Giant Hypothalamic Hamartoma

Giant Hypothalamic Hamartoma

Giant hypothalamic hamartomas (GHH) are extremely rare lesions in infants and usually intrinsically epileptogenic.

Giant HH is an exceptionally difficult neurological disease. Primary hypofractionated GKRS may be an alternative approach as mono/multitherapy with promising results and minimal complication 1).

Although the exploration of epileptic activity and the extent of ablation are limited by the number of SEEG electrodes for the complete disconnection. One-stage high-density focal stereo-array SEEG-guided radiofrequency was safe and effective for treating pediatric giant HH patients. It can be an alternative method to treat giant HHs where LITT is unavailable 2).

Stereotactic radiofrequency thermocoagulation (SRT) provided minimal invasiveness and excellent seizure outcomes even in patients with giant HHs 3).

Three pediatric patients (age range 17-65 mo) underwent primary hypofractionated GKRS in 2-3 consecutive days with an interfraction interval of 24 h. All patients had precocious puberty and were on GnRH analog. Frame-based GKRS was done with 8.1-9.2 Gy radiation per fraction at 50% isodose in 2-3 fractions targeting the entire hamartoma volume. The mean target volume was 5.67 cc (4.45-7.39 cc). The authors followed these patients for clinical and endocrinological assessment at every 6 mo interval while the repeat MRI has done at 6 mo and then annually. The seizure outcome analysis was done using the Engel Epilepsy Surgery Outcome Scale.

At a mean follow up of 27 mo (24-30 mo), 2 patients became Engel class 3 while one achieved Engel class 1 control. 2 patients showed halted pubertal growth with no additional hormonal aberration. 2 patients showed significant volumetric reduction (48% and 32%) and patchy necrosis inside the hypothalamic hamartoma (HH). There was no deficit in visual function, memory and cognition. One patient showed reduction in aggressiveness.

Giant HH is an exceptionally difficult neurological disease. Primary hypofractionated GKRS may be an alternative approach as mono/multitherapy with promising results and minimal complication 4)


Wang et al. analyzed the clinical data of six patients with giant HHs (masses with a maximum diameter >30 mm) who underwent stereotactic electrode implantation between November 2017 and April 2019. After a multidisciplinary discussion, we designed a high-density focal stereo-array electrode implantation strategy. SEEG-guided bipolar coagulations were performed between two contiguous contacts of the same electrode, or between two adjacent contacts of different electrodes. Results: Among the six patients, three were male and three were female, with an average age of 5.08 ± 4.73 years (range, 1.4-12 years); the average follow-up duration was 20.17 ± 5.49 months. One patient had previously undergone open surgery. Four patients had gelastic seizures, one had gelastic and tonic seizures, and one had gelastic and generalized tonic-clonic seizures. The number of implanted electrodes ranged from 3 to 7, with an average of 5.33. One patient had transient diabetes insipidus after the operation, and no child had fever or new hormone metabolisms disorder after surgery. Four patients had Engel I classification outcomes (free from disabling seizures), and two patients had Engel II classification outcomes. Conclusion: Although the exploration of epileptic activity and the extent of ablation are limited by the number of SEEG electrodes for the complete disconnection. One-stage high-density focal stereo-array SEEG-guided radiofrequency was safe and effective for treating pediatric giant HH patients. It can be an alternative method to treat giant HHs where LITT is unavailable 5).


Cristobal et al. presented a unique case of an asymptomatic giant hypothalamic hamartoma diagnosed prenatally by fetal magnetic resonance imaging and followed throughout infancy. This case demonstrates the utility of multimetric analysis using difference sequences, including diffuse-weighted imaging, to assess specific properties of intracranial lesions detected in utero and to aid in accurate diagnosis prior to birth 6).


A 10-month-old girl child presenting with drug-resistant seizures and a giant hypothalamic lesion that was confirmed as hamartoma on histopathology. Surgical decompression and disconnection from the hypothalamus was performed with the intent of controlling her seizures. Unfortunately, the patient developed right middle cerebral artery and posterior cerebral artery territory infarction, possibly due to vasospasm or thrombosis of the vessels. The patient had a stormy postoperative course but has recovered well neurologically at the 18-month follow-up. Histopathological examination revealed abnormal clusters of NeuN-positive neurons, which was confirmatory of hypothalamic hamartoma 7).


1) , 4)

Tripathi M, Maskara P, Sankhyan N, Sahu JK, Kumar R, Kumar N, Ahuja CK, Kaur P, Kaur R, Batish A, Mohindra S. Safety and Efficacy of Primary Hypofractionated Gamma Knife Radiosurgery for Giant Hypothalamic Hamartoma. Indian J Pediatr. 2021 Jan 27. doi: 10.1007/s12098-020-03637-w. Epub ahead of print. PMID: 33501606.
2) , 5)

Wang M, Zhou Y, Zhang Y, Shi W, Zhou S, Wang Y, Li H, Zhao R. One-Stage High-Density Focal Stereo-Array SEEG-Guided Radiofrequency Thermocoagulation for the Treatment of Pediatric Giant Hypothalamic Hamartomas. Front Neurol. 2020 Sep 2;11:965. doi: 10.3389/fneur.2020.00965. PMID: 32982954; PMCID: PMC7493627.
3)

Shirozu H, Masuda H, Ito Y, Sonoda M, Kameyama S. Stereotactic radiofrequency thermocoagulation for giant hypothalamic hamartoma. J Neurosurg. 2016 Oct;125(4):812-821. doi: 10.3171/2015.6.JNS15200. Epub 2016 Jan 1. PMID: 26722850.
6)

Cristobal A, Vorona G, Ritter A, Lanni S, Urbine J. Pre- and postnatal MR imaging of an asymptomatic giant hypothalamic hamartoma. Radiol Case Rep. 2020 Jun 16;15(8):1250-1255. doi: 10.1016/j.radcr.2020.05.041. PMID: 32577141; PMCID: PMC7303913.
7)

Kandregula S, Savardekar AR, Nandeesh BN, Arivazhagan A, Rao MB. Giant Hypothalamic Hamartoma in an Infant: A Case Report and Review of the Literature. Pediatr Neurosurg. 2017;52(1):55-61. doi: 10.1159/000448738. Epub 2016 Oct 26. PMID: 27780163.

Posterior Fossa A ependymoma

Posterior Fossa A ependymoma

Posterior fossa ependymoma comprise three distinct molecular variants, termed PF-EPN-A (PFA), PF-EPN-B (PFB), and PF-EPN-SE (subependymoma1).


While supratentorial ependymomas are characterized by recurrent oncogenic fusions, infratentorial ependymomas can be classified by their epigenetic signatures into two main groups, pediatric-type (PFA) and adult-type (PFB) ependymomas


Group A patients are younger, have laterally located tumors with a balanced genome, and are much more likely to exhibit recurrence, metastasis at recurrence, and death compared with Group B patients. Identification and optimization of immunohistochemical (IHC) markers for PF ependymoma subgroups allowed validation of findings on a third independent cohort, using a human ependymoma tissue microarray, and provides a tool for prospective prognostication and stratification of PF ependymoma patients 2).

H3K27me3 (me3) loss by immunohistochemistry (IHC) is a surrogate marker for PFA wherein its loss is attributed to overexpression of Cxorf67/EZH2 inhibitory protein (EZHIP), C17orf96, and ATRX loss. Nambirajan et al. aimed to subgroup posterior fossa ependymomas using me3 IHC and study correlations of the molecular subgroups with other histone-related proteins, 1q gain, Tenascin C, and outcome. IHC for me3, acetyl-H3K27, H3K27MATRXEZH2EZHIPC17orf96Tenascin C, and fluorescence in-situ hybridization for chromosome 1q25 locus were performed on an ambispective posterior fossa ependymomas cohort (2003-2019). H3K27M-mutant gliomas were included for comparison. Among 69 patients, PFA (me3 loss) constituted 64%. EZHIP overexpression and 1q gain were exclusive to PFA seen in 72% and 19%, respectively. Tenascin C was more frequently positive in PFA (p = 0.02). H3K27M expression and ATRX loss were noted in one case of PFA-EPN each. All H3K27M-mutant gliomas (n = 8) and PFA-EPN (n = 1) were EZHIP negative. C17orf96 and acetyl-H3K27 expression did not correlate with me3 loss. H3K27me3 is a robust surrogate for PF-EPN molecular subgrouping. EZHIP overexpression was exclusive to PFA EPNs and was characteristically absent in Diffuse midline glioma H3 K27M-mutants and the rare PFA harboring H3K27M mutations representing mutually exclusive pathways leading to me3 loss 3).

Ramaswamy and Taylor found that the strongest predictor of poor outcome in patients with posterior fossa ependymoma across the entire age spectrum was molecular subgroup PFA, which was reported in the paper entitled “Therapeutic impact of cytoreductive surgery and irradiation of posterior fossa ependymoma in the molecular era: a retrospective multicohort analysis” in the Journal of Clinical Oncology. Patients with incompletely resected PFA tumors had a very poor outcome despite receiving adjuvant radiation therapy, whereas a substantial proportion of patients with PFB tumors can be cured with surgery alone 4).


A total of 72 Posterior fossa ependymomas cases were identified, 89% of which were PFA. The 10-year progression-free survival rate for all patients with PFA was poor at 37.1% (95% confidence interval, 25.9%-53.1%). Analysis of consecutive 10-year epochs revealed significant improvements in progression-free survival and/or overall survival over time. This pertains to the increase in the rate of gross (macroscopic) total resection from 35% to 77% and the use of upfront radiotherapy increasing from 65% to 96% over the observed period and confirmed in a multivariable model. Using a mixed linear model, analysis of longitudinal neuropsychological outcomes restricted to patients with PFA who were treated with focal irradiation demonstrated significant continuous declines in the full-scale intelligence quotient over time with upfront conformal radiotherapy, even when correcting for hydrocephalus, number of surgeries, and age at diagnosis (-1.33 ± 0.42 points/year; P = .0042) 5).

Effective treatment is limited to surgical resection and focal radiotherapy.


1)

Cavalli FMG, Hübner JM, Sharma T, Luu B, Sill M, Zapotocky M, Mack SC, Witt H, Lin T, Shih DJH, Ho B, Santi M, Emery L, Hukin J, Dunham C, McLendon RE, Lipp ES, Gururangan S, Grossbach A, French P, Kros JM, van Veelen MC, Rao AAN, Giannini C, Leary S, Jung S, Faria CC, Mora J, Schüller U, Alonso MM, Chan JA, Klekner A, Chambless LB, Hwang EI, Massimino M, Eberhart CG, Karajannis MA, Lu B, Liau LM, Zollo M, Ferrucci V, Carlotti C, Tirapelli DPC, Tabori U, Bouffet E, Ryzhova M, Ellison DW, Merchant TE, Gilbert MR, Armstrong TS, Korshunov A, Pfister SM, Taylor MD, Aldape K, Pajtler KW, Kool M, Ramaswamy V. Heterogeneity within the PF-EPN-B ependymoma subgroup. Acta Neuropathol. 2018 Aug;136(2):227-237. doi: 10.1007/s00401-018-1888-x. Epub 2018 Jul 17. PMID: 30019219; PMCID: PMC6373486.
2)

Witt H, Mack SC, Ryzhova M, Bender S, Sill M, Isserlin R, Benner A, Hielscher T, Milde T, Remke M, Jones DT, Northcott PA, Garzia L, Bertrand KC, Wittmann A, Yao Y, Roberts SS, Massimi L, Van Meter T, Weiss WA, Gupta N, Grajkowska W, Lach B, Cho YJ, von Deimling A, Kulozik AE, Witt O, Bader GD, Hawkins CE, Tabori U, Guha A, Rutka JT, Lichter P, Korshunov A, Taylor MD, Pfister SM. Delineation of two clinically and molecularly distinct subgroups of posterior fossa ependymoma. Cancer Cell. 2011 Aug 16;20(2):143-57. doi: 10.1016/j.ccr.2011.07.007. PMID: 21840481; PMCID: PMC4154494.
3)

Nambirajan A, Sharma A, Rajeshwari M, Boorgula MT, Doddamani R, Garg A, Suri V, Sarkar C, Sharma MC. EZH2 inhibitory protein (EZHIP/Cxorf67) expression correlates strongly with H3K27me3 loss in posterior fossa ependymomas and is mutually exclusive with H3K27M mutations. Brain Tumor Pathol. 2020 Nov 1. doi: 10.1007/s10014-020-00385-9. Epub ahead of print. Erratum in: Brain Tumor Pathol. 2021 Jan 9;: PMID: 33130928.
4)

Ramaswamy V, Taylor MD. Treatment implications of posterior fossa ependymoma subgroups. Chin J Cancer. 2016 Nov 15;35(1):93. doi: 10.1186/s40880-016-0155-6. PMID: 27846874; PMCID: PMC5111181.
5)

Zapotocky M, Beera K, Adamski J, Laperierre N, Guger S, Janzen L, Lassaletta A, Figueiredo Nobre L, Bartels U, Tabori U, Hawkins C, Urbach S, Tsang DS, Dirks PB, Taylor MD, Bouffet E, Mabbott DJ, Ramaswamy V. Survival and functional outcomes of molecularly defined childhood posterior fossa ependymoma: Cure at a cost. Cancer. 2019 Jun 1;125(11):1867-1876. doi: 10.1002/cncr.31995. Epub 2019 Feb 15. PMID: 30768777; PMCID: PMC6508980.

Tectal glioma

Tectal gliomas fall under the grouping of childhood midbrain gliomas and unlike the other tumours in that group they are typically low grade astrocytomas with good prognosis.

Tectal plate gliomas are encountered in children and adolescents.

A male predilection has sometimes been reported although this is by no means certain.

An association with neurofibromatosis type I (NF1) has been reported 1).

Their expansion within the brainstem causes narrowing the aqueduct of Sylvius and causing obstructive hydrocephalus with presentation usually secondary to headache.

Additional symptoms may include gaze palsy, due to compression of the medial longitudinal fasciculus leading to an upgaze palsy, diplopia or Parinaud syndrome, although these are uncommon.

The vast majority of lesions are low grade astrocytoma, although occasionally other glial series tumours are encountered in the tectal region including ependymoma, ganglioglioma and primitive neuroectodermal tumours (PNET).

see Tectal ganglioglioma.

Since many of these are not biopsied, meaningful statistical analysis is not possible.

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.

The superb sensitivity of MR and its multiplanar imaging capability permit unparalleled diagnostic accuracy in this region. The sagittal and axial planes are ideal for evaluating the tectum. CT remains important in the detection of acute hemorrhage and calcification. Grouping of abnormalities on the basis of anatomic boundaries (tectum, aqueduct, and quadrigeminal plate cistern) is useful in establishing the correct diagnosis 2).

CT

Typical CT finding is homogeneous expansion of tectal plate, isodense to grey matter with minimal enhancement on postcontrast images.

On CT it is not uncommon to find a central tectal calcification.


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.

Typically the tumours demonstrate expansion of the tectal plate by a solid nodule of tissue.

T1: iso to slightly hypointense to grey matter

T2: hyperintense to grey matter

T1 C+ (Gd): usually no enhancement

With time the mass can develop small cystic spaces (sometimes associated with neurological deficits) or calcification.

Higher grade tumours tend to be larger and tend to enhance more vividly.

Tumors leading to occlusion of the aqueduct of Sylvius include those of pineal, thalamic, and tectal origins.

see Aqueductal tumor.

The differential diagnosis from germ cell tumor or pineal cyst is essential for treatment.

When the tectum is near-normal then the differential is largely limited to:

aqueductal stenosis

no mass lesion

a focal stenosis or web may be visible

With larger lesions, where the mass is not definitely arising from the tectal plate then the differential is essentially that of a pineal region mass and therefore includes:

pineal parenchymal tumours and germ cell tumours

pineal cyst

meningioma

cerebral metastasis

cavernous malformation

In patients with NF1 a hamartoma should also be considered. They tend to have some T1 hyperintensity.

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 3)

Tumors leading to occlusion of the sylvian aqueduct include those of pineal, thalamic, and tectal origins. These tumors cause obstructive hydrocephalus and thus necessitate a CSF diversion procedure such as an endoscopic third ventriculostomy (ETV), often coupled with an endoscopic biopsy (EBX).

As tectal plate gliomas are low grade and often very slow growing, shunting is often the only required intervention for long term survival. As surgical biopsy can have significant morbidity in this area, usually the diagnosis is made on imaging findings alone.

Imaging predictors of patients who will need further treatment include a size greater than 2.5 cm and presence of contrast enhancement.

In the minority of patients who progress, radiotherapy often leads to local control or even tumour regression.

Surgical excision is sometimes necessary.

Tectal plate gliomas showed indolent clinical courses, even after radiologic tumor progression. After the treatment of obstructive hydrocephalus, clinical and radiologic follow-up can be recommended for indolent tectal plate gliomas 4).


2015

Between October 2002 and May 2011, 11 patients with tectal gliomas were treated with Gamma Knife radiosurgery. Five patients had pilocytic astrocytomas and six nonpilocytic astrocytomas. Ten patients presented with hydrocephalus and underwent a CSF diversion procedure [7 V-P shunt and 3 endoscopic third ventriculostomy (ETV)]. The tumor volume ranged between 1.2-14.7 cc (median 4.5 cc). The prescription dose was 11-14 Gy (median 12 Gy).

Patients were followed for a median of 40 months (13-114 months). Tumor control after radiosurgery was seen in all cases. In 6/11 cases, the tumors eventually disappeared after treatment. Peritumoral edema developed in 5/11 cases at an onset of 3-6 months after treatment. Transient tumor swelling was observed in four cases. Four patients developed cysts after treatment. One of these cases required aspiration and eventually disappeared, one became smaller spontaneously, and two remained stable.

Gamma Knife radiosurgery is an effective and safe technique for treatment of tectal gliomas. Tumor shrinkage or disappearance after Gamma Knife radiosurgery may preclude the need for a shunt later on 5).


Dabscheck et al., conducted a retrospective review of all patients with tectal gliomas over a 22-year period at a single institution. Data extraction included sex, age at presentation of tectal glioma and age of presentation with seizures, magnetic resonance imaging (MRI) findings, seizure frequency and semiology, and EEG findings. They identified 79 patients, 66 of whom had adequate imaging and clinical data for further analysis. Eight patients (12.1%) had a history of seizures. Three patients had a clear symptomatic cause of seizures. Three patients were diagnosed with a tectal glioma as an incidental finding after a first seizure. One patient had a history of febrile convulsions. One patient had a generalized seizure 5 years after presenting with macrocephaly. Although the risk of seizure in children with known tectal glioma was relatively high, we did not identify specific clinical, radiologic, EEG, or MRI features that are predictive of increased risk. Thus, in children with tectal gliomas who have seizures, alternative causes for the seizures must be sought 6).


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 this 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 7).

2014

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 8).

2013

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 9).

2012

A 5-year-old boy who had tectal plate low grade glioma with obstructive hydrocephalus was managed with Codman programmable ventriculoperitoneal shunt. There was a spontaneous change in the opening pressure of the shunt valve leading to shunt malfunction. Routinely used household appliances produce a magnetic field strong enough to cause change in the setting of shunt valve pressure and may lead to valve malfunction 10).

2003

Tumors involving the tectal region constitute a distinct subgroup of brain stem gliomas with an indolent clinical course. Here, we present the clinical and neuroradiologic features of 9 children with tectal tumors. All patients presented with signs and symptoms of hydrocephalus and were managed with ventriculoperitoneal shunt insertion. MRI studies revealed focal hyperintense lesions on T2-weighted images without any contrast enhancement, and no evidence of progression was demonstrated in any patient. We also reviewed the published series of tectal gliomas in the literature to compare with our results. Based on these and other published series, it was concluded that intrinsic tectal gliomas of childhood with sizes less than 2 cm in diameter and without any tumor extension or contrast enhancement constitute a specific subgroup of tectal masses which rarely display invasive clinical behavior and should be managed conservatively. CSF diversion procedures and long-term yearly follow-up examinations with MRI scans are sufficient in these patients 11).

1999

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 12).

1998

A retrospective review was done of the medical records and imaging studies of 32 children (16 boys and 16 girls; mean age, 8 years) with tectal tumors. Eight children had CT, 11 had MR imaging, and 13 had both CT and MR studies. Findings from surgical and pathologic reports as well as from follow-up examinations (mean follow-up period, 5 years; range, 3.6 months to 17 years) were included in the review.

All patients had hydrocephalus and all but one required CSF diversion. The tectum was the center of the tumor in all cases and the majority of the tumors appeared isodense on CT scans, isointense on T1-weighted MR images, and hyperintense on T2-weighted images. Twenty patients required no further treatment. In this group, the mean maximum tumor diameter was 1.8 cm and enhancement occurred in two cases. At follow-up, 18 patients had stable tumor size, one had an increase in tumor size with cyst formation but no worsening of symptoms, and one had a decrease in tumor size. Twelve patients required further treatment (excision and/or radiotherapy) because of progression as indicated by either increased tumor size or worsening of symptoms. In this group, the mean maximum tumor diameter was 2.5 cm and contrast enhancement occurred in nine cases. Further follow-up in this group showed decreased tumor size in eight and stable residual tumor in three.

Tectal tumors in childhood have variable behavior. MR imaging assists in the clinical determination of which children need treatment beyond CSF diversion. Larger tumor size and enhancement are radiologic predictors of the need for further treatment 13).

1994

A series of 12 patients with tectal plate gliomas, is presented treated by direct surgery. Mean age was 19 years. All patients presented with signs of raised intracranial pressure and supratentorial hydrocephalus on CT scan. Diplopia was the most common local sign. CT scan and MR imaging showed 4 intrinsic, 6 exophytic, and 2 ventrally infiltrating tectal tumours. The histological diagnosis was low-grade astrocytoma in 7, high-grade astrocytoma in 2, oligodendroglioma in one, oligo-astrocytoma in one, and ependymoma in one case. The suboccipital supra- and transtentorial approach was used in every cases. Tumour resection was generous at the level of the superior colliculi, but on the contrary, resection was limited at the level of inferior colliculi due to the auditory risk. Tumour removal was total (macroscopically) in 9 cases and partial in 3 cases. There were 4 surgical complications and one death related to surgery. Parinaud’s syndrome was the most-common postoperative sequelae. Auditory hallucinations and the acoustic neglect syndrome were seen once. In three cases additional radiotherapy and chemotherapy were given once with severe sequelae. The treatment of tectal plate gliomas is controversial. The role of different therapeutic options remains open. We consider the tectal plate as a relatively safer territory for surgery than the ventral part of the midbrain. The brain stem auditory evoked potentials (BAEPs) and middle latency potentials (MLPs) monitoring can help to determine the appropriate limit of surgery 14).

see Tectal glioma case reports


1)

Dinçer A, Yener U, Özek MM. Hydrocephalus in patients with neurofibromatosis type 1: MR imaging findings and the outcome of endoscopic third ventriculostomy. AJNR Am J Neuroradiol. 2011 Apr;32(4):643-6. doi: 10.3174/ajnr.A2357. Epub 2011 Feb 17. PubMed PMID: 21330395.
2)

Friedman DP. Extrapineal abnormalities of the tectal region: MR imaging findings. AJR Am J Roentgenol. 1992 Oct;159(4):859-66. Review. PubMed PMID: 1529854.
3)

Igboechi C, Vaddiparti A, Sorenson EP, Rozzelle CJ, Tubbs RS, Loukas M. Tectal plate gliomas: a review. Childs Nerv Syst. 2013 Oct;29(10):1827-33. doi: 10.1007/s00381-013-2110-z. Epub 2013 Apr 24. Review. PubMed PMID: 23612874.
4)

Kim JW, Jung JH, Baek HJ, Kim SK, Jung TY. Case Reports of Tectal Plate Gliomas Showing Indolent Course. Brain Tumor Res Treat. 2020 Oct;8(2):109-112. doi: 10.14791/btrt.2020.8.e17. PMID: 33118343.
5)

El-Shehaby AM, Reda WA, Abdel Karim KM, Emad Eldin RM, Esene IN. Gamma Knife radiosurgery for low-grade tectal gliomas. Acta Neurochir (Wien). 2015 Feb;157(2):247-56. doi: 10.1007/s00701-014-2299-y. Epub 2014 Dec 16. PubMed PMID: 25510647.
6)

Dabscheck G, Prabhu SP, Manley PE, Goumnerova L, Ullrich NJ. Risk of seizures in children with tectal gliomas. Epilepsia. 2015 Sep;56(9):e139-42. doi: 10.1111/epi.13080. Epub 2015 Jul 21. PubMed PMID: 26193802.
7)

Gass D, Dewire M, Chow L, Rose SR, Lawson S, Stevenson C, Pai AL, Jones B, Sutton M, Lane A, Pruitt D, Fouladi M, Hummel TR. Pediatric tectal plate gliomas: a review of clinical outcomes, endocrinopathies, and neuropsychological sequelae. J Neurooncol. 2015 Mar;122(1):169-77. doi: 10.1007/s11060-014-1700-2. Epub 2015 Jan 13. PubMed PMID: 25582835.
8)

Griessenauer CJ, Rizk E, Miller JH, Hendrix P, Tubbs RS, Dias MS, Riemenschneider K, Chern JJ. Pediatric tectal plate gliomas: clinical and radiological progression, MR imaging characteristics, and management of hydrocephalus. J Neurosurg Pediatr. 2014 Jan;13(1):13-20. doi: 10.3171/2013.9.PEDS13347. Epub 2013 Nov 1. PubMed PMID: 24180680.
9)

Romeo A, Naftel RP, Griessenauer CJ, Reed GT, Martin R, Shannon CN, Grabb PA, Tubbs RS, Wellons JC 3rd. Long-term change in ventricular size following endoscopic third ventriculostomy for hydrocephalus due to tectal plate gliomas. J Neurosurg Pediatr. 2013 Jan;11(1):20-5. doi: 10.3171/2012.9.PEDS12243. Epub 2012 Nov 9. PubMed PMID: 23140213.
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