Malignant meningioma as a second malignancy after therapy for acute lymphatic leukemia without cranial radiation

Rationale Meningiomas in the pediatric age group are very rare tumors, comprising about 1–4.2% of all primary pediatric intracranial tumors.Case report We present a 17-year-old patient who suffered from an intraventricular malignant meningioma. At the age of 2 years, acute lymphatic leukemia (common ALL [cALL]) was diagnosed and successfully treated with chemotherapy. There was no cranial radiation therapy.In December 2001, 13 years after diagnosis of cALL, he complained of headache, vomiting, and walking difficulties. Magnetic resonance imaging showed an enhancing mass with cystic components in the trigone of the right lateral ventricle. The tumor was removed completely. Histological diagnosis revealed a malignant papillary meningioma. After removal of a recurrent meningioma 16 months later, he received local radiotherapy.Conclusion Pathogenetic mechanisms, treatment options, and prognosis of meningiomas and secondary malignancies of this age group are discussed.     

The Osteoporotic Condition as a Predictive Factor for Hemorrhagic Transformation in Acute Cardioembolic Stroke

ObjectiveHemorrhagic transformation (HT) can be occurred after acute cerebral infarction. HT can worse symptoms in severe cases and adversely affect long-term prognosis. As bone and vascular smooth muscle are composed of type 1 collagen, we aimed to identify a potential relationship between bone mineral density (BMD) and HT after acute cardioembolic stroke. MethodsAs an indicator of BMD, we used mean frontal skull Hounsfield unit (HU) values on brain computed tomography (CT). Multivariative hazard ratios were calculated using Cox regression analysis to identify whether the osteoporotic condition was an independent predictor of HT after acute cardioembolic stroke. ResultsThis 11-year analysis enrolled 506 patients who diagnosed as acute cardioembolic infarction. The first tertile of skull HU value was an independent predictor of HT development compared to the third tertile (hazard ratio, 2.12; 95% confidence interval, 1.13–3.98; p=0.020). We observed no interactions between age and skull HU with respect to HT statistically. ConclusionThe results of this study revealed an association between osteoporotic conditions and HT development after acute cardioembolic stroke. A convenient method to measure the cancellous bone HU value of the frontal skull using brain CT images may be useful for predicting HT in patients with acute cerebral infarction.     

Clinical Features and Treatment Outcome of Chordoid Meningiomas in a Single Institute

Objective

Meningioma is the second most common primary central nervous system neoplasm. In contrast, chordoid meningioma is rare; due to the paucity of cases, little is known about its clinical features or treatment outcomes. The objectives of this study were to describe the clinical characteristics and outcomes for patients with chordoid meningioma.

Methods

In total, 16 patients, with newly diagnosed chordoid meningioma who underwent surgical excision between 1999 and 2012 were included. We retrospectively evaluated the medical records, radiological findings, and pathological findings. The median follow-up period was 56.5 (range, 3-170) months. The MIB-1 labeling index ranged from 1 to 26.60% (median, 5.04).

Results

Simpson grade I, II, and III resections were performed in four, nine, and three patients, respectively. The overall recurrence rate was 37.5%. Overall progression-free survival (PFS) after resection was 94.7 months (95% CI=62.9-126.6). Of the 4 patients with Simpson grade I resection, recurrence occurred in one patient. Among the Simpson grade II and III resection groups, eight patients underwent adjuvant radiation therapy and they showed significantly longer PFS (121 months, 95% CI=82.1-159.9) than the patients who underwent surgery alone (40.5 months, 95% CI=9.6-71.3) by the log-rank test (p<0.05).

Conclusion

Chordoid meningiomas are difficult to manage and have a high rate of recurrence. Complete resection of the tumor is a key determinant of better outcomes. Adjuvant radiation therapy is recommended, eparticulary when Simpson grade I resection was not achieved.     

非典型抗精神病药物所致恶性综合征的临床特征

本文目的是探讨非典型抗精神病药物所致恶性综合征(NMS)的临床特征,为临床早期诊断和治疗提供参考。抗精神病药物所致NMS是一种严重的药物不良反应,几乎所有的抗精神病药物均可引起。与典型抗精神病药物相比,非典型抗精神病药物所致NMS发生率更低、病情更轻、死亡率更低。不同种类非典型抗精神病药物所致NMS临床特点各异。对于老年人及应用抗抑郁药物者发生的NMS尤应引起重视,因为这些因素可增加死亡的风险。     

A Malignant Transformation of a Spinal Epidural Mass from Ganglioneuroblastoma to Neuroblastoma

Ganglioneuromas are benign tumors. Surgical excision is the treatment of choice with very good prognosis. However, neuroblastomatous malignant transformation of ganglioneuromas was previously reported. We report a patient with spinal neuroblastoma recurrent from a ganglioneuroblastoma after disease free survival of 13 years. This is one of the rare examples of spinal neuroblastoma and to our knowledge the second case report with malignant transformation from a ganglioneuroblastoma or a ganglioneuroma. The present case is the only report in the literature with further genetic investigations.     

鞍结节脑膜瘤的临床特征及手术治疗（附21例报告）

目的探讨鞍结节脑膜瘤的临床特征及治疗策略。方法回顾性分析2003年10月2008年10月收治的21例鞍结节脑膜瘤患者的临床资料。结果21例肿瘤均全切。术后视力改善19例,无变化1例,死亡1例。结论鞍结节脑膜瘤临床表现以视力下降为主,合适的手术入路及术中对鞍结节周围重要结构的保护对改善患者的预后有重要帮助。     

Extracranial Extension of Intracranial Atypical Meningioma En Plaque with Osteoblastic Change of the Skull

Meningioma is a common primary tumor of central nervous system. However, extracranial extension of the intracranial meningioma is unusual, and mostly accompanied the osteolytic change of the skull. We herein describe an atypical meningioma having extracranial extension with hyperostotic change of the skull. The patient was a 72-year-old woman who presented a large mass in the right frontal scalp and left hemiparesis. Brain magnetic resonance imaging and computed tomography scans revealed an intracranial mass, diffuse meningeal thickening, hyperostotic change of the skull with focal extension into the right frontal scalp. She underwent total removal of extracranial tumor, bifrontal craniectomy, and partial removal of intracranial tumor followed by cranioplasty. Tumor pathology was confirmed as atypical meningioma, and she received adjuvant radiotherapy. In this report, we present and discuss a meningioma en plaque of atypical histopathology having an extracranial extension with diffuse intracranial growth and hyperostotic change of the skull.     

显微镜下锁孔手术治疗56例颅内小脑膜瘤的疗效分析

目的探讨小脑膜瘤的治疗策略。方法回顾性分析2012年1月至2014年12月采用显微镜下经锁孔手术治疗的56例小脑膜瘤(直径1~3 cm)患者的临床资料并随访观察疗效。结果 56例患者中,男性15例、女性41例,年龄平均(52±10)岁。肿瘤位于大脑半球凸面35例,矢状窦旁4例,大脑镰旁1例,小脑半球凸面2例,前颅底3例,鞍区6例,中颅底2例,桥小脑角3例。所有肿瘤采用锁孔手术入路,肿瘤全切除,术后出现不良反应2例(3.57%)。42例获得随访,随访时间平均30个月,均未发现肿瘤复发,无永久性神经功能障碍。结论锁孔手术治疗颅内小脑膜瘤安全有效,支持对于小脑膜瘤应该早期手术治疗的观点。     

Surgical Experience of Infratentorial Meningiomas : Clinical Series at a Single Institution during the 20-Year Period

Objective

Based on surgical outcomes of patients with infratentorial meningiomas surgically treated at our institution, we analyzed the predictors for surgical resection, recurrence, complication, and survival.

Methods

Of surgically treated 782 patients with intracranial meningioma, 158 (20.2%) consecutive cases of infratentorial location operated on between April 1993 and May 2013 at out institute were reviewed retrospectively. The patients had a median age of 57.1 years (range, 16--77 years), a female predominance of 79.7%, and a mean follow-up duration of 48.4 months (range, 0.8--242.2 months).

Results

Gross total resection (Simpson''s grade I & II) was achieved in 81.6% (129/158) of patients. Non-skull base location was an independent factor for complete resection. The recurrence rate was 13.3% (21/158) and the 5-, 10-, and 15-year recurrence rates were 8.2%, 12.0%, and 13.3%, respectively. Benign pathology, postoperative KPS over than 90, low peritumoral edema, and complete resection were significantly associated with longer recurrence-free survival rate. The 5-, 10-, and 15-year survival rates were 96.2%, 94.9%, and 94.9%, respectively. Benign pathology, postoperative KPS over than 90 and complete resection were significantly associated with a longer survival rate. The permanent complication rate was 13% (21/158). Skull base location and postoperative KPS less than 90 were independent factors for the occurrence of permanent complication.

Conclusion

Our experience shows that infratentorial meningiomas represent a continuing challenge for contemporary neurosurgeons. Various factors are related with resection degree, complications, recurrence and survival.     

Malignant transformation of intracranial meningeal melanocytoma. Case report and review of the literature

Meningeal melanocytoma is an uncommon pigmented neoplasm that affects the CNS and develops in the cranial and spinal leptomeninges. Here we report on a case of malignant transformation of intracranial supratentorial meningeal melanocytoma which recurred after 3 years as malignant melanoma. This case demonstrates that the biological behavior of melanocytoma is uncertain and that these lesions may recur as malignant melanoma.     

Malignant progression in pleomorphic xanthoastrocytoma: personal experience and review of the literature

Pleomorphic xanthoastrocytoma (PXA) is a rare primary low-grade astrocytic tumor, recently classified as a neuroglial tumor. It generally occurs in children and young adults and shows benign behaviour (WHO II), although an anaplastic variant and malignant potential have been described. Pleomorphic xanthoastrocytomas with malignant transformation have been reported in three out of eight patients operated on for this type of tumor in our department in the last 15 years. The three patients were two adult women and a child, the primary tumors were located in the cortex of the right temporal lobe, and treatment consisted of complete surgical resection. Histological examination revealed simple PXA in two patients and a PXA with anaplastic foci in the other. Mean recurrence time was 5.7 years, with the original xanthoastrocytoma evolving to glioblastoma in two cases and anaplastic astrocytoma in the third. All three patients underwent a second operation, followed by adjuvant therapies. Two died from tumor progression and one from brain edema after intracerebral haemorrhage. A review of the available PXA literature dating back to 1979 revealed 16 cases of primary anaplastic astrocytoma and 21 cases of PXA with malignant transformation. Our experience adds three more cases of malignant transformations, outlining once again the potential malignancy of pleomorphic xanthoastrocytomas and the fact that prognosis in these cases is the same as for primary anaplastic astrocytoma and glioblastoma. Analysis of glioneuronal markers, Ki67 and p53 in all pleomorphic xanthoastrocytomas did not prove to be a discriminating factor to identify a subgroup of xanthoastrocytomas prone to malignancy. Accordingly, these tumors demand close long-term clinical and radiological follow-up.     

Malignant glioblastomatous transformation of a low-grade glioma in a child

Background  The term of low-grade glioma addresses a favorable clinical outcome with indolent histological features in general consideration; however, recent studies underline the inconsistency, which originates from the accumulation of different histologic subtypes in this terminology. The malignant transformation of a low-grade glioma is unusual but presents a poor prognosis. Case history  We report a case of a 12-year-old boy, who was referred for complaints of recurrent seizures. His physical examination was unremarkable, but it was learned that a peripheral mass lesion located on the left posterior parietal lobe—which had been thought to be a low-grade glioma—had been detected on a magnetic resonance imaging 2 years ago at a different hospital. The patient was then treated with valproate and carbamazepine for the seizures and advised to be followed up without any additional diagnostic and therapeutic studies for his suspected low-grade glioma. A recent magnetic resonance imaging study showed enlargements of the mass and surrounding edema with additional necrosis. Surgical excision of the tumor was performed. After the diagnosis of glioblastoma multiforme, the patient received radiation therapy and chemotherapy with a good clinical recovery without any evidence of residue or recurrence at 12-month follow-up. Conclusion  The first line treatment modality in the management of low-grade glioma—especially in suitable patients—is clearly surgery. The gross total resection guarantees the distinguishing of the histological types of the low-grade gliomas and reflects the biologic behavior of these tumors. Observation without surgery must be reserved for selected unoperable cases.     

Meningiomas associated with hemorrhage: A report of two cases with a review of the literature

Two cases of extensive spontaneous hemorrhage related to intracranial angioblastic meningiomas are reported. One of these also had glandular and papillary formations with mucin production. A review of 115 meningiomas with significant hemorrhage including the two current instances disclosed that they occurred in the first to ninth decades of life but were most common in the fourth to eighth decades (85%), with a peak occurrence in the fifth decade (24%). The average age of 51 years was 9 years more than in those patients with non-bleeding meningiomas. The female- to-male ratio was 6:5 for the entire series. Bleeding meningiomas arose most often on the cerebral convexity followed by the parasagittal region, the lateral ventricle, and the sphenoid ridge. Bleeding most frequently involved multiple sites, and was followed in decreasing frequency by hemorrhage into the subarachnoid space, subdural space, intracerebral tissue including the peritumoral region, and intratumoral substance. Meningocytic menin-gioma was the most common type of bleeding meningioma, followed in decreasing frequency by the angioblastic, fibroblastic, transitional and malignant types. The mech-anism of bleeding was uncertain in many cases, and multiple factors are probably involved. Patients with bleeding meningiomas had worse prognoses than those with non-bleeding meningiomas.     

Malignant transformation in a case of megalencephalic leukoencephalopathy with subcortical cysts: An extreme rarity in a rare disorder

Megalencephalic leukoencephalopathy with subcortical cysts (MLC) is an autosomal recessive inherited disorder characterized by macrocephaly, progressive motor disability, seizures, mild cognitive decline, slow progression, and typical magnetic resonance imaging (MRI) findings. Age of onset of symptoms is described from birth to 25 years. Late onset presentation is very rare, only few cases have been reported worldwide. Most important clue for diagnosis is the characteristic MRI changes that include diffuse involvement of subcortical white matter mainly in frontoparietal region with relative sparing of central white matter along with subcortical cysts mostly in anterior temporal region. Cysts are usually benign and slowly progressive. Malignant transformation of cysts has not been reported as yet. We herein report a very unusual and probably the first case of MLC who presented to us in a unique manner with late onset and malignant transformation of cyst in left temporal region leading to rapid neurological decline. This case report highlights a possible life-threatening complication of a previously known slowly progressive disease warranting urgent neurosurgical intervention.     

Evaluation of radiological recurrence patterns following gamma knife radiosurgery for solitary meningioma previously treated via cranial surgery

The use of gamma knife radiosurgery (GKS) for meningiomas after cranial surgery has been extensively evaluated; however, studies on tumor progression, including recurrence out of the margin dose line, are scarce. Hence, we aimed to evaluate the meningioma recurrence after GKS within and out of the margin dose. We included 37 consecutive patients with World Health Organization (WHO) grade 1 meningiomas who were treated with GKS following cranial surgery. Radiologically indicated recurrences were classified into three patterns by their relationship to the margin dose and tumor. The median follow-up was 58.9 months; 2 (5.4%) patients died. Only 2 (5.4%) patients did not keep active daily lives because of tumor progression. Cumulative local control at 5 years was 85.2%. Local recurrence and recurrence out of the margin dose occurred in 5 (13.5%) and 13 (35.1%) patients, respectively. A larger preoperative maximum diameter was a risk factor for local recurrence (hazard ratio [HR]: 2.118; P = 0.033), adjacent progression (HR: 1.633; P = 0.015), and remote progression (HR: 2.016; P = 0.003). Symptomatic adverse radiation effects occurred in 1 patient. Salvage GKS and cranial surgery were performed in 9 (24.3%) and 8 (21.6%) patients, respectively. Progression to WHO grade 2–3 occurred in 5 (13.5%) patients. A larger preoperative maximum diameter was a risk factor for progression of WHO grade (HR: 2.016, P = 0.033). Progression out of the margin dose was associated with a larger preoperative tumor size.     

Malignant paraganglioma of the sellar region mimicking a pituitary macroadenoma

We report a case of malignant sellar and suprasellar paraganglioma presenting as pituitary macroadenoma, causing headache and visual deficits. The characteristics of this tumor at this rare location are discussed.     

脑微出血对非腔隙性梗死抗栓治疗后早期出血转化的预测价值的初步研究

【摘要】 目的 评估脑微出血（cerebral microbleed,CMB）对采用抗栓治疗的非腔隙性梗死患者早期脑出血转化的预测价值。 方法 本研究为前瞻性研究,入选2011年6月～2012年10月广东省人民医院神经科发病后24 h内住院的心源性脑栓塞及大动脉粥样硬化性梗死患者。根据临床情况对所有患者予以抗血小板或抗凝治疗。采用颅脑磁共振成像（magnetic resonance imaging,MRI）检测CMB数目及分布情况,用颅脑计算机断层扫描（computed tomography,CT）判断发病后1周内出血转化（hemorrhagic transformation,HT）情况。根据有无HT将患者分为有HT组和无HT组,比较两组间一般临床资料、病因、美国国立卫生研究院卒中量表（National Institutes of Health Stroke Scale,NIHSS）评分及CMB是否存在,并采用多因素逐步Logistic回归模型分析HT的独立危险因素。 结果 167例患者中18例发生HT（11%）,57例检出CMB（34%）,CMB检出率在是否合并HT的患者之间差异无显著性（分别为22%和36%,P＞0.05）。抗血小板治疗患者是否存在CMB,早期HT差异无显著性（分别为12%和9%,P＞0.05）;抗凝治疗患者差异无显著性（分别为17%和12%,P＞0.05）。Logistic回归分析显NIHSS（≥8）和心源性脑栓塞与HT有关,其比值比（odds ratio,OR）值分别为3.65［95%可信区间（confidence interval,CI）1.47～8.26］和5.82（95%CI 1.89～15.38）;高血压、大动脉粥样硬化以及CMB并不增加HT风险,其OR和95%CI值分别为1.05（0.97～1.12）,2.31（0.91～3.45）以及0.35（0.09～1.41）。 结论 CMB对非腔隙性脑梗死患者抗栓治疗后早期HT缺乏预测价值,疾病严重程度以及病因类型有助于估计HT风险。     

以神经系统症状首发的恶性肿瘤37例临床分析

恶性肿瘤以神经系统症状起病的并非少见,但许多原发肿瘤症状不表现或隐匿.常以神经系统症状就诊从而被误诊.故现将自2001年10月至2006年11月南京军区总医院神经内科收治的以神经系统症状首发且均经CT、MRI或活检等检查后确诊的37例恶性肿瘤患者的临床资料报道如下,以期提高其诊断正确率.