Leptomeningeal dissemination of low-grade intramedullary gliomas: About one case and review

Leptomeningeal (LM) dissemination arises rarely in patients with low-grade gliomas. Few cases of LM dissemination of spinal cord low-grade gliomas have been reported in children. Here, we report a case of an intramedullary spinal cord low-grade glioma occurring in an adult presenting by LM dissemination.     

The Clinical Features of Spinal Leptomeningeal Dissemination from Malignant Gliomas

Objective

The incidence of leptomeningeal dissemination from malignant glioma is rare, so the clinical features of this are not well documented yet. We attempted to determine the clinical features of leptomeningeal dissemination from malignant gliomas.

Methods

We retrospectively analyzed 11 cases of leptomeningeal dissemination of malignant glioma, who were treated at our institution between 2006 and 2009. We investigated the clinical features of these patients by considering the following factors : tumor locations, the events of ventricular opening during surgery and the cerebrospinal fluid (CSF) profiles, including the cytology.

Results

The group was composed of 9 males and 2 females. The histological diagnosis of their initial intracranial tumors were 4 primary glioblastoma, 3 anaplastic astrocytoma, 1 anaplastic oligoastrocytoma, 2 ganglioglioma and 1 pleomorphic xanthoastrocyotma with anaplastic features. The mean age of the patients at the time of the initial presentation was 42.8±10.3 years. The mean time between surgery and the diagnosis of spinal dissemination was 12.3±7.9 (3-28) months. The mean overall survival after dissemination was 2.7±1.3 months. All our patients revealed a history of surgical opening of the ventricles. Elevated protein in the CSF was reported for eight patients who had their CSF profiles checked.

Conclusion

We propose that in the malignant gliomas, the surgical opening of ventricles can cause the spinal leptomeningeal dissemination and the elevated protein content of CSF may be a candidate marker of leptomeningeal dissemination.     

Glioblastoma Multiforme in the Pineal Region with Leptomeningeal Dissemination and Lumbar Metastasis

We report a case of a 31-year-old woman with glioblastoma multiforme (GBM) in the pineal region with associated leptomeningeal dissemination and lumbar metastasis. The patient presented with severe headache and vomiting. Magnetic resonance imaging (MRI) of the brain showed a heterogeneously enhanced tumor in the pineal region with obstructive hydrocephalus. After an urgent ventricular-peritoneal shunt, she was treated by subtotal resection and chemotherapy concomitant with radiotherapy. Two months after surgery, MRI showed no changes in the residual tumor but leptomeningeal dissemination surrounding the brainstem. One month later, she exhibited severe lumbago and bilateral leg pain. Thoracico-lumbar MRI showed drop like metastasis in the lumbar region. Finally she died five months after the initial diagnosis. Neurosurgeons should pay attention to GBM in the pineal region, not only as an important differential diagnosis among the pineal tumors, but due to the aggressive features of leptomeningeal dissemination and spinal metastasis.     

Intracranial Meningioma with Leptomeningeal Dissemination : Retrospective Study with Review of the Literature

Objective

The purposes of this article are to present 5 cases of intracranial meningioma with leptomeningeal dissemination (LD) and investigate the characteristics of this disease.

Methods

We present a retrospective case series of 5 females at our institutions (age ranged 21-72 years, mean 54.6 years) diagnosed with LD of an intracranial meningioma after surgery between 1998 and 2013. A database search revealed 45 cases with LD of meningioma in the English literature. Characteristic features were analyzed and compared.

Results

The incidence rate at our institutions of LD of meningioma was 0.9% (5/534). World Health Organization (WHO) grade was distributed as follows: I : 2, II : 2, and III : 1. Time to LD ranged from 2.5 months to 6.9 years; the patient with WHO grade III had the shortest interval to LD. The patient with an intraventricular meningioma (WHO grade II) had the second shortest interval to LD (1.7 years), and simultaneously revealed both LD and extraneuronal metastases. Four of 5 patients showed a disease progression, with the survival ranging from 1 month to 3.8 years after LD. Based on the literature, the initial tumor was an intraventricular meningioma in 9 patients, and their time to LD was shorter on average (mean 1.9 years). Histologically, 26 of 45 (58%) were initially diagnosed with a WHO grade II or III meningioma, and 6 of 19 patients (32%) with WHO grade I revealed malignant transformation.

Conclusion

This study shows that intraventricular location and histologically aggressive features seem to increase the chance of LD of meningioma.     

Recurrent low-grade glioma in children with special reference to computed tomography findings and pathological changes

Recurrent low-grade gliomas in children were studied with special reference to correlation between the computed tomography (CT) scan and pathological findings at recurrence. During the past 16 years (1970–1986) 105 cases of primary pediatric brain tumors were treated in our hospital. Seventeen of these had low-grade glioma, seven of which (five astrocytomas, one ependymoma, and one subependymoma) had recurrence of tumor by the end of 1987. The enhanced CT findings were classified into three types — cystic, false cystic, and solid, according to Lapras' classification. The pathological findings of recurrent tumors were reviewed with particular attention to malignant transformation. The results showed that in two out of three cases where CT findings had changed from initial cystic to solid type at recurrence malignant transformation was revealed. Enhanced CT scan was also useful for detecting small asymptomatic recurrent tumors in children. On the other hand, surgical procedures and adjuvant therapies demonstrated no significant relationship with recurrence. It is suggested that the recurrence of low-grade glioma in children is not rare, and that changes in findings on contrast enhanced CT might reflect malignant transformation in a recurrent tumor, necessitating a careful follow-up.     

Direct Infiltration of Brainstem Glioma Along the Cranial Nerves

The authors describe a case of a low-grade brainstem glioma extending along the cranial nerves without any evidence of leptomeningeal spread. The tumor extended directly along the VII-VIIIth cranial nerve complex and also along the trigeminal nerve, which is quite an unusual characteristic of the glial tumors.     

Intracranial Undifferentiated Sarcoma Arising from a Low-Grade Glioma: A Case Report and Literature Review

Undifferentiated sarcomas are rarely identified in the intracranial region. A 23-year-old man was admitted with a chief complaint of headache. Initial magnetic resonance images showed signs of low-grade glioma in the frontal lobe. Stereotactic biopsy was performed, and a diagnosis of diffuse astrocytoma was confirmed. Three months later, the patient presented with a high-grade tumor as seen on imaging studies. He underwent total resection of the tumor and histopathological tests identified an undifferentiated sarcoma. The patient died eight months later due to massive tumor bleeding. To the best of our knowledge, this is the first report of undifferentiated sarcoma arising from low-grade glioma without any chemotherapy or radiotherapy.     

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.     

Clinical and Genetic Features of Brainstem Glioma in Adults: A Report of 50 Cases in a Single Center

Background and PurposeBrainstem gliomas (BSGs) in adults are rare brain tumors with dismal outcomes. The aim of this study was to determine the clinical and genetic features in a series of BSGs and their association with the prognosis.MethodsFifty patients who underwent a stereotactic biopsy between January 2016 and April 2018 at a single institution were collected. Data on clinicopathological characteristics were analyzed and factors associated with patient survival were identified using a Cox regression model.ResultsThe median age at diagnosis was 55.5 years, and 62% of the patients were male. Glioblastoma (44%) accounted for the largest proportion of BSGs, and oligodendroglioma (2 of 50) was rarely encountered. The IDH mutation (6 of 44) occurred infrequently in astrocytomas, and IDH-mutant tumors harbored both ATRX loss and MGMT promoter methylation at a relatively low level. Wild-type IDH astrocytomas were identified as having high rates of 1p/19q codeletion (5 of 38) and loss of heterozygosity 1p (8 of 38) or 19q (8 of 38) only. In diffuse midline glioma H3K27M mutant, MGMT promoter methylation occurred in three of four cases. Patients were offered radiotherapy and/or concurrent/adjuvant temozolomide chemotherapy, and their median survival time was 13 months. Multivariate analysis revealed that a low tumor grade, absence of tumor enhancement, duration of symptoms ≥3 months, Karnofsky performance status ≥70, and ATRX loss conferred a survival advantage.ConclusionsAdult BSGs showed different molecular genetic characteristics, but also resembled supratentorial gliomas in their clinical features associated with oncological outcomes.     

Leptomeninges as a site of relapse in locally controlled,diffuse pontine glioma with review of literature

Case report Leptomeningeal involvement in diffuse intrinsic brain stem gliomas is rarely diagnosed clinically and in majority of the instances diagnosed only on postmortem examination. We report two cases of diffuse pontine glioma diagnosed clinicoradiologically and treated with conventional radiotherapy.Observations On follow-up, both patients showed clinical features suggestive of meningeal spread although imaging for pontine tumor showed stable disease. Leptomeningeal disease in the spine was confirmed on imaging and in one case by cerebrospinal fluid examination also. During the follow-up of patients with pontine glioma, the possibility of leptomeningeal involvement must be borne in mind.     

膜结合型肿瘤坏死因子相关凋亡诱导配体修饰的人骨髓基质干细胞对脑胶质瘤细胞的体外靶向抗肿瘤作用

目的：探索经膜结合型肿瘤坏死因子相关凋亡诱导配体（TRAIL）修饰的人骨髓基质干细胞（hMSCs）对脑胶质瘤的靶向抗肿瘤作用。方法：通过体外Transwell系统研究hMSCs向胶质瘤的迁徙能力。采用经全长人TRAIL（hTRAIL）重组的腺相关病毒（rAAV）载体（rAAV hTRAIL）对骨髓基质干细胞（MSCs）进行修饰。将hTRAIL修饰的MSCs与人胶质瘤细胞（U87MG）在体外共同培养,分析其对脑胶质瘤的靶向抗肿瘤作用。结果：hMSCs向胶质瘤细胞的迁徙具有高度特异性。经TRAIL修饰的hMSCs不仅在MSCs表面表达全长TRAIL,且能在培养基中分泌可溶性TRAIL。经rAAV-hTRAIL转染后,hMSCs细胞凋亡作用无增加。将TRAIL修饰的hMSCs与U87MG细胞共培养,与采用可溶性TRAIL治疗后相比较,可明显提高U87MG细胞的凋亡率。结论：在体外实验中,hMSCs向胶质瘤细胞的迁徒具有高度特异性,且能诱导胶质瘤细胞凋亡,并提高对sTRAIL不敏感胶质瘤细胞的凋亡率。由此推测膜结合型TRAIL修饰的MSCs在肿瘤微环境中对脑胶质瘤具备靶向抗肿瘤效应。MSCs可能成为脑胶质瘤靶向治疗的有效载体。     

Early cognitive impairment in a subset of patients with presumed low-grade glioma

We investigated the presence of cognitive impairment, in adults with presumed low-grade glioma at early stage of disease prior to major treatments, in relation to neurological symptoms and radiological characteristics of the tumour. Sixteen patients were evaluated. A subset of patients was identified with clearly impaired cognition. Patients with cognitive impairment often had large tumours in the left frontal lobe, were relatively young, and most of them were males. We conclude that cognitive dysfunction may be present already at early stage of disease, and that early identification of patients at risk is warranted.     

Unusual Presentation of Sarcoidosis: Solitary Intracranial Mass Lesion Mimicking a Glioma

We present a patient with sarcoidosis with an isolated intraparenchymal mass lesion that was similar to a glioma on magnetic resonance imaging. On fluid‐attenuated inversion recovery images, a small hyperintense signal change in the right uncus was observed. Three months later, enlargement of the abnormal signal lesion was observed. An initial diagnosis of glioma was made. A biopsy of the temporal lobe tumor was done. On histology, a noncaseating granulomatous inflammation consistent with neurosarcoidosis was diagnosed. Albeit rarely, we should consider the possibility of neurosarcoidosis in the differential diagnosis of isolated intraparenchymal mass lesion, when the mass is located beside the pia mater.     

Complex visual hallucinations as a presentation of temporal low-grade glioma

We report a patient with an unusual presentation of a temporal low-grade glioma with visual symptoms of formed, coloured meaningful images without coexistent psychiatric symptoms or epileptiform activity consistent with a diagnosis of visual hallucinosis. The location and extent of the lesion on the MRI differs from the lesions commonly associated with this diagnosis.     

Recurrence in a different location of a cerebral arteriovenous malformation in a child after radiosurgery

The case of a 9-year-old girl with a right parietal arteriovenous malformation (AVM) of the brain obliterated after gamma knife (GK) radiosurgery with subsequent regrowth in a different site is reported. As far as we know, this is the first reported case of regrowth of an AVM in a different location after radiosurgery in a child. This situation has to be considered within the context of causes of unsuccessful treatment of AVMs with radiosurgery and justifies angiographic monitoring of pediatric patients until they reach adulthood. Received: 27 October 1999     

Risk of seizure recurrence after a first unprovoked seizure in childhood

ObjectiveThe aim of this study was to assess the risk of recurrence after a first unprovoked seizure in childhood and to explore the correlation between the first and second seizures in recurrent patients.MethodsIn a prospective study, we included 467 children aged 1 month to 16 years, who were attended to between November 1, 2008 and October 31, 2016 following a first seizure. Children who had been started on treatment with antiepileptic drugs were excluded. Recurrence rates were calculated using Kaplan–Meier survival analyses. Univariate and multivariate analyses for recurrence risk were performed using the Cox proportional hazards model. The kappa coefficient of correlation for categorical data was calculated.ResultsRecurrences occurred in 280 children (60.0%), of which 75 (26.8%) occurred in the first month, 184 (65.7%) within 6 months, and 256 (91.4%) within 2 years. None of the patients had new neurologic sequelae after their first or second seizure. The estimates of seizure recurrence risk were 39.5%, 48.1%, 55.1%, 60.8%, 61.8% and 61.8% at 0.5, 1, 2, 5, 8, and 10 years after the first seizure, respectively. Multivariate analysis showed that abnormal electroencephalogram and neuroimaging findings significantly increased the risk of recurrence. First and second seizures were significantly associated with state of arousal, status epilepticus, and multiple seizures in recurrent patients.ConclusionOver half of untreated children had recurrence after a first unprovoked seizure, but prognosis was good overall.     

绿色荧光蛋白报道基因在鼠C6胶质瘤细胞中的应用研究

目的 :探讨绿色荧光蛋白 (GFP)报道基因转染C6鼠胶质瘤细胞的体内外表达及对细胞生物学性状的影响。方法 :荧光相差显微镜筛选稳定表达GFP的克隆 ,流式细胞术分析细胞周期。将已转染和未转染瘤细胞植入SD大鼠脑内 ,建立动物模型 ,定期随机处死大鼠 ,鼠脑标本行病理学、增殖与凋亡的检测 ,激光共聚焦显微镜检查肿瘤细胞内荧光强度及其分布。结果 :GFP在C6瘤细胞的体外和体内均获得长期稳定表达 ,检测肿瘤细胞的敏感性及特异性优于HE染色。结论 :GFP对肿瘤细胞生物学性状无影响是比较理想的报道基因