Diagnosis and Therapeutic Management of Ventricular Gangliogliomas: An Illustrated Review.

BACKGROUND: Gangliogliomas (GGs) are extremely rare benign neoplasms frequently located within the temporal lobe that usually present with seizures. GGs growing predominantly within the ventricular system (VGGs) are even more infrequent, so definite conclusions concerning their diagnosis and therapeutic management are lacking. METHODS: A retrospective review of case reports of VGGs was performed from the introduction of modern imaging techniques, including 4 new illustrative cases treated in our department. RESULTS: Thirty-four cases were collected. Ages ranged from 10 to 71 years (mean, 26.62 years), and 55.9% were male. Most patients developed symptoms related to high intracranial pressure. The lateral ventricles were predominantly involved (58.8%). Obstructive hydrocephalus was observed in 54.5% of patients. Cystic degeneration and calcification were frequently observed. Surgical treatment was carried out in all cases. Morbidity and mortality were 17.6% and 2.9%, respectively. Gross total tumor resection was achieved in 64.5% of patients. Four patients experienced tumor dissemination along the neural axis. More than 90% of patients maintained a good functional status at last follow-up. CONCLUSIONS: Despite their low incidence, a diagnosis of VGGs should be considered in young male adults who progressively develop intracranial hypertension, caused by a ventricular mass showing signs of cystic degeneration and calcification. Maximal and safe surgical resection represents the gold standard for the treatment of symptomatic VGGs, although total removal is frequently precluded by difficulties in defining appropriate tumor boundaries. Adjuvant radiotherapy should be considered if an incomplete resection was carried out, especially in World Health Organization grade III neoplasms.

Transcallosal approach for intraventricular gliomas: neuropsychological outcome and functionality of the corpus callosum.

The transcallosal approach is commonly used for surgery of lateral and third ventricle lesions. Cognitive deficits due to the transcallosal approach still remain controversial. Even if enormous efforts have been made in order to understand specific functions of the corpus callosum, still little is known. The present study was aimed to evaluate the neuropsychological results and the functionality of the corpus callosum in transferring visual, auditory and tactile information between the two hemispheres in a group of patients who were treated using the transcallosal approach. The study evaluated the neuropsychological status of five selected patients presenting low-grade lesions of lateral ventricles that had not previously undergone surgical treatments and that did not receive radiotherapy and chemotherapy. All patients were administered an extensive neuropsychological testing postoperatively and the interhemispheric transfer of visual, auditory and tactile information was also evaluated. Two patients were tested preoperatively. Incisions of 2.4 cm maximum of the corpus callosum length were operated. The postoperative cognitive profile was normal. In some patients, a postoperative subnormal performance in memory functions was found but it cannot be attributable to the surgical approach given that it was altered even pre-surgically. Small incisions of the corpus callosum preserved the integrity of this anatomical structure in transferring lateralized information between the two hemispheres. The transcallosal approach is a safe surgical route to lateral ventricles lesions and the neuropsychological evaluation of these cases could give new insights in the comprehension of corpus callosum functions.

Hypothalamic relapse of a cardiac large B-cell lymphoma presenting with memory loss, confabulation, alexia-agraphia, apathy, hypersomnia, appetite disturbances and diabetes insipidus.

A 37-year-old Hispanic man with a right atrial intracardiac mass diagnosed as diffuse large B-cell lymphoma (DLBCL) was successfully treated with surgery and chemotherapy. During 4 years, several total-body positron emission tomography and MRI scans showed no extracardiac lymphoma. On year 5 after the cardiac surgery, patient presented with sleepiness, hyperphagia, memory loss, confabulation, dementia and diabetes insipidus. Brain MRI showed a single hypothalamic recurrence of the original lymphoma that responded to high-dose methotrexate treatment. Correction of diabetes insipidus improved alertness but amnesia and cognitive deficits persisted, including incapacity to read and write. This case illustrates two unusual locations of DLBCL: primary cardiac lymphoma and hypothalamus. We emphasise the importance of third ventricle tumours as causing amnesia, confabulation, behavioural changes, alexia-agraphia, endocrine disorders and alterations of the circadian rhythm of wakefulness-sleep secondary to lesions of specific hypothalamic nuclei and disruption of hypothalamic-thalamic circuits.

Clinical, radiological, pathological and prognostic aspects of intraventricular oligodendroglioma: comparison with central neurocytoma.

Studies comparing intraventricular oligodendroglioma (IVO) and central neurocytoma (CN) in terms of their clinical, radiological and pathological features are scarce. We, therefore, investigated the similarities and differences between these types of tumors to get a better understanding of how they may be more properly diagnosed and treated. The clinical manifestations, CT/MRI findings, pathological characteristics and clinical outcomes of 8 cases of IVOs and 12 cases of CNs were analyzed retrospectively. Both IVO and CN occurred most commonly in young adults and manifested with symptoms of increased intracranial pressure secondary to obstructive hydrocephalus. However, they were radiologically different in location (p = 0.007), diffusion-weighted imaging (p = 0.001), "scalloping" appearance (p = 0.006), flow void sign (p = 0.006) and ventricular wall invasion (p = 0.000). Histologically, significant differences in mitotic count (p = 0.008) and parenchymal infiltration (p = 0.01) were noted. Immunohistochemically, significant differences in the expression of Olig2 (p = 0.000), Syn (p = 0.01) and NeuN (p = 0.000) were observed. In addition, MIB-1 labeling index (p = 0.035) and case fatality rate (p = 0.021) of IVO were much higher than those of CN, while survival rate of IVO was much lower than that of CN (p = 0.028). IVO and CN are similar in onset age and clinical manifestations, but have different imaging and pathological features. Patients with IVOs may have a relatively poorer prognosis compared to those with CNs.

Use of mTOR inhibitor everolimus in three neonates for treatment of tumors associated with tuberous sclerosis complex.

BACKGROUND: Tuberous sclerosis complex is characterized by the growth of benign tumors in multiple organs, caused by the disinhibition of the mammalian target of rapamycin (mTOR) protein. mTOR inhibitors, such as everolimus, are used in patients with tuberous sclerosis complex, mainly to reduce the size of renal angiomyolipomas and subependymal giant cell astrocytomas. There are minimal data available regarding its use during the neonatal period. METHODS: We report clinical and pharmacological data of three neonates treated with the mTOR inhibitor everolimus (two hemodynamically significant cardiac rhabdomyomas and one voluminous subependymal giant cell astrocytoma). RESULTS: Beneficial clinical responses were observed in all three patients and the medication was generally well-tolerated. Optimal dose was 0.1 mg orally once daily and was confirmed with therapeutic drug monitoring. CONCLUSION: Everolimus is a promising pharmacological approach to treat clinically significant inoperable cardiac rhabdomyomas or subependymal giant cell astrocytoma associated with tuberous sclerosis complex during the neonatal period.

'Goose bumps' as presenting feature of intraventricular glioblastoma multiforme.

'Goose-bumps' seizures are rare manifestations of epilepsy. They are rarely reported by patients and can be easily dismissed by clinicians. Clinically, it carries some diagnostic localising value especially with unilateral onset. In this report, we present a case of intraventricular glioblastoma multiforme with ipsilateral goose bumps and review the literature.

[Identification of highly specific electroencephalographic patterns in patients with neuroepithelial tumors of the III ventricle by algorithmic methods].

An algorithm was suggested for identifying highly specific electroencephalographic (EEG) patterns in neurooncologic patients. The algorithm provides selection of patients with their further classification into main and control groups based on the already existing database of EEG indicators; requests to it; generation of mono-indicator candidates for EEG-patterns on the basis of a 4-dipole table for selecting and verifying sensitive and specific EEG patterns and outlining the best ones. Our material included 368 patients with basal-diencephalic tumors. Algorithmic methods revealed new EEG patterns in patients with different anatomical and topographical variants of neuroepithelial tumors in the III ventricle. We think it reasonable to use the revealed syndromes to improve diagnosis and identify pathophysiological basis of clinical syndromes.

Secondary normal pressure hydrocephalus in a patient with isolated frontal dilatation--an insight into pathophysiology?

Current theories of the pathophysiology of normal pressure hydrocephalus suggest the classical symptoms are a consequence of disruption of normal frontal function. We present the case of a 70-year-old patient with an isolated, frontal dilatation of his lateral ventricles in the presence of a complete triad as supportive of these theories.

Disorders affecting the fourth ventricle: etiology and clinical correlates.

OBJECTIVE: The fourth ventricle encompasses many vital structures including the brainstem as its floor and the cerebellum as its lateral wall and roof. Therefore, lesions affecting the fourth ventricle may present as cerebellar or brainstem manifestations. Herein, we presented our experience in the diagnosis of disorders affecting the fourth ventricle during the past 15 years. STUDY DESIGN: Retrospective study. SETTING: University hospital. SUBJECTS: From September 1995 to August 2010, 24,838 patients with vertigo/dizziness visited the clinic. Of them, 13 patients (0.5%) had tumor/stroke affecting the fourth ventricle. MAIN OUTCOME MEASURES: All patients underwent a battery of audiovestibular function test. RESULTS: All patients had vertigo (100%) and then ataxia (92%) and nausea/vomiting (85%). Eight patients (68%) displayed persistent nystagmus. Most (>75%) patients showed central signs in electronystagmographic recordings and abnormal caloric coupled with visual suppression test. Three patients underwent both ocular and cervical vestibular-evoked myogenic potential tests, and abnormal results were noted in all 3 patients indicating a brainstem involvement. However, magnetic resonance imaging failed to demonstrate brainstem lesion in these 3 patients. Final diagnoses consisted of the following: cavernoma, n = 3; metastatic cancer, n = 3; astrocytoma, n = 2; meningioma, n = 2; epidermoid cyst, n = 1; ependymoma, n = 1; and lymphoma, n = 1. The prognosis was poor as 10 patients (77%) died within 2 years. CONCLUSION: When a vertiginous patient displayed ataxia, persistent vomiting, and persistent nystagmus, lesion affecting the fourth ventricle should be kept in mind because its prognosis is poor. Abnormal results in electronystagmography and in ocular and cervical vestibular-evoked myogenic potentials may serve as a supplementary to magnetic resonance imaging to delineate the involvement of the lesion.

An unusual presentation of colloid cyst--implications for lifestyle advice.

Colloid cysts are rare intracranial neoplasms which typically present with headaches. There is risk of neurological deterioration or death due to acute hydrocephalus. We report a case of colloid cyst presenting after a sudden acceleration/deceleration force from a theme park ride, highlighting the importance of lifestyle advice in these patients.

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.

[Three pediatric cases with choroid plexus papilloma in the fourth ventricle].

Choroid plexus papillomas are rare; they comprise less than 1% of all intracranial tumors. In children, most of these neoplasms arise in the lateral ventricle while in adults they are primarily located in the fourth ventricle. We report 3 children with choroid plexus papilloma in the fourth ventricle; they were one 5-month-old girl and 2 boys aged 8-and 15-years. The baby girl presented with macrocephaly and signs of raised intracranial pressure, the 2 boys exhibited cerebellar signs. On magnetic resonance imaging (MRI) the tumors were well-enhanced with a cauliflower-like contour and hydrocephalus. In one case, diffusion weighted images (DWIs) showed an isointense tumor signal clearly different from the well-known hyperintensity of medulloblastomas, Via suboccipital craniotomy we succeeded in the total or subtotal surgical resection of these tumors and there were no sequela. None of the tumors have recurred in the intervening 2-9 years. In conclusion, choroid plexus papilloma should be included as a differential diagnosis in children presenting with tumors in the fourth ventricle. The contour of the tumor on MRI and its intensity on DWI may be clues for a correct preoperative diagnosis.