[Bevacizumab-Related Hyperintense Lesions on Diffusion-Weighted Imaging at Different Locations in a Patient with Malignant Glioma].

We report the case of a 60-year-old man who first presented with transient difficulty of word recall. Subsequent MRI revealed an invasive brain tumor in the left frontal lobe. The patient underwent open biopsy, and diffuse astrocytoma(WHO grade II)was diagnosed. However, the malignant potential of this tumor was not particularly low because of a few enhancement on preoperative evaluation, and radiation therapy was initially performed. Four months after ending irradiation, temozolomide treatment was introduced for tumor regrowth. After another 2 months, combined chemotherapy with bevacizumab was also started due to tumor enlargement, which was evaluated as malignant transformation to glioblastoma. Two focal lesions with signal hyperintensity on DWI appeared in the frontal and temporal lobes at different locations 3 months after starting bevacizumab. The left temporal lesion subsequently changed to a ring-enhanced tumor, and glioblastoma(WHO grade IV)was finally diagnosed at decompressive surgery. Another frontal lesion, however, continued to maintain a favorable course without any changes in signal despite appearing as similar signal-hyperintense lesions. The temporal hyperintense lesion may undergo malignant transformation into glioblastoma with typical radiological appearance. Recent studies on image changes following bevacizumab treatment have attracted widespread attention, and the clinical significance of such hyperintense lesions has gained attention. This present case was thought to be valuable because of the contradistinctive aspects at the same time, in which the hyperintense lesions of the frontal and temporal lobes seemed to represent antitumor activity or drug refractory effects based on bevacizumab treatment.

[A Case of Organizing Chronic Subdural Hematoma Treated with Endoscopic Burr-Hole Surgery Using a Curettage and Suction Technique].

A 70-year-old man presented to our hospital because of difficulty with discrete movement of the right upper limb and dysarthria. Computed tomography(CT)of the head revealed a chronic subdural hematoma(CSDH)on the left side. The patient underwent single burr-hole irrigation and drainage on the same day. In addition to the burr hole, a cross-shaped dural incision was made which revealed a thick outer membrane and solidified hematoma. We removed as much of the clotted hematoma as possible using a curved suction tube under neuroendoscopy. The postoperative CT revealed that the hematoma was partially removed and the mass effect was reduced. As a result, the patient's neurological deficits improved. We reached a diagnosis of organizing CSDH following histologic examination of the removed hematoma that showed inflammatory cell infiltration and multiplication of fibroblasts. Neuroendoscopic hematoma evacuation via a burr hole is minimally invasive and may be a useful procedure in the treatment of some cases of organizing CSDH.

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.

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.

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.

A case of a heavily pigmented orbital melanocytoma.

We present an extremely rare case of an orbital melanocytoma that occurred in a 51-year-old man. The patient suffered from diplopia and mild exophthalmos of the right eye for 2 months. Brain magnetic resonance imaging showed a well-demarcated round mass 3.5 cm in diameter in the right orbit. We performed total resection of this tumor. Histological findings revealed a proliferation of large polygonal cells with fine pigment granules in the cytoplasm and prominent nucleoli. Although these tumor cells revealed immunohistochemical reactivity in HMB-1, there was no S-100 or Melan A antibody reactivity. Also, there were no malignant findings of nuclear polymorphism, mitoses, or necrosis. The brown pigments were confirmed to be melanin by bleaching and the Fontana-Masson silver stain method. The MIB-1 labeling index was less than 1%. This tumor also consisted of 50% melanophages, which revealed immunohistochemical reactivity in CD68, CD163, and in (1-AT antibodies. These histological findings led us to diagnose an orbital melanocytoma with partial tumor regression.

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.

Ruptured tectal arteriovenous malformation demonstrated angiographically after removal of an unruptured occipital lobe arteriovenous malformation.

We report a case of ruptured tectal arteriovenous malformation (AVM) that was demonstrated angiographically only after removal of an unruptured occipital AVM. A 57-year-old man presented with sudden onset of diplopia and tinnitus. Computed tomography revealed a small hemorrhage in the right tectum mesencephali with intraventricular hemorrhage. Magnetic resonance imaging and angiography disclosed AVM in the right occipital lobe which was separate from the hemorrhagic lesion. Angiography demonstrated that the right occipital AVM was fed by the parieto-occipital artery and drained into the superior sagittal sinus and vein of Galen. However, no abnormal vascular lesion was detected near the tectum mesencephali. As venous hypertension was considered the reason for hemorrhage, the occipital AVM was completely resected. Postoperative angiography demonstrated disappearance of the occipital AVM, but it also disclosed a small tectal AVM fed by branches from the superior cerebellar artery, which had not been detected on preoperative angiography. This was considered the true cause of hemorrhage, and gamma knife surgery was accordingly performed. Even if an AVM is demonstrated, if the lesion does not correspond to the hemorrhage we recommend serial angiographical evaluation so that a small AVM is not missed.

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.

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.

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.

Nonfunctioning endocrine tumor arising from intracranial ectopic pancreas associated with congenital brain malformation.

CASE REPORT: We report a case of a nonfunctioning pancreatic endocrine tumor arising from intracranial ectopic pancreatic tissue. HISTORY: An 11-year-old girl was admitted to our hospital with a brain tumor. Hydrocephalus and brain malformation were apparent at birth. We first identified a mass-like lesion in the child's brain at age 4 months. We monitored the lesion yearly by magnetic resonance imaging (MRI) until she reached age 11 years. Gadolinium-enhanced MRI showed the lesion to be a tumor, which was resected. PATHOLOGY: Examination of surgical specimens revealed a mature pancreatic tissue. We also identified monotonous neoplastic cells with round nuclei and positive immunoreactivity for synaptophysin, chromogranin A, and neurospecific enolase. However, these cells were negative for pancreatic endocrine markers. We diagnosed nonfunctioning pancreatic endocrine tumor arising from intracranial ectopic pancreatic tissue. CONCLUSION: Migrating pancreatic elements may have induced brain malformation during embryonic development and subsequently become malignant.

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.

[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.

[Unilateral sudden deafness as a primary symptom of brainstem and cerebellar infarction].

We report a case with a unilateral sudden sensorineural hearing loss caused by an infarction of brainstem and cerebellum. The patient was a 74-year-old male presented with a sudden onset of hearing loss and tinnitus in the right ear and dizziness. Steroid was administered on suspicion of idiopathic sudden deafness. However, the initial symptoms were deteriorated approximately 2 weeks later. He newly complained of the numbness of the right face and double vision, and he was transferred to our hospital for further evaluation. Neurological examination demonstrated horizontal nystagmus, diminution in the right facial sensation, right peripheral facial palsy, right hearing loss and cerebellar ataxia. Urgent MRI disclosed fresh infarctions of the right middle cerebellar peduncle and cerebellum localized in the territory of anterior inferior cerebellar artery. In general, idiopathic sudden deafness and Meniere's disease are frequent diagnosis in cases of sudden hearing loss with vertigo, but these symptoms may rarely be caused by cerebrovascular disorder. In patients with risk factors for arteriosclerosis, cerebrovascular disorder should be taken into consideration even if idiopathic sudden deafness may be suspected clinically. We emphasize the diagnostic importance of careful observation on neurological findings and early detection of radiological abnormalities on MRI.