Tectal Plate Gliomas Research Articles

Pre-op Deaf Post-op Dumb: A Case Report of a Tectal Plate Glioma and a Brief Literature Review

Any disorder of the brain stem that involves the auditory pathways and/or centers may produce hearing abnormalities. Auditory pathways are tonotopically organized, and they cross the midline at several points both below and at the level of the inferior colliculus but the central nucleus of the inferior colliculus and higher centers have a clear contralateral dominance. Ipsilateral auditory abnormalities are caused by lesions below or within the cochlear nuclei; bilateral auditory abnormalities are caused by lesions rostral to cochlear nuclei or they may be silent. A patient with a tectal plate glioma can present with bilateral hearing impairment due to the involvement of both inferior colliculi. Our case is a 28-year-old male who presented with progressive bilateral hearing impairment and a generalized headache despite normal pure-tone audiometry. Computed tomography (CT) of the brain revealed a tectal plate glioma (TPG) extending into the right thalamus, and an obstructive hydrocephalus. Following a ventriculoperitoneal shunt for cerebrospinal fluid (CSF) diversion, navigation-guided tumour excision was performed via a supracerebellar infratentorial approach. Postoperatively he developed unexpected aphasia probably due to ischemic changes in the cerebellar vermis and a focus of the adjacent right cerebellar hemisphere. This case highlights the rarity of bilateral auditory impairment in a TPG, surgical challenges posed by the tumour’s location, and potentially rare post-op complication of cerebellar mutism syndrome.

Trigeminal Neuralgia and Associated Hydrocephalus: Not a Mere Incidental Finding? A Case Report and Literature Review

BACKGROUND: Trigeminal neuralgia (TN) is most commonly related to vascular compression of the trigeminal root entry zone into the brainstem. Secondary causes of compression are much less common. TN as a sole manifestation of hydrocephalus has rarely been reported in the literature. CASE REPORT: We describe a young woman with medically resistant TN, who was discovered to have a tectal plate glioma causing secondary obstructive hydrocephalus. In addition, she had an incidental finding of ecchordosis physaliphora. Cerebrospinal fluid diversion by an endoscopic third ventriculostomy resulted in TN resolution. CONCLUSION: Hydrocephalus is a rare diagnosis to consider as a secondary cause for TN. Since the literature on this is sparse, it can be only suggested that in very select cases, treating the hydrocephalus may also treat the TN. Additional studies and reports are required. KEY WORDS: Trigeminal neuralgia, hydrocephalus, ecchordosis physaliphora, tectal plate glioma

Tectal Plate Glioma: A Clinical and Radiologic Analysis of Progression and Management in Adults

BackgroundTectal Plate Gliomas (TPG) are a heterogeneous group of uncommon brain tumours. TPGs are considered indolent and are usually managed conservatively but they have the potential to transform into higher grade tumours. The aims of this study were to investigate the natural history of adult TPG, treatment outcomes, and overall survival. MethodsThis was a retrospective cohort analysis of adult patients with TPG between 1993 and 2021. Baseline clinical, radiological and management characteristics were collected. The primary outcome was tumour progression, defined as increasing size on radiological assessment or new gadolinium contrast enhancement. Secondary outcomes included: management and mortality. ResultsThirty-nine patients were included, of which 23 (52.2%) were males. Median age at diagnosis was 35 years (Interquartile Range [IQR] 27-53). Radiological tumour progression was observed in eight (20.5%) patients. The 10-year PFS rate was 72.6% (95% Confidence Interval [CI] 0.58-0.91). The 10-year OS rate was 86.5% (95% CI 0.75-1.0). CSF diversion procedures were used in 62% of the cohort (n=24). Seventeen (43.6%) patients underwent at least one Endoscopic Third Ventriculostomy, while only six (15.4%) patients underwent at least one Ventriculoperitoneal Shunt. ConclusionsTPG has an overall favourable clinical prognosis, although progression occurs in 1 in 5 patients. Demonstrating accurate factors by which TPG patients may be risk stratified should be a key area of further research. A follow up duration of 10 years would be a reasonable window based on the radiological progression rates in this study however, larger cohort studies are needed to answer both questions definitively.

Predicting disease progression and the need for tumor-directed treatment in tectal plate gliomas.

Tectal plate gliomas are rare, slow-growing tumors of the midbrain that are discovered predominantly in the pediatric population. Because of their indolent nature, treatment mainly consists of observation and management of hydrocephalus. Unfortunately, a subset of tectal gliomas may exhibit tumor enlargement and disease progression. Currently, there are no established guidelines for predicting future progression of tectal gliomas or the need for tumor-directed treatment. In this paper, the authors present a large case series of tectal plate gliomas with the aim of determining early indicators of tumor progression and the need for tumor-directed treatment in a pediatric population, along with providing their experience in treating progressive tumors. A retrospective chart review of 170 patients diagnosed with tectal plate glioma from a single institution, of whom 67 were pediatric patients (≤ 18 years of age), was performed. Univariate analysis was used to determine statistically significant predictors of symptomatic disease progression requiring eventual tumor-directed therapy. The median patient age of the full cohort was 24 years (range 0-73 years). Compared with the pediatric population, the adult population had more instances of incidental lesions (p < 0.001) and lower rates of hydrocephalus (50% vs 84%, p < 0.001). Of the pediatric patients who had ≥ 5 years of follow-up (n = 51), 12 (24%) experienced radiological progression and 13 (25%) required treatment for their tumor. The 1-year, 5-year, and 10-year radiographic progression-free survival (PFS) rates were 98%, 90%, and 86%, respectively. In univariate analysis, lesion involvement of the pons, moderate T1 hypointensity, and moderate contrast enhancement on baseline radiology were significantly associated with worse radiographic PFS. Alternatively, significant predictors of requiring tumor-directed treatment included extraocular eye movement abnormalities at presentation, involvement of the lesion beyond the tectum on baseline radiology, moderate T1 hypointensity, moderate contrast enhancement, and an increase in total lesion size during progression. At the most recent follow-up, 94% of the patients had stable/nonprogressive disease, 2% had progressive disease, and 4% died of tumor progression. Patients who demonstrate radiographic progression may not necessarily experience clinical/symptomatic progression or require tumor-directed treatment. Certain patient presentation characteristics and baseline radiographic features may be predictive of worse radiographic PFS or the need for future tumor-directed treatment in the pediatric population. Typically, the natural history of these lesions lends to excellent long-term survival, even in patients who experience clinical progression, should appropriate treatment be initiated.

342 Predicting Early Disease Progression in Tectal Plate Gliomas

INTRODUCTION: Tectal gliomas are slow-growing tumors of the midbrain. Due to their indolent nature, treatment mainly consists of observation and management of hydrocephalus. However, a subset of tectal gliomas may exhibit tumor enlargement and disease progression. Currently, there are no established guidelines for predicting future progression of tectal gliomas. Further, given the rarity of these tumors, studies in the literature are limited to &lt;50 patients. METHODS: We performed a retrospective chart review of 172 patients diagnosed with tectal glioma. In addition to descriptive statistics, we performed univariate analysis using Student’s t-test and Chi-squared testing to determine if significant differences exist between patients with tumor progression versus those without progression. RESULTS: The median patient age was 24 years (range: 0-73 years); of these, 69 were pediatric (≤18 years). Hydrocephalus was present in 64% of cases. Lesions were incidentally detected in 37% of the cohort and were only present in the adult population. Of the patients that had &gt; 1 year follow-up (n = 145), 26 experienced radiological progression (18%) with 19 requiring tumor-directed treatment (13%). Predictors of progression included younger age at diagnosis (p = 0.005), expansion of the lesion beyond the tectum on initial imaging (p &lt; 0.001), moderate T1 signal (p &lt; 0.001), and mild or moderate contrast enhancement (p = 0.011 and p &lt; 0.001, respectively). At most recent follow-up, 92% had stable disease, 3% had progressive disease, 1% died from tumor progression, and 3% died of other causes. CONCLUSIONS: Certain characteristics of radiographic imaging may be predictive of future disease progression. In patients with progression, excellent survival can be achieved if progression is detected early and appropriate treatment is initiated.

Results of the treatment of pineal tumors in children: the Lyon experience.

Pineal tumors are rare and their incidence is of 1% among all pediatric tumors of the central nervoussystem. Patient survival depends on the histology, the extension of the surgical removal, and the efficacy of thecomplementary treatment (chemotherapy and cranio-spinal irradiation), as well as the age of the patient. In this study, we analyzed 151 pediatric patients treated for pineal tumors from 1997 to2020 in Lyon, France. All patients were recorded in the French Register of Pineal tumors, which has beencentralized and maintained in Lyon since 2010. Our analysis shows that benign tumors have an overall positive prognosis with total surgical removal.Concerning pineal parenchymal tumors, pinealoblastomas have a poor prognosis , especially in children less thanthree years old. A new pathological classification system allows for a better stratification of patient risk withindifferent groups of patients with pineal tumors. It is also important to note that the identification of DICER 1syndrome in families with pinealoblastomas warrant further medical investigation. Patients with Germ Cell Tumors have more favorable outcomes, with a global survival rate of 87 % and a puregerminoma survival rate of almost 97%. When analyzing the prognosis of pineal gland gliomas, otherwise knownas tectal plate gliomas, pilocytic astrocytomas had a promising prognosis. Otherwise, prognosis of other tectal plategliomas are related to the grade of malignancy and the efficacy of complementary treatment. Lastly, papillarytumors need a complete removal for the best chance of survival, and Atypical teratoid/ rabdoid tumors (AT/TR)still have a bad prognosis, regardless of surgical resection. Our results show that, with regards to pediatric pineal region tumors, there are still areas inprognostic indicators that need to be improved. Similarly, these pathologies need to be treated via amultidisciplinary approach to improve a patient's survival rate and their quality of life.

Tectal Plate Gliomas Masquerading as Idiopathic Aqueduct Stenosis

Aqueduct stenosis is a recognized cause of obstructive hydrocephalus in children and can be treated effectively with endoscopic third ventriculostomy. Preoperative magnetic resonance imaging is often diagnostic of the cause of aqueduct stenosis. We describe 2 pediatric cases with obstructive hydrocephalus secondary to a working diagnosis of idiopathic aqueduct stenosis. Following successful endoscopic third ventriculostomy, repeat magnetic resonance brain imaging revealed tectal plate glioma as the primary cause of obstruction. We believe these 2 reported cases demonstrate a previously unreported phenomenon whereby concealed tectal gliomas presenting with hydrocephalus are only unmasked following relief of hydrocephalus and decompression and normalization of the ventricular system. We aim to raise awareness about this unusual phenomenon and recommend routine postoperative interval imaging following endoscopic third ventriculostomy to avoid missing underlying pathology masquerading as aqueduct stenosis.

Long-Term Outcomes of Patients with Hydrocephalus Secondary to Tectal Plate Glioma versus Idiopathic Aqueductal Stenosis: Results from a Single Center

Introduction: Tectal plate gliomas (TPG) constitute a distinct entity of benign tumors of the brain stem which show an indolent clinical course. Adequate treatment of secondary hydrocephalus is undoubtedly a major factor in the outcome. However, little is known about to what degree the tumor itself determines the long-term outcome of these patients. Methods: We retrospectively analyzed and compared the clinical and radiological data of 16 pediatric TPG patients with data of 12 pediatric idiopathic aqueductal stenosis (IAS) patients treated in our center from 1988 to 2018. For both groups, we assessed the long-term outcome in terms of hydrocephalus management, and for the TPG group, we assessed tumor growth during follow-up. In a separate prospective part of the study, we performed a neuropsychological evaluation in a subgroup of patients using a standardized testing battery, covering intelligence, learning, memory, executive functions, and an inventory on depression. Results: In the TPG group, the mean clinical and radiological follow-up was 84 and 70 months, respectively. On average, the maximum diameter of the tumor increased by 11% (p = 0.031) and the estimated tumor volume with 35% (p = 0.026) on radiological follow-up. The fronto-occipital horn ratio (FOHR) decreased by 23% on average after treatment. In the IAS group, the mean clinical and radiological follow-up was 117 and 85 months, respectively. In this group, the FOHR decreased by 21% on average. Neurocognitive testing revealed significant higher scores in the TPG group on global intelligence (TPG = 109, IAS = 85.5, U = 3, p < 0.01, z = −2.71), performance (TPG= 100, IAS = 85, U = 7, p = 0.03, z = −2.2), and verbal intelligence (TPG = 122, IAS = 91.5, U = 2, p < 0.00, z = −2.87) as well as working memory (TPG = 109.5, IAS = 77, U = 0.5, p = 0.01, z = −2.46). Conclusion: Our results suggest that the long-term outcome in TPG patients is acceptable and that cognition is substantially better preserved than in patients with IAS. This puts the idea of a significant contribution of the tumoral mass to disease outcome on the long term in question. Adequate and prompt management of hydrocephalus is the most important factor in long-term cognitive outcome.

Imaging and differential diagnosis of tectal plate gliomas

Imaging and differential diagnosis of tectal plate gliomas

Pediatric tectal plate gliomas: a review of clinical outcomes, endocrinopathies, and neuropsychological sequelae.

Pediatric tectal plate gliomas are indolent slow-growing gliomas that often present with increased intracranial pressure or incidentally on routine brain imaging. We investigated clinical outcomes, endocrinopathies, and neuropsychological sequelae associated with tectal plate gliomas. Twenty-six patients with tectal plate glioma were identified in a 20-year retrospective review. Clinical outcomes, treatments, endocrine function, neuropsychological testing outcomes and radiographic imaging were reviewed for possible signs correlating with tumor progression. Among 26 patients, 19 presented with signs or symptoms of increased intracranial pressure (73 %) versus an incidental finding in 7 (27 %). Median follow-up was 46 months (range 8-143 months). Six of 26 (23 %) experienced progressive disease after diagnosis. Five of 26 (19 %) required more than one surgical procedure due to failure of initial endoscopic third ventriculostomy. Seven of 26 had history of endocrine dysfunction, of which, five presented with endocrine dysfunction (precocious puberty or short stature), 1 developed menstrual irregularities after surgical intervention and 1 had preexisting pan hypopituitarism. Of 12 patients with available neuropsychological testing, eleven had at least one indicator of executive functioning in the low-average to impaired range. While tectal plate gliomas have been considered indolent tumors that are rarely progressive, 23 % of patients in our cohort experienced disease progression and required further therapy. Neurocognitive deficits may occur, while endocrine deficiency is uncommon. Regular multidisciplinary oncology follow-up, routine monitoring with MRI and formal neurocognitive evaluation are imperative to provide early recognition of disease progression or recurrent hydrocephalus and to improve school functioning in this population.

The treatment of endoscopic third ventriculostomy for hydrocephalus caused by tectal plate gliomas

Methods Preoperative MRI prompted that there were tegmental region masses in 13 children with hydrocephalus. The increase was not obvious. The fiber neuroendoscopy was used for the endoscopic third ventriculostomy. During the operative observation of mesencephalon tegmental region, the hyperplasia of periaqueductal nerve tissue with light pink rough surface and the occlusive aqueduct opening could be observed.

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

Pediatric tectal plate gliomas: clinical and radiological progression, MR imaging characteristics, and management of hydrocephalus

Tectal plate gliomas are generally low-grade astrocytomas with favorable prognosis, and observation of the lesion and management of hydrocephalus remain the mainstay of treatment. A cohort of patients with tectal plate gliomas at 2 academic institutions was retrospectively reviewed. Forty-four patients with a mean age of 10.2 years who harbored tectal plate gliomas were included in the study. The mean clinical and radiological follow-up was 7.6 ± 3.3 years (median 7.9 years, range 1.5-14.7 years) and 6.5 ± 3.1 years (median 6.5 years, range 1.1-14.7 years), respectively. The most frequent intervention was CSF diversion (81.8% of patients) followed by biopsy (11.4%), radiotherapy (4.5%), chemotherapy (4.5%), and resection (2.3%). On MR imaging tectal plate gliomas most commonly showed T1-weighted isointensity (71.4%), T2-weighted hyperintensity (88.1%), and rarely enhanced (19%). The initial mean volume was 1.6 ± 2.2 cm(3) and it increased to 2.0 ± 4.4 cm(3) (p = 0.628) at the last follow-up. Frontal and occipital horn ratio (FOHR) and third ventricular width statistically decreased over time (p < 0.001 and p < 0.05, respectively). The authors' results support existing evidence that tectal plate gliomas frequently follow a benign clinical and radiographic course and rarely require any intervention beyond management of associated hydrocephalus.

Tectal plate gliomas: a review

Tectal plate gliomas are generally benign neoplastic lesions arising in the brainstem which can, with local extension, obstruct the aqueduct of Sylvius and lead to hydrocephalus. Diagnosis is based on initial suspicion fostered by the presentation of an obstructive hydrocephalus followed by physical exam which may potentially reveal indications of pyramidal tract dysfunction or cranial nerve palsies. MRI studies reveal a characteristic well-circumscribed, isodense or hypodense mass on T1-weighted images, with hyperdensity on T2 imaging. Yet current radiological methods insufficiently distinguish tectal plate gliomas from brainstem tumors or gliomas in the neighboring structures, and a definitive diagnosis requires biopsy and histopathological analysis. Management is planned according to the degree of associated signs and symptoms, and may range from diligent observation and periodic screening for advancing tumor development, to cerebrospinal fluid shunting in an effort to resolve obstructive hydrocephalus, to radio- and chemotherapy. A wide range of minimally invasive approaches using endoscopy is available for the neurosurgeon, including endoscopic third ventriculostomy and endoscopic aqueductoplasty.

Long-term change in ventricular size following endoscopic third ventriculostomy for hydrocephalus due to tectal plate gliomas

Endoscopic third ventriculostomy (ETV) is an alternative to shunt placement in children with hydrocephalus due to tectal plate gliomas (TPGs). However, controversy remains regarding the amount of ventricular size reduction that should be expected after ETV. This study investigates ventricular size change after ETV for TPGs. Twenty-two children were identified from a 15-year retrospective database of neuroendoscopic procedures performed at the authors' institution, Children's Hospital of Alabama, in patients with a minimum of 1 year of follow-up. Clinical outcomes, including the need for further CSF diversion and symptom resolution, were recorded. The frontal and occipital horn ratio (FOR) was measured on pre- and postoperative, 1-year, and last follow-up imaging studies. In 17 (77%) of 22 children no additional procedure for CSF diversion was required. Of those in whom CSF diversion failed, 4 underwent successful repeat ETV and 1 required shunt replacement. Therefore, in 21 (96%) of 22 patients, CSF diversion was accomplished with ETV. Preoperative and postoperative imaging was available for 18 (82%) of 22 patients. The FOR decreased in 89% of children who underwent ETV. The FOR progressively decreased 1.7%, 11.2%, and 12.7% on the initial postoperative, 1-year, and last follow-up images, respectively. The mean radiological follow-up duration for 18 patients was 5.4 years. When ETV failed, the FOR increased at the time of failure in all patients. Failure occurred 1.6 years after initial ETV on average. The mean clinical follow-up period for all 22 patients was 5.3 years. In all cases clinical improvement was demonstrated at the last follow-up. Endoscopic third ventriculostomy successfully treated hydrocephalus in the extended follow-up period of patients with TPGs. The most significant reduction in ventricular size was observed at the the 1-year followup, with only modest reduction thereafter.

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

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.

Contribution of the daily melatonin profile to diagnosis of tumors of the pineal region

Tumors of the pineal region (TPR) include different entities: germ cell tumors (GCT), pineal parenchymal tumors (PPT), meningiomas, and glial tumors. Except for GCT, there are no peripheral markers and histopathological diagnosis needs biopsy or surgery. We studied daily melatonin variations in twenty-nine patients with TPR and five with tectal plate glioma (TPG), used as controls, before and/or after surgery. Before surgery, a melatonin nycthemeral rhythm was observed in patients with TPG and TPR (one cyst, three PPT, one papillary tumor of the pineal region, two meningiomas, six gliomas). Melatonin rhythm was dramatically reduced for undifferentiated or invasive tumors. After surgery, the absence of melatonin variation in some cases could be the consequence of pineal damage by surgery. The contribution of determination of melatonin profiles to the diagnosis of TPR remains limited but of interest. The evidence for melatonin deficiency could justify melatonin administration to prevent the postpinealectomy syndrome.

Tectal glioblastoma

Brain stem gliomas are a heterogeneous group of neoplasms arising mostly in paediatric patients. Tectal plate gliomas represent a particular type of brain stem tumours usually with a benign, indolent clinical course, presenting with signs of raised intracranial hypertension due to supra-tentorialhydrocephalous caused by aqueductal stenosis. Seldom high-grade lesions arise in this location with tremendous therapeutic implications. When a malignant tumour is clinically and radiographically suspected a biopsy should be performed to obtain histological confirmation. Treatment is then planned in a case-by-case basis. We present the case of a glioblastoma of the tectal plate in a 22 years-old woman operated upon by a supracerebellar-infratentorial approach.

Management of Intrinsic Gliomas of the Tectal Plate in Children

The natural history, management, and long-term outcome for patients with benign, intrinsic tectal plate gliomas remain controversial in spite of their propensity to cause late-onset hydrocephalus. A 10-year retrospective review has identified 11 consecutive children with tectal plate lesions. Headache, vomiting, a decline in school performance, tremor, and complex partial seizures were common presenting symptoms. All patients presented with signs and symptoms of hydrocephalus. Magnetic resonance (MR) imaging delineated an intra-axial mass lesion of the midbrain primarily localized to the tectal plate which uniformly was hyperintense on T2-weighted imaging and had a more variable appearance on T1-weighted imaging and rare enhancement with gadolinium. No patient underwent surgical resection, chemotherapy, or radiotherapy. Three of 11 patients (27%) showed evidence of progression in size or a new focus of enhancement on MR imaging, which was clinically asymptomatic. In this series, no patient with a tectal plate lesion less than 1.5 cm in maximal diameter and without gadolinium enhancement showed any evidence of clinical or radiological progression. Although intrinsic tectal lesions in children are clinically indolent and the initial management consists of CSF diversion, these lesions may eventually progress and still warrant long-term follow-up with serial MR imaging.