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.

[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.

[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.

[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.

Advanced Chemoembolization by Anti-angiogenic Calcium-Phosphate Ceramic Microspheres Targeting the Vascular Heterogeneity of Cancer Xenografts.

The purpose of the present study was to develop an advanced method of anti-angiogenic chemoembolization to target morphological vascular heterogeneity in tumors and further the therapeutic efficacy of cancer treatment. This new chemoembolization approach was designed using resorbable calcium-phosphate ceramic microspheres (CPMs), in a mixture of three different sizes, which were loaded with an anti-angiogenic agent to target the tumor vasculature in highly angiogenic solid tumors in humans in vivo. The human uterine carcinosarcoma cell line, FU-MMT-3, was used in this study because the tumor is highly aggressive and exhibits a poor response to radiotherapy and chemotherapeutic agents that are in current use. CPMs loaded with TNP-470, an anti-angiogenic agent, were injected into FU-MMT-3 xenografts in nude mice three times per week for 8 weeks. The treatment with TNP-470-loaded CPMs of three different diameters achieved a greater suppression of tumor growth in comparison to treatment with single-size TNP-470-loaded CPMs alone, and the control. Severe loss of body weight was not observed in any mice treated with any size of TNP-470-loaded CPMs. These results suggest that treatment with a mixture of differently-sized anti-angiogenic CPMs might be more effective than treatment with CPMs of a single size. This advanced chemoembolization method, which incorporated an anti-angiogenic agent to target the morphological vascular heterogeneity of tumors may contribute to effective treatment of locally advanced or recurrent solid tumors.

[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.

[Primary central nervous system lymphoma: a case report and evaluation of the diagnostic value of IL-10 level in the cerebrospinal fluid].

We report a case of primary central nervous system lymphoma (PCNSL) that presented with visual disturbance. A 76-year-old man developed decreased bilateral visual acuity. He was diagnosed with bilateral retrobulbar neuritis by an ophthalmologist. Treatment with high-dose corticosteroids was initiated and resulted in mild improvement of visual acuity. However, the patient gradually became apathetic and bradykinetic, experiencing difficulty performing the activities of daily living; he was admitted to our hospital because of this progressive illness. Neurological examination revealed bradyphrenia and bradykinesia with frontal lobe release signs, disorientation, and ideomotor apraxia. Magnetic resonance imaging showed abnormal signals in the bilateral basal ganglia and thalamus. Cerebrospinal fluid (CSF) examination revealed no pleocytosis and slightly elevated protein levels: ß2-microglobulin level was mildly increased, and IL-10 level in the CSF was markedly elevated. These findings suggested a diagnosis of PCNSL, and a brain biopsy specimen was obtained from the left caudate head. Pathological findings indicated diffuse large B-cell lymphoma. Nonspecific neurological manifestations and radiological findings can make the diagnosis of PCNSL difficult and result in delayed diagnosis. Visual impairment has been suggested as a feature of PCNSL, and an elevated IL-10 level in the CSF may be a useful marker for diagnosing PCNSL.

[Primary clivus diffuse large B cell lymphoma presenting with posterior neck pain and bilateral abducens nerve palsy].

Bilateral abducens nerve palsy is an unusual clinical presentation, which could be caused by stroke, aneurysm, trauma and malignant neoplasm. We describe here a patient with bilateral abducens nerve palsy caused by large B cell lymphoma originated from clivus. An 83-year-old woman admitted to our hospital because of diplopia and severe posterior neck pain. Her diplopia developed one month before and progressed to her admission. Neurological examination revealed bilateral abducens nerve palsy. Brain MRI with enhancement lesion in the clivus, suggesting that bilateral petroclival segment of the abducens nerves were affected by the lesion. Biopsied was performed via a transsphenoidal approach, and histological diagnosis was made as diffuse large B cell lymphoma. She received oral corticosteroid administration combined with radiation therapy. After initiation of the treatment, posterior neck pain was resolved and tumor size was reduced in the repeated brain MRI. However, diplopia and bilateral abducens nerve palsy were still unresolved. Although malignant lymphoma originated at the clivus is uncommon, according to a presenting case as well as previously reported cases, lymphoma can present as an isolated involvement in the clivus associated with headache, and bilateral abducens nerve palsy. It is suggested that the clivus tumor affected the petroclival segment of abducent nerve in our case.

A case of choroid plexus papilloma with stromal sclerosis and indistinct papillary structures.

We present a case of choroid plexus papilloma with unusual histopathological findings. A 41-year-old Japanese man presented with dizziness. Brain magnetic resonance imaging scan showed a distinct mass in the fourth ventricle. Histopathological examination showed proliferation of cuboidal cells forming irregular trabeculae, small nests, and pseudoglandular and indistinct papillary structures, accompanied by a variable degree of stromal sclerosis. The tissue sections also showed rare mitotic activity, no hypercellularity, cellular pleomorphism, or necrosis. The immunohistochemical findings were tumor cells positive for cytokeratin 7, vimentin, S-100 protein, and transthyretin but negative for cytokeratin 20, BerEP4 and carcinoembryonic antigen. These findings were consistent with choroid plexus papilloma. The combination of these immunohistochemical markers was helpful in establishing the diagnosis, although the morphological finding of stromal sclerosis is rare for choroid plexus papilloma.

Intracerebral hemorrhage despite prophylactic administration of vitamin K in infants--two case reports.

The incidence of vitamin K deficiency in infancy has decreased markedly, due to prophylactic administration of vitamin K during the neonatal period. However, vitamin K deficiency bleeding may occur during or after the neonatal period despite prophylactic administration in Japan. Two cases are reported of intracranial hemorrhage associated with coagulopathy in full-term infants who had received prophylactic administration of vitamin K. More reliable methods for prophylactic administration should be established.

Radiation-induced astrocytoma with rapid malignant transformation: case report.

A 23-year-old man was admitted with a rare case of radiation-induced astrocytoma manifesting as 3-month history of unstable gait. He had received 50 Gy of irradiation therapy for a germ cell tumor in the right basal ganglia 13 years earlier. Magnetic resonance (MR) imaging on admission showed a non-enhanced mass lesion in the right cerebellar hemisphere with expansion to the vermis. The histological diagnosis of the stereotaxic biopsy specimen was grade II astrocytoma. Two months later, he developed drowsiness, and MR imaging demonstrated that the tumor had enlarged and was enhanced after gadolinium injection. The clinical diagnosis was high-grade glioma resulting from malignant transformation. The tumor had compressed the mesencephalic aqueduct, leading to obstructive hydrocephalus. Endoscopic third ventriculostomy was performed to improve the cerebrospinal fluid circulation. He underwent chemotherapy with temozolomide postoperatively, but died 8 months after the initial diagnosis of astrocytoma. The clinical course of radiation-induced astrocytoma is not benign. The potential for malignant transformation necessitates careful postoperative follow up for patients with this tumor.

Comparison of clinical outcomes of intraventricular hematoma between neuroendoscopic removal and extraventricular drainage.

The efficacy of treatment for intraventricular hematoma by neuroendoscopic surgery and extraventricular drainage was compared in 10 patients with intraventricular hematoma and hydrocephalus who underwent neuroendoscopic surgery (endoscopic group), and eight patients with intraventricular hematoma and hydrocephalus treated with extraventricular drainage (EVD group). The outcomes in each group were assessed retrospectively using the Graeb scores on the pre- and postoperative computed tomography (CT), duration of extraventricular drainage, requirement for a shunt operation, and modified Rankin scale score at 12 months. The Graeb scores on the preoperative CT were not significantly different between the two groups, but the duration of catheter placement was significantly shorter (69.3%) in the endoscopic group (2.7 days) than in the EVD group (8.8 days). None of the patients in either group required a shunt procedure for communicating hydrocephalus 12 months after surgery. Neuroendoscopic removal is a safe and effective procedure for intraventricular hematoma. Advantages include rapid removal of hematoma in the ventricular systems and reliable improvement of non-communicating hydrocephalus in the acute phase. The procedure resulted in faster removal of the catheter in the postoperative period and earlier patient ambulation.

Intracranial ectopic pancreatic tissue.

In 2007 a young Japanese female was reported to suffer from a congenital brain malformation with a non-functioning pancreatic endocrine tumor arising from intracranial ectopic pancreatic tissue. Ectopic pancreas is normally confined to other endodermally-derived organs and not previously reported to be found in the brain. Therefore, we sought to better understand the true pancreatic nature of the tissue and to further understand the mechanism by which ectopic pancreas could appear in the brain. A detailed immunohistochemical analysis for pancreatic hormones, transcription factors, ductal/exocrine markers and stem cell markers on sections from the resected tumor tissue was performed. All five endocrine cell types are observed but pancreatic polypeptide cells are quite rare and ghrelin and glucagon cells are more numerous than in normal human pancreas. Insulin immunoreactive cells stain for c-peptide. The ß-cell specific transcription factor, Nkx6.1, is expressed only in the insulin immunoreactive cells while neither Ptf1a or PDX-1 immunoreactive cells can be observed. Duct-like structures stain strongly for pan-cytokeratin and E-cahderin. The exocrine like tissue stains strongly for pancreatic amylase, lipase and chymotrypsin. Ngn-3 cells were very rare and not in the pancreatic area. Examining for endodermal markers we observed Sox17 had a weak staining in some areas of the pancreatic tissue but was much less widely expressed than FoxA2. The tumor tissue did not stain for the stem cell markers, Oct-4 and Sox2. It is speculated that the ectopic pancreas domain may arise from misexpression of homeodomain transcription factors related to Pdx1 within a domain of Ptf1a expression.

Subependymoma with prominent rosenthal fiber formation: case report.

A 62-year-old woman presented with a rare case of subependymoma associated with prominent Rosenthal fibers located in the left lateral ventricle manifesting as right hemiparesis and mild motor aphasia. The tumor was well demarcated and consisted of clusters of round nuclei embedded in an abundant gliofibrillary matrix with some microcysts and prominent Rosenthal fibers. Immunohistochemically, the tumor stained positively for glial fibrillary acidic protein and negatively for synaptophysin. This case of subependymoma containing Rosenthal fiber formation is very unusual.

Endoscopic management of a traumatic meningo-encephalocele through a planum sphenoidale defect: case report.

A 37-year-old man involved in a motor vehicle accident was admitted to our hospital with disturbed consciousness. Computed tomography (CT) showed an acute, right-sided epidural hematoma and pneumocephalus. Emergency evacuation of the acute epidural hematoma was carried out, and his condition gradually recovered. However, cerebrospinal fluid (CSF) rhinorrhea continued despite conservative treatment. Three-dimensional reconstructed CT revealed a bone defect, which was approximately 20 mm in size, in the planum sphenoidale and tuberculum sellae, and magnetic resonance imaging demonstrated a traumatic meningo-encephalocele through the defect, with CSF collection in the sphenoid sinus. Endoscopic endonasal transsphenoidal surgery was performed. The 9-mm diameter dural defect was clearly visualized in the sphenoid sinus and was reconstructed with a combination of suturing and overlay with abdominal fascia, and absorbable polyglycolic acid felt. The CSF leakage from a traumatic meningo-encephalocele through the planum sphenoidale was successfully repaired by endoscopic endonasal surgery. Thorough preoperative evaluation of the feasibilities of the endoscopic and transcranial approaches should be based on the preoperative identification of the fistula, the bone defect, and vital structures.

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.

Primary yolk sac tumor within the lateral ventricle.

A 13-year-old girl presented with an exceedingly rare case of primary yolk sac tumor located within the lateral ventricle, manifesting as headache, nausea, and diplopia. Magnetic resonance imaging revealed a 4-cm-diameter solid enhanced mass within the left inferior horn of the lateral ventricle. The tumor was removed subtotally via left middle temporal corticotomy. The histological and immunohistochemical diagnosis was pure yolk sac tumor. The serum alpha-fetoprotein (AFP) level was elevated at 1957.2 ng/ml and the serum beta-human chorionic gonadotropin level was 4 mIU/ml after surgery. The patient underwent radiotherapy (whole brain, 30 Gy; tumor bed, 21 Gy; whole spinal axis, 30 Gy) and chemotherapy (ifosfamide, cisplatin, etoposide). After three treatment cycles, the serum AFP level had decreased to 4.5 ng/ml. However, the tumor recurred with cerebrospinal fluid dissemination after nine cycles of chemotherapy. She died 18 months after surgery. The possibility of germ cell tumor should be considered in pediatric patients with brain tumors occurring outside the pineal or suprasellar region.

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.