Tectal Glioma Research Articles

Neuropsychological outcome after endoscopic third ventriculostomy.

Cognitive dysfunction is a common complaint associated with obstructive hydrocephalus. The purpose of this study was to determine the effect of endoscopic third ventriculostomy (ETV) on the neuropsychological outcome in patients presenting with cognitive decline and obstructive hydrocephalus. A retrospective review of patients who underwent ETV at the University of Calgary and had both pre and post operative neuropsychological testing, was completed. Presenting clinical features, etiology of hydrocephalus and ventricle size utilizing frontal occipital horn ratio was obtained. Outcomes and complications of the ETV were recorded. Detailed measures of intelligence, attention and concentration, executive function, visual and verbal memory, language functions and fine motor skills were completed. Post treatment change was determined utilizing Reliable Change Index. A total of 13 patients were identified. Etiology of the hydrocephalus was aqueductal stenosis in 8 and tectal glioma in 4. The majority of patients (11 of 13, 85%) demonstrated cognitive dysfunction at the borderline (≤1 SD) or impairment level (≤1.5 SDs) in at least one domain. Nine patients (69%) showed reliable improvement in at least one cognitive domain. The greatest improvement was seen with visual memory (42%). One quarter to one third of patients demonstrated improvement on tests of intelligence quotient, verbal memory, attention and concentration, and executive function. Two patients declined in executive functioning. Ventricle size improved in eight patients. ETV is a safe effective procedure, capable of producing reliable improvements in cognitive dysfunction with hydrocephalus. Patients with cognitive complaints alone may benefit from ETV.

Pineal lesions: a multidisciplinary challenge.

The pineal region is a complex anatomical compartment, harbouring the pineal gland surrounded by the quadrigeminal plate and the confluents of the internal cerebral veins to form the vein of Galen. The complexity of lesions in that region, however, goes far beyond the pineal parenchyma proper. Originating in the pineal gland, there are not only benign cysts but also numerous different tumour types. In addition, lesions such as tectal gliomas, tentorial meningiomas and choroid plexus papillomas arise from the surrounding structures, occupying that regions. Furthermore, the area has an affinity for metastatic lesions. Vascular lesions complete the spectrum mainly as small tectal arteriovenous malformations or cavernous haemangiomas.Taken together, there is a wide spectrum of lesions, many unique to that region, which call for a multidisciplinary approach. The limited access and anatomical complexity have generated a spectrum of anatomical approaches and raised the interest for neuroendoscopic approaches. Equally complex is the spectrum of treatment modalities such as microsurgery as the main option but stereotactic radiosurgery as an alternative or adjuvant to surgery for selected cases, radiation as for germinoma (see below) and or combinatorial chemotherapy, which may need to precede any other ablative technique as constituents.In this context, we review the current literature and our own series to obtain a snapshot sentiment of how to approach pineal lesions, how to interrelate alternative/competing concepts and review the recent technological advances.

O8.07 * HISTOLOGY, LOCALISATION, TREATMENT AND OUTCOME OF 339 CONSECUTIVE CHILDREN AND ADOLESCENTS WITH LOW GRADE GLIOMAS TREATED AT THE MEDICAL UNIVERSITY OF VIENNA BETWEEN 1993 AND 2012

Low grade gliomas (LGG) comprise a spectrum of different tumor entities corresponding to WHO grade I and II, including pilocytic astrocytoma, diffuse glioma and glioneuronal tumors. LGG in children differ in histologic distribution, molecular profile, localisation in the CNS and outcome from LGGs in adults. We report on a series of patients treated at a single center over 20 years. PATIENTS: Between 1993 and 2012, 339 consecutive patients with a median age of 8 years (range 4 months - 18 years, p25 = 3.7 years, p75 = 13.4 years) were treated at the Medical University of Vienna. 19.5% of the patients were <3 years of age at diagnosis. Gender distribution was equal. Tumors were associated with syndromes in 84 of the patients (NF1: n = 64, TSC: n = 17, and other: n = 3). Localisation was supratentorial midline in 112, cerebral hemispheres in 105, posterior fossa in 86, ventricular system in 27 and spinal in 9 patients. 66 patients with a median age of 4.4 years had optic pathway gliomas. RESULTS: Gross total resection was performed in 109, subtotal resection in 49, partial resection in 57, and biopsy in 22 patients. 83 patients had no surgery (patients with NF1, TSC, tectal glioma or tumors confined to the optic nerves and chiasm), and in the remaining the degree of resection was not evaluable. 37 patients had 2 tumor surgeries, 14 three, 5 four and one had 5 tumor surgeries during their course of disease. Histology was pilocytic astrocytoma in 122, pilomyxoid astrocytoma in 10, diffuse astrocytoma in 42, ganglioglioma in 30, subependymal giant cell astrocytoma in 17, oligoastrocytoma in 11, and other rarer histologies in the remaining patients. Twelve patients (3.5%) died and 84 patients (24.8%) had at least one event. After a median follow-up of 107 months (14-300) the 1-year overall survival (OS) was 100%, the 10-year OS 96.1 ± 1.2%, and the 20-year OS 95.3 ± 1.4%. Event-free survival (EFS) after 1 year was 92.9 ± 1.4%, after 10 years 73.8 ± 2.6% and after 20 years 67.9 ± 3.4%. CONCLUSION: While long term survival of children and adolescents with low grade gliomas is better than of adults, recurrence and death may occur even in WHO grade I tumors and regular surveillance MRI is recommended.

Endoscopic third ventriculostomy for hydrocephalus due to tectal glioma.

Tectal gliomas commonly present with hydrocephalus from obstruction of the aqueduct of Sylvius. The creation of a ventriculostomy in the floor of the third ventricle (ETV) has been previously reported to by-pass aqueduct obstruction. The goal of this study was to determine the safety and efficacy of ETV in the presence of an obstructing tectal glioma. We retrospectively reviewed the clinical presentation, management, and clinical outcome after ETV in patients diagnosed with tectal glioma and obstructive hydrocephalus in our institution over a period of 15 years. Shunt freedom at follow-up was the main outcome variable. Long-term clinical outcome was assessed at the most recent clinic visit. Clinical outcome was ranked as excellent, good, or poor according to resolution of symptoms and patient functional status. The median age at presentation was 16.5 years (range: 6.4 to 59 years) and the most common presenting symptom was headache. Eleven patients had ETV as a primary procedure and three patients underwent ETV as a substitute for shunt revision at the time of shunt failure. At follow-up (median 3.9 years, range: 2.2 to 7 years) 13 of 14 patients remain shunt independent with excellent (n=9) or good outcomes (n=5). In patients with tectal glioma causing obstructive hydrocephalus, ETV can be performed safely in the primary setting or as a substitute for shunt revision. A high rate of shunt freedom (78%-100%) at prolonged follow-up can be expected in this patient population.

Tectal plate glioma: Lessons learned from a patient with hydrocephalus, Chiari malformation-1 and concomitant aqueductal stenosis

Chiari malformation type I (CM-1) is characterized by a reduced olume in the posterior fossa and by the caudal displacement of he cerebellar tonsils through the foramen magnum. CM-1 is usully congenital, although rarely may be acquired. Since its initial escription in 1891, many clinical symptoms of brainstem, cereellar, and spinal cord pathology have been attributed to this isease. Hydrocephalus, in particular, has been noted in approxmately 4–18% of patients with CM-1 [1]. The exact pathogenesis s unknown, but the association between hydrocephalus and CM-1 s believed to be multifactorial. The standard treatment for CMis decompressive suboccipital craniotomy. Though short-term mprovements have been reported in up to 75–90%, there is a ubset of as many as 35% of CM-1 patients whom experience sympom recurrence years after surgery [2]. This subset of patients may epresent a symptom etiology that is not localized to the hindrain decompression, but rather one that is related to a separate athology. We report a case of an adult patient with hydrocephalus nd CM-1 that had symptom recurrence after surgical decomression following years of uneventful follow up. The patient was

Tectal glioma with hydrocephalus presenting with spastic and ataxic quadriparesis.

Tectal glioma with hydrocephalus presenting with spastic and ataxic quadriparesis.

A Rare Cause of Hydrocephaly: Tectal Glioma

Tectal gliomas are a distinct form of pediatric brainstem tumor usually present with increased intracranial pressure. The tumor may arise from different origins and the most common histologic form is low-grade astrocytoma. We would like to remind and emphasize why it is important to report a tectal glioma in magnetic resonance imaging reports giving two case samples.

Complications and subsequent removal of retained shunt hardware after endoscopic third ventriculostomy: case series

This case series highlights multiple complications and subsequent removal of retained shunt hardware in pediatric patients after successful endoscopic third ventriculostomy (ETV). Removal or retention of existing shunt hardware following ETV represents an important dilemma. Prior studies have reported infections and organ perforation related to nonfunctioning shunts but none in the context of successful ETV. Data obtained in 3 children with hydrocephalus treated at the authors' institution were retrospectively reviewed after the patients experienced complications due to retained shunt hardware following ETV. Etiologies of hydrocephalus included tectal glioma and intraventricular hemorrhage. All 3 patients had a history of multiple shunt revisions and underwent urgent ETV in the setting of a shunt malfunction. In each case, the entire shunt system was left in situ, but it became the source of subsequent complications. Two of the 3 patients presented with the shunt infected by gram-negative bacilli 10 days and 4.5 months postoperatively, respectively. The remaining patient experienced wound dehiscence over the shunt valve 4.5 months after ETV. In all patients, the complications were managed successfully by removing the shunt hardware. None of the patients required repeat shunt insertion from the time of removal throughout the follow-up period (mean 24 months, range 9-36 months). During the study period, a total of 6 patients with indwelling shunt hardware underwent ETV with the expectation of being shunt independent. Among these 6 patients, 3 experienced no complications from the retained hardware whereas 3 patients (50%) ultimately experienced adverse consequences related to retained hardware. This case series illustrates complications involving retained shunt hardware after successful ETV. These examples support consideration of shunt removal at the time of ETV in the appropriate context.

Brainstem Glioma: A Review

Brainstem gliomas (BGs) are a heterogenous group of gliomas that occur predominately in children. They can be separated into groups on the basis of anatomy and clinical behavior: diffuse intrinsic pontine glioma (DIPG), exophytic medullary glioma, and tectal glioma. DIPG is the commonest BG. Median age at onset is 6.5years and median survival is less than 1year. Adults with DIPG survive longer, suggesting a less aggressive and biologically different tumor from that in children. Patients present with cranial nerve dysfunction, long tract signs, or ataxia, either in isolation or in combination. Magnetic resonance imaging shows an infiltrative lesion occupying most of the pons and contrast enhancement is usually not prominent. Standard treatment is fractionated radiotherapy. Platelet-derived growth factor receptor alpha and epidermal growth factor receptor mutations have been identified. Inhibitors of these growth factor receptors are being evaluated in clinical trials. Exophytic medullary and tectal gliomas are relatively indolent tumors that can often be followed closely without treatment.

Hyponatremia following endoscopic third ventriculostomy: a report of 5 cases and analysis of risk factors

Electrolyte and endocrinological complications of endoscopic third ventriculostomy (ETV) are infrequent but serious events, likely due to transient hypothalamic-pituitary dysfunction. While the incidence of diabetes insipidus is relatively well known, hyponatremia is not often reported. The authors report on a series of 5 patients with post-ETV hyponatremia. The records of patients undergoing ETV between 2008 and 2010 were reviewed. All ETVs were performed with a rigid neuroendoscope via a frontal bur hole, standard third ventricle floor blunt perforation, Fogarty catheter dilation, and intermittent normal saline irrigation. Postoperative MR images were evaluated for endoscope tract injury as well as the trajectory from the bur hole center to the fenestration site. Thirty-two patients (20 male and 12 female) underwent ETV. Their median age was 6 years (range 3 weeks-28 years). Hydrocephalus was most commonly due to nontumoral aqueductal stenosis (43%), nontectal tumor (25%), or tectal glioma (13%). Five patients (16%) had multicystic/loculated hydrocephalus. Five patients (16%) developed hyponatremia between 1 and 8 days following ETV, including 2 patients with seizures (1 of whom was still hospitalized at the time of the seizure and 1 of whom was readmitted as a result of the seizure) and 3 patients who were readmitted because of decline in their condition following routine discharge. No hypothalamic injuries were noted on imaging. Univariate risk factors consisted of age of 2 years or less (p = 0.02), presence of cystic lesions (p = 0.02), and ETV trajectory angle 10° or more from perpendicular (p = 0.001). Endoscopic third ventriculostomy is a well-tolerated procedure but can result in serious complications. Hyponatremia is rare and may be more likely in younger patients or those with cystic loculations. Patients with altered craniometry may be at particular risk with a rigid endoscopic approach requiring greater manipulation of subforniceal or hypothalamic structures.

Adult Brainstem Gliomas

Brainstem gliomas are uncommon in adults and account for only 1%-2% of intracranial gliomas. They represent a heterogeneous group of tumors that differ from those found in their pediatric counterparts. In adults, a low-grade phenotype predominates, which is a feature that likely explains their better prognosis compared to that in children. Because biopsies are rarely performed, classifications based on the radiological aspect of magnetic resonance imaging results have been proposed to establish treatment strategies and to determine outcomes: (a) diffuse intrinsic low-grade, (b) enhancing malignant glioma, (c) focal tectal gliomas, and (d) exophytic gliomas. Despite significant advances in neuroradiology techniques, a purely radiological classification remains imperfect in the absence of a histological diagnosis. Whereas a biopsy may often be reasonably avoided in the diffuse nonenhancing forms, obtaining histological proof seems necessary in many contrast-enhanced brainstem lesions because of the wide variety of differential diagnoses in adults. Conventional radiotherapy is the standard treatment for diffuse intrinsic low-grade brainstem gliomas in adults (the median survival is 5 years). In malignant brainstem gliomas, radiotherapy is the standard treatment. However, the possible benefit of combined radiotherapy and chemotherapy (temozolomide or other agents) has not been thoroughly evaluated in adults. The role of anti-angiogenic therapies in brainstem gliomas remains to be defined. A better understanding of the biology of these tumors is of primary importance for identifying homogeneous subgroups and for improving therapy options and outcomes.

Blinking—an unusual presentation of obstructive hydrocephalus relieved by endoscopic third ventriculostomy

A 9-year-old girl presented with symptoms and signs of raised intracranial pressure over a period of 10 days. In addition, she had persistent fast blinking. The onset of this persistent blinking corresponded with the onset of symptoms of raised intracranial pressure. On examination, her corrected visual acuity was normal; however, bilateral papilloedema was present. There was no upgaze palsy. An urgent computerised tomographic (CT) scan identified dilatation of the lateral and third ventricles with associated periventricular lucency. Magnetic resonance imaging (MRI) of the brain revealed obstructive hydrocephalus due to a lesion of the tectal plate. (Figure 1a and b). The radiological features of the tectal plate lesion were consistent with a tectal plate glioma. The hydrocephalus was treated by endoscopic third ventriculostomy. Following this procedure, there was immediate resolution of her headaches and the persistent blinking. The papilloedema resolved over the next 4 weeks. Follow-up MRI brain showed the ventricles had decompressed and the periventricular lucency had resolved (Fig. 2a and b). Discussion

Incidental findings of mass lesions on neuroimages in children

Increasing use of neuroimaging in children has led to more incidental findings of CNS mass lesions, the management of which is uncertain. The authors' aims in this study are to describe these mass lesions and their evolution, as well as to discuss the management options and determine the prevalence of incidental CNS mass lesions at their pediatric clinic. A retrospective study was undertaken in children with primary CNS tumors who were younger than 18 years old and were admitted to the University Children's Hospital of Zurich, Switzerland, between January 1995 and December 2010. In 19 (5.7%) of 335 patients with newly diagnosed CNS tumors, the diagnosis of a CNS mass lesion was an incidental finding. Reasons for obtaining neuroimages in these 19 patients were head trauma (in 6 patients); research protocols (in 3); nasal/orbital malformations (in 2); endocrinological and psychiatric evaluations (in 2); and vertebral bone anomaly without neurological signs, absence seizures, congenital ataxia, recurrent vomiting, developmental delay, and "check-up" at the explicit request of the parents (in 1 patient each). Seven patients underwent immediate surgery for low-grade glioma (4 patients) and craniopharyngioma, ependymoma, and choroid plexus papilloma (1 patient each); and 12 were treated conservatively or were observed. Ten of 12 conservatively treated patients remained stable (median follow-up time 1.8 years) and the other 2 underwent delayed surgery because of tumor progression (medulloblastoma in one patient and fibrillary astrocytoma in the other). Clinicians are increasingly challenged by the discovery of incidental CNS mass lesions. A subgroup of such lesions (with typical imaging patterns such as tectal glioma and dysembryoplastic neuroepithelial tumor) can be monitored conservatively, clinically, and radiographically. Future prospective studies are needed to define optimal management strategies based on larger collections of natural histories, as well as to assess the true prevalence of incidental CNS mass lesions.

Papillary tumor of the pineal region in a 15-month-old boy

The papillary tumor of the pineal region (PTPR) is a distinct entity that is particularly rare in the pediatric population. The authors document the youngest reported patient with this clinicopathological entity to date. A case of PTPR in a 15-month-old boy is described. Initially thought to be a tectal glioma, the tumor was later identified as a pineal region tumor after demonstrating growth on routine imaging. Diagnosis of PTPR was established by histopathological evaluation of biopsy samples, which revealed papillary, cystic, and solid tumor components. The patient's postoperative course was complicated by tumor growth despite several debulking procedures and chemotherapy, as well as persistent hydrocephalus requiring 2 endoscopic third ventriculostomies and eventual ventriculoperitoneal shunt placement. After a 15-month follow-up period, the patient has received proton-beam therapy and has a stable tumor size. The PTPR is a recently described tumor of the CNS that must be included in the differential diagnosis of pineal region masses. The biological behavior, prognosis, and appropriate treatment of PTPR have yet to be fully defined.

Successful treatment of tremor by endoscopic third ventriculostomy in an adolescent with obstructive hydrocephalus due to tectal glioma: case report

Hydrocephalus presenting with movement disorder is very rare, especially in children. We present the case of a 16-year-old boy who presented with bilateral intention tremor and slowed speech as a result of obstructive hydrocephalus secondary to a tectal glioma. Treatment with endoscopic third ventriculostomy improved his symptoms. We review the literature regarding this unusual presentation of an otherwise common condition.

Utility of ventricular access in an acute deterioration after endoscopic third ventriculostomy

Endoscopic third ventriculostomy (ETV) has now been accepted widely as a safe procedure for treatment of non-communicating hydrocephalus. Despite its learning curve, most of the neurosurgeons have understood its technical details, benefits, and risks and have started to practice it to perfection. The benefit of shunt independence with minimal risks offers a remarkable advantage which has made this procedure widely popular. However, late closure of stoma leading to morbidity and even death has been reported off late. We report a case of a 7-year-old girl with hydrocephalus due to tectal glioma who deteriorated after 7 months following a successful procedure. She developed a cardio-respiratory arrest and was resuscitated with aspiration of cerebrospinal fluid from the ommaya reservoir kept during the primary surgery. Keeping all the patients under strict surveillance for stoma patency is mandatory and in addition, ommaya reservoir in certain high-risk patients may be a useful option for achieving quick ventricular access by medical and nonmedical personnel in case of deterioration. This case is the first reported case of acute deterioration after ETV from India. Previously, 14 such cases have been reported worldwide and only 2 of them have survived.

Management of tectal gliomas:review of twelve cases

Objective To explore the management strategy of tectal gliomas. Method From 2003 to 2007, twelve cases of tectal gliomas were treated in our department. We retrospectively analyzed their clinical characteristics,imaging features and pathologies, and reported the results after a mean follow- up of fifty - one months. Results Three tumors were completely removed, nine were partially removed. Eleven tumors were low grade gliomas,one was a high grade glioma. One patient died after the surgery, one patient died of tumor progression, and other ten patients were stable without tumor progression during the follow - up study. Conclusions As most tectal gliomas have an indolent nature in clinical course,we recommend for patients without hydrocephalus or with relieved hydrocephalus, a long - term MRI follow - up should be conducted ;for patients with worsening neurological deficits or with a progressing tumor(larger than 2 cm in diameter or with significant enhancement),a surgery should be performed. Key words: Brain stem neoplasms; Glioma; Therapy; Follow - up

Mitochondrial ND5 mutations mimicking brainstem tectal glioma

We report MRI periaqueductal T2 hypersignal suggestive of tectal glioma in 3 unrelated children with reduced vision and normal mental development (figure). Increased CSF lactate and optic atrophy in the first case suggested …

Cerebrospinal fluid rhinorrhea after ventriculoperitoneal shunt in a patient with tectal plate glioma

Cerebrospinal fluid (CSF) rhinorrhea due to a remote tumor is a rare but increasingly reported condition, where it is usually a presenting complaint. CSF rhinorrhea occurring after tumor decompression has also been reported. We report a patient with tectal plate glioma that caused CSF rhinorrhea following insertion of ventriculoperitoneal shunt. The pathophysiology of CSF rhinorrhea is also discussed.

6‐YEAR‐OLD GIRL WITH HYDROCEPHALUS

With rare exceptions, pediatric tectal gliomas have been generally reported as low-grade tumors with relatively good prognosis. The patients are usually treated conservatively to manage the signs and symptoms of obstructive hydrocephalus. We report a case of a tectal glioma in a 6-years-old girl with histological features of anaplastic mixed oligoastrocytoma that continues to progress despite chemotherapy and radiation therapy.