Fourth ventricular meningioma: a case report and literature review

A 60-year-old man was admitted with slowly progressive dizziness. Cranial nerve evaluation found no abnormalities. Magnetic resonance imaging revealed a well-circumscribed mass with homogeneous enhancement located in the fourth ventricle. The patient underwent surgery for the removal of the tumor via the bilateral suboccipital approach. Subtotal removal of the tumor was achieved in a piecemeal fashion. Histological diagnosis was meningothelial meningioma. Fourth ventricular meningiomas are extremely rare. We reviewed the literature and discussed the features of fourth ventricular meningiomas.     

非典型性脑室外中枢神经细胞瘤一例报告及文献复习

目的 探讨提高非典型性脑室外中枢神经细胞瘤(EVN)诊疗水平的策略和方法.方法 对2010年1月南京大学医学院附属鼓楼医院神经外科收治的1例非典型性EVN的临床表现、影像学表现、病理学特征及显微外科手术预后等进行回顾性分析.结果 肿瘤位于左侧额部,与脑组织分界清楚,MRI检查T1WI相表现为等信号或稍低信号,T2WI相表现为略高信号,增强后强化不均匀,术前诊断为脑膜瘤.术中见肿瘤组织呈灰红色,质软、血供一般,快速病理倾向为高级别胶质瘤.术后免疫组化肿瘤细胞示GFAP部分(+),S100(+),NeuN(+),Syn(+),Olig2(+),CgA(+),NSE(+),EMA(-),NF(+),CD99(-),P53个别细胞(+),Ki-67约10%(+);提示增殖指数高,考虑为非典型性EVN.结论 EVN影像学表现虽具有一定的特征,但免疫组化结果对确诊很重要.显微手术是治疗最佳手段,预后可能与肿瘤的间变性和增殖指数有关.     

Central neurocytoma presenting with intraventricular hemorrhage: case report and review of literature

We report a case of a 25 year old man presenting with acute headache, vomiting and nuchal rigidity. Computed Tomography (CT) scan and MRI without contrast showed a right ventricular hemorrhage surrounding a mass lesion. The tumor and hematoma were completely removed by neurosurgical transcortical-transventricular approach. Anatomopathological analysis revealed a central neurocytoma. Central neurocytoma seldom present with hemorrhage. We review 16 cases of neurocytoma with hemorrhage. It is important to recognize central neurocytoma as a cause of intraventricular hemorrhage, especially in adolescents and young adults. Outcome is often favorable when the tumor is completely removed. In some patients the clinical course is more aggressive and additional treatment such as radiotherapy, radiosurgery or chemotherapy is needed.     

中枢神经细胞瘤5例报告及文献复习

目的探讨提高中枢神经细胞瘤诊疗水平的策略和方法。方法回顾分析2004年8月至2006年4月我院收治的5例中枢神经细胞瘤，总结其临床表现、影像学表现、病理学特征及显微外科手术治疗效果。结果肿瘤位于侧脑室前部或室间孔附近，呈宽基底与侧脑室壁或透明隔相连。肿瘤边缘及内部可见多发囊泡，常见钙化，MRI检查T1WI、T2WI相均表现为等或略高信号，增强后呈轻至中度强化。免疫组织化学染色均显示神经突触素阳性。显微外科手术治疗5例，其中全切除4例，次全切除1例，1例死于术后并发症，4例随访至今未复发。结论中枢神经细胞瘤影像学表现具有一定特征，是术前诊断的重要参考。显微手术切除肿瘤是最佳的治疗手段，预后良好。     

Primary fourth ventricular meningioma: case report and review of the literature

Primary fourth ventricle meningiomas are extremely rare, and they are defined as meningiomas arising from the choroid plexus and lying strictly within the fourth ventricle. In this report we present a 61-year-old man with progressive worsening vertigo and gait disturbance, and new onset of diplopia. Neurological examination revealed bilateral abducens nerve paralysis, horizontal nystagmus, and gait disturbance with truncal ataxia. Neuroimaging revealed a mass lesion in the fourth ventricle with brain stem compression, and obstructive hydrocephalus. The patient was operated in the prone position with suboccipital craniotomy and splitting the lower vermis. Total resection of the tumor was achieved with no intra- or post-operative complications. Histopathologic examination revealed fibroblastic type meningioma (WHO grade I).     

四脑室神经鞘瘤1例

中枢神经系统髓内神经鞘瘤十分少见，主要发生于脊髓，也可见于颅内的幕上、幕下。发生于四脑室、小脑蚓部者更为罕见，翻阅国外文献曾报导2例。我科近年遇到1例，现结合文献报道如下。     

Malignant progression of an extraventricular neurocytoma arising from the VIIIth cranial nerve: A case report and literature review

A rare case of extraventricular neurocytoma (EVN) arising from the VIIIth cranial nerve in a 34‐year‐old woman is reported. The patient had a 20‐year history of hearing loss and facial palsy. Computed tomography showed a 3‐cm enhancing lesion in the left cerebellopontine angle (CPA). At operation, the tumor was seen to originate from the cochlear and vestibular nerves. The tumor was subtotally resected. Histologically, the tumor consisted of uniform cells with oval to round nuclei and scant cytoplasm. Immunohistochemically, the tumor cells were positive for synaptophysin, but negative for glial fibrillary acid protein and S‐100 protein. The Ki‐67 labeling index was 0%. Twelve years after the operation, magnetic resonance imaging (MRI) showed tumor recurrence at the left CPA. The tumor was subtotally resected, and radiation therapy was given. Histologically, the tumor consisted of round cells with mild atypia and one mitosis/20 high‐power fields (HPF). Immunohistochemically, tumor cells showed the same findings as the first operation sample, except for the Ki‐67 labeling index (3%). Twelve years after the second operation, MRI showed a second tumor recurrence at the left CPA and surroundings of the brain stem. The tumor was subtotally resected. Histologically, the tumor consisted of anaplastic short spindle cells and five mitoses/10 HPF. The immunohistochemical findings were almost the same as the earlier operation samples. However, the Ki‐67 labeling index was 20%. In addition, tumor cells from the third specimen were more strongly and more diffusely positive for GAB1 (growth factor receptor‐bound protein 2‐associated binding protein 1) compared to those of the earlier specimens. Electron microscopy showed the presence of numerous cell processes with a dense core and clear vesicles and microtubules. GAB1 immunostaining also indicated that malignant progression might be associated with the sonic hedgehog signaling pathways. To the best of our knowledge, this is the first report of an EVN arising from the VIIIth cranial nerve with malignant progression.     

Third ventricle, cerebral aqueduct, and fourth ventricle mixed ependymal neurocytoma: a case report and review of the literature

Purpose

A case report of an 8-year-old female who underwent interhemispheric transcallosal approach to the third ventricle, cerebral aqueduct, and fourth ventricle for resection of a mixed ependymal neurocytoma and review of the literature was evaluated.

Methods

An Ovid MEDLINE review of the literature was conducted starting in 1946 to current using search terms as described in our keywords.

Results

A total of 16 neurocytoma have been described in the literature as either posterior third ventricle or posterior fossa in origin. Of these lesions, five have been described as mixed glial–neuronal and all of these were located in the fourth ventricle. To our knowledge, this is the first described mixed glial–neuronal tumor located in the posterior third ventricle and aqueduct.     

Hemihydranencephaly: case report and literature review

Hydranencephaly is a severe brain condition characterized by complete or almost complete absence of cerebral cortex with preservation of meninges, basal ganglia, pons, medulla, cerebellum, and falx. It has been ascribed to different causes (infections, irradiations, fetal anoxia, medications, twin-twin transfusion), all leading to vascular disruption. Hemihydranencephaly is an extremely rare condition in which the vascular anomaly is unilateral. We report on a patient who was suspected to have hydrocephalus in utero; a brain magnetic resonance imaging scan showed left-sided hydranencephaly with preservation of basal ganglia. The patient developed signs of right hemiparesis but notably has only mild language delay. The available literature on hemihydranencephaly is reviewed.     

Lipomeningioma: case report and review of the literature

We present a case of histologically confirmed lipomeningioma, the first to our knowledge reported in Australia. A 61-year-old man presented with seizures and confusion, and was found to have a non-enhancing left extra axial temporo-parietal lesion on CT and MRI scan. On MRI, the mass lesion showed hyper-intensity on the T1 weighted images, hypo-intensity on fat suppressed T2 weighted images and no enhancement with intravenous gadolinium, indicating a mass consisting predominately of fatty tissue. A subsequent CT also showed the mass lesion to be hypodense with Hounsfield units indicating fatty tissue. A durally based tumour with high fat content macroscopically was excised at craniotomy under ultrasound guidance. Post-operative recovery was uneventful. Histology demonstrated a meningioma with high lipid content in the form of mature adipocytes and without atypical features. While not exceedingly rare, fewer than 30 cases of lipomeningioma, lipomatous meningioma, or lipidised meningioma have been reported in the world literature.     

Sunct syndrome: case report and literature review

The case of a woman with short neuralgiform paroxysmal attacks located in orbital-periorbital area and associated with autonomic features of ten years duration is reported. This headache syndrome is compared with trigeminal neuralgia involving the first branch of the nerve. Duration, intensity, spreading of the pain and presence of accompanying ipsilateral vasomotor phenomena may be of help in the differential diagnosis. According to the latest reports, sex distribution which passed from 17 men/2 women to 18/6 and effect of the carbamazepine on pain would not appear to have an effect. Nevertheless other reports are needed to distinguish these two clinical syndromes and to develop an etiological and pathogenesis hypothesis.     

Metastatic craniopharyngioma: case report and literature review

Background  Distant spread of craniopharyngioma is a rare but important complication. Most cases are a result of spread along the surgical path. We describe a rare case of metastatic leptomeningeal craniopharyngioma as a result of dissemination along CSF pathways in a child. A review of previously described cases is provided. Case presentation  A 14-year-old male was diagnosed with metastatic craniopharyngioma on routine follow-up imaging after multiple surgeries and radiation for locally recurrent craniopharyngioma. The lesion was erosive through the right parietal bone, but had remained clinically silent. The lesion was distant from previous surgical paths. The patient underwent right parietal craniotomy and resection of the lesion. Duraplasty and cranioplasty were necessary for closure. Histopathology confirmed adamantinomatous craniopharyngioma. One-year follow-up demonstrated no recurrence. Discussion  A review of reported cases suggests that leptomeningeal implantation may be an important step in metastases of craniopharyngioma, although the mechanism is poorly understood. Attention to tumor spillage at the time of surgery may be important in preventing distant recurrences.     

Tacrolimus-related polyneuropathy: case report and review of the literature

Patients, in particular recipients of orthotopic liver transplants, receiving the immunosuppressant tacrolimus (FK-506), are at risk for developing central neurotoxic adverse events. We report the occurrence of a tacrolimus-induced peripheral neurotoxic event, i.e. pure motor axonal polyneuropathy of the lower limbs in a 44-year-old woman, 9 days after combined orthotopic liver and pancreas transplantation. She was treated for 5 days with intravenous immunoglobulins. Partial recovery followed over months to years. An overview of all 11 reported FK506-associated polyneuropathies is given.     

Brainstem disconnection: case report and review of the literature

We report a neonate with brainstem disconnection. Only five similar cases have previously been described, the longest survival was 7 weeks. The newborn exhibited muscular hypertonia, absent suction, insufficient breathing, and seizures. Magnetic resonance imaging showed a disconnection between the upper pons and the medulla oblongata and cerebellar hypoplasia. The basilar artery was not visible. These neuroimaging findings are clearly different from other midbrain or hindbrain malformations with cerebellar hypoplasia. This pattern and a previously reported autopsy point to a malformation, not a disruption.