocytes and plasma cells was identified, suggesting inflammatory changes. Histologic examination of the cranial bone showed fibrosis in the bone marrow. We considered the hyperostosis to have resulted from a long-term nonspecific inflammatory reaction. Idiopathic hypertrophic pachymeningitis associated with skull changes is rare. There are only 4 reported cases including ours. This rare condition is important in the differential diagnosis of cranial hyperostosis.

Quantitative assessment of gliomas by proton magnetic resonance spectroscopy.

BACKGROUND: Advanced magnetic resonance (MR) techniques provide physiological and metabolic information that complements the anatomical information available from conventional MR imaging. The purpose of this study was to evaluate the clinical usefulness of proton MR spectroscopy (1H-MRS) in preoperative quantitative assessment of intracranial gliomas. PATIENTS AND METHODS: Eight patients with histologically verified gliomas, comprising 2 cases with glioblastoma multiforme (GBM, grade 4), 5 cases with anaplastic oligodendroglioma (AO, grade 3; high-grade glioma), and 1 case with fibrillary astrocytoma (FA, grade 2; low-grade glioma) were evaluated using the 1H-MRS protocol following conventional MR imaging, diffusion-weighted imaging (DWI), and perfusion-weighted imaging (PWI) preoperatively. RESULTS: High-grade gliomas tended to demonstrate signal hyperintensity by DWI and higher relative cerebral blood volume (rCBV) by PWI. Increased ratios of choline (Cho) to N-acetylaspartate (NAA) (Cho/NAA) and Cho to creatine (Cr) (Cho/Cr) correlated highly with tumor malignancy. The presence of lactate and lipid was predominately detected in patients with high-grade glioma. CONCLUSION: The combination of multiple MR parameters, based on DWI, PWI and 1H-MRS, appears valuable for preoperatively predicting the degree of malignancy in glioma.

Ruptured aneurysm of the distal anterior cerebral artery: clinical features and surgical strategies.

Ruptured aneurysms of the distal anterior cerebral artery (ACA) are relatively rare and surgical management provides some unique technical challenges. This retrospective analysis of 20 patients with distal ACA aneurysms evaluated the clinical features and surgical strategies. The characteristic findings were small and common concurrent aneurysms, and frequent intracerebral hematoma (ICH). Aneurysms were divided by location on the genu (n = 13), infracallosal (n = 5), and supracallosal portions (n = 2). All patients except one underwent surgery via an interhemispheric route. Unilateral craniotomy was performed for aneurysms on the genu portions without massive ICH. Bilateral craniotomies were selected for aneurysm located on the infracallosal portion or combined with massive ICH. No intraoperative rupture was observed. Favorable outcomes were achieved in 15 of 20 patients, and only one patient died. The preoperative Hunt and Kosnik grade was closely correlated with the outcome.

[Ruptured aneurysm of distal posterior inferior cerebellar artery located at caudal loop].

We report an unusual case of subarachnoid hemorrhage (SAH) due to ruptured aneurysm originating at the caudal loop of posterior inferior cerebellar artery (PICA). The patient was a 77-year-old female presented with a sudden onset of headache and vomiting. Initial CT scan demonstrated a SAH with thick hematoma mainly in the cisterna magna. Vertebral angiogram revealed a saccular aneurysm arising from the tonsillomedullary segment of the left PICA, and communicating artery with supplying a territory of contralateral vermis as an anastmotic vessel. At surgery, no vessel branches were confirmed in the vicinity of the aneurysm, and this aneurysm was successfully clipped. In the fetus, numerous basilar and vertebral arteries are organized in plexiform formations around the brain stem. It has been suggested that the pathogenesis of such aneurysm or communicating artery could be related with a remnant of a primitive vertebrobasilar anastomosis. Based on these considerations, congenital vessel-wall weakness and hemodynamic stress associated with communicating artery may contribute to the development of distal PICA aneurysms.

Anti-tumor activity of de novo designed small globular protein (SGP) in vivo.

BACKGROUND: The design and synthesis of a small globular protein (SGP) based on the colicin family of bacteriocins has previously been reported. It has subsequently been shown that this artificial protein possessed cell membrane-disrupting properties. Here, the anti-tumor effects of SGP in subcutaneous nude mice tumor models of human glioma were examined. MATERIALS AND METHODS: Human glioblastoma cells (U-87MG) were transplanted subcutaneously into the backs of nude mice. When tumor volume was between 100 mm(3) and 350 mm(3), tumors were locally injected with SGP three times a week. The four SGP injection groups were 0.5 mM/50 microl, 0.5 mM/100 microl, 1.0 mM/50 microl and 1.0 mM/100 microl (concentration/injection volume). The control group was injected with physiological saline; 100 microl three times a week. RESULTS: Tumor volume exhibited a statistically significant decrease (p=0.001) and survival was significantly longer in the SGP-injected groups (p=0.0001). No clear difference in survival time was observed between the 0.5 mM SGP and 1.0 mM SGP groups (p=0.0654). However, the reduction in tumor volume was significantly affected by SGP concentration (p=0.021). CONCLUSION: SGP has an anti-tumor effect in human glioma animal models. The present results also suggest that applied treatment based on local injection of SGP will be effective against deep brain-seated malignant glioma.

Mechanisms of small globular protein-induced plasma membrane permeability and cytotoxicity in U87-MG human malignant glioblastoma cells.

BACKGROUND: Small globular protein (SGP) is an artificially designed protein that forms small pores on lipid bilayer membranes. The anti-tumor effect of SGP has been reported in several papers. However, the exact mechanism underlying SGP-induced cell death remains to be determined. MATERIALS AND METHODS: The mechanisms of SGP-mediated cytotoxicity were examined using cultured malignant glioblastoma cells (U87-MG). Morphological changes were observed under a light microscope. Ultrastructural changes were examined by electron microscopy. Changes in membrane permeability were evaluated by measuring the extracellular release of lactate dehydrogenase (LDH) and intracellular calcium concentration ([Ca2+]i). A new cell viability assay was also conducted using DAPI/calcein fluorescent-double staining methods. RESULTS: SGP cytotoxic effects were strongly influenced by fetal bovine serum (FBS) concentration in culture media. After 30 min of incubation, cytotoxic effects were noted at SGP concentrations greater than 1.5 microM. [Ca2+]i increased immediately after SGP addition. Extracellular efflux of LDH increased linearly with SGP doses (r(2) =0.936), and occurred even at concentrations less than 1.5 microM. Non-viable and viable cells were simultaneously detected by fluorescent staining with DAPI and calcein-AM, respectively. After 30 min of incubation with SGP, DAPI-positive nuclei were evident at concentrations higher than 1.5 microM. Cultures were then maintained in 10% FBS-containing media for 24 h before addition of calcein-AM. Calcein-positive cells were found at SGP concentrations lower than 1.5 5M. Cells positive for both DAPI and calcein appeared at a concentration of 1.5 microM. CONCLUSION: Our results indicate that SGP induces rapid but transient cytotoxicity, which may be beneficial in local therapy of malignant brain tumors.