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CASE REPORT
Ahead of print publication
 

Childhood pineal glioblastoma: Case report


1 Department of Neurosurgery, School of Medicine, Erciyes University, Kayseri, Turkey
2 Department of Pediatrics, Division of Pediatric Neurology, Faculty of Medicine, Gaziantep University, Şahinbey, Gaziantep, Turkey
3 Department of Radiology, Medicalpark Hospital, Gaziantep, Turkey
4 Department of Pathology, Gaziantep Patomer, Gaziantep, Turkey
5 Department of Neurosurgery, Medicalpark Hospital, Gaziantep, Turkey

Date of Submission08-Sep-2020
Date of Decision22-Mar-2021
Date of Acceptance26-Jan-2021
Date of Web Publication07-Jan-2022

Correspondence Address:
Tugay Atalay,
Department of Neurosurgery, School of Medicine, Erciyes University, Kayseri.
Turkey
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jpn.JPN_232_20

 

   Abstract 

Pineal glioblastomas (GBMs) are extremely rare tumors. Herein we will present a pediatric patient with GBM located in pineal region who was admitted with the symptoms of increased intracranial pressure and treated with surgical resection and radiotherapy. Introduction: Pineal region tumors are extremely rare accounting for less than 1% of all brain tumors. The most common type of pineal region tumors is germ cell tumor, followed by pineal parenchymal tumors, gliomas, atypical tumors, and the others.Case Report: A 5-year-old girl was admitted with complaints of headache, dizziness, imbalance in walking, and impaired vision for 1 month. Her neurological examination revealed a tendency to sleep, anisocoric pupillae, mesh eye pupil, dilated lateral gaze paralysis, and left hemiparasia (4/5 muscle strength). In magnetic resonance imaging, a mass was observed in the pineal region that infiltrates the right thalamus and right superior peduncle, isointense and hyperintense in T1 sections, hyperintense in T2 sections, having centrally contrasted areas in post-contrast sections. Due to the presence of evident hydrocephalus, a ventricular shunt was inserted and then through supracerebellar to infratentorial approach the lesion was removed subtotally. The histopathological diagnosis was GBM. GBMs in the pineal region are extremely rare tumors carrying poor prognosis. The patients are generally presented with the signs and symptoms of increased intracranial pressure. GBMs should be kept in mind in differential diagnosis of tumors in the pineal region.


Keywords: Childhood, glioblastoma, pineal



How to cite this URL:
Atalay T, Isikay S, Guzel E, Sari I, Guzel A. Childhood pineal glioblastoma: Case report. J Pediatr Neurosci [Epub ahead of print] [cited 2022 Dec 6]. Available from: https://www.pediatricneurosciences.com/preprintarticle.asp?id=335192





   Introduction Top


Pineal region tumors are extremely rare accounting for less than 1% of all brain tumors. The most common type of pineal region tumors is germ cell tumor, followed by pineal parenchymal tumors, gliomas, atypical tumors, and the others.[1],[2]

Glioblastoma (GBM) is the common CNS malignancy which is an aggressive diffuse glioma of astrocytic lineage. Unfortunately, pediatric GBM is a lethal cancer with no effective therapies due to destructive genetic alterations.[3],[4]

Herein we will present a pediatric patient with GBM located in the pineal region.


   Case Report Top


A 5-year-old girl was admitted with complaints of headache, dizziness, imbalance in walking, and impaired vision for 1 month. Her neurological examination revealed a tendency to sleep, anisocoric pupillae, mesh eye pupil, dilated lateral gaze paralysis, and left hemiparasia (4/5 muscle strength).

In brain computed tomography, a slightly hyperdense mass lesion with a size of approximately 40 × 50 × 52 mm having cystic-necrotic hypodense areas with lobulated contours located in the pineal region was present. Lateral ventricle was slightly wider than normal. In magnetic resonance imaging (MRI), a mass was observed in the pineal region that infiltrates the right thalamus and right superior peduncle, isointense and hyperintense in T1 sections, hyperintense in T2 sections, having centrally contrasted areas in post-contrast sections [Figure 1]. In her laboratory analysis, no hematological or biochemical abnormalities were determined. Tumor markers including alpha-fetoprotein, human–human chorionic gonadotropin, and placental alkaline phosphatase were within normal limits.
Figure 1: The pineal tumor that holds in T1 the sagittal and axial sections and enhances contrast in the cystic structure

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Due to the presence of evident hydrocephalus, a ventricular shunt was inserted and then through supracerebellar to infratentorial approach the lesion was removed subtotally. In histological evaluations, tumoral lesions characterized by hypercellularity, nuclear hyperchromasia, atypia, increased vascular structures, vascular endothelial proliferation, and necrosis were reported [Figure 2]. In histochemical studies, the results were as follows: [Figure 3] GFAP (+), S-100 (+), P53 (+) [Figure 3], Vimentin (+), Ki 67 (40%), Chromogranine (‒), Synaptophysin (‒), CD68 (‒), PanCK (‒), EMA (‒), CD95 (‒), CD30 (‒), CD117 (‒), Actin (‒), Myogenin (‒), amd CD34 (‒). The histopathological diagnosis was GBM. The patient was discharged 8 days after the operation to another center for radiotherapy. Her treatment is still ongoing from the 7 month of diagnosis.
Figure 2: Ischamic necrosis of tumour cells and microvascular proliferation (x200, H.E.)

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Figure 3: GFAP positivity on the tumour cells (x200, DAB)

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   Discussion Top


Pineal gland tumors are highly rare and pineal gland GBMs are extremely rare. To the best of our knowledge, less than 40 cases with pineal GBM are reported before. In general, GBMs of the pineal region infiltrate the upper portion of the midbrain and disseminate through the ependymal and leptomeningeal regions. Patients usually present with symptoms of intracranial hypertension due to obstructive hydrocephalus and to a deficit in vertical conjugate gaze. Nausea, vomiting, gait abnormalities, and vision changes are the most common symptoms of patients at admission. Radiological findings are the mainstay in diagnosis. Presence of the obstructive hydrocephalus, irregular borders, and heterogeneous contrast uptake due to central necrotic regions are the main radiological findings.[2],[5],[6] Our patient was also admitted with headache, dizziness, imbalance in walking, and impaired vision, and a pineal tumor was first diagnosed with the radiological findings.

Orrego et al.[7] reported four cases of GBMs in the pineal region who presented a severe headache and vomiting and treated with subtotal or total resection and radiotherapy with/without chemotherapy. The authors reported that the survival periods of these cases were between 8 and 31 months after the initial diagnosis. In the previous literature, the median reported survival was about 6 months.[8]

Pediatric GBMs are aggressive tumors. In a study of Nikitović et al.,[9] on a series of 15 children with GBM, the median survival time was 13.5 months, the presence of neurological deficit initially prior to radiotherapy was reported to have an impact on the overall survival, and children treated with gross total resection had longer overall survival. The main treatment options in pediatric GBMs are total surgical resection of the tumor, followed by chemotherapy and/or radiotherapy. In our patients, we also performed a local resection as large as possible, followed by radiotherapy.

GBMs in the pineal region are extremely rare tumors carrying poor prognosis. The patients are generally presented with signs and symptoms of increased intracranial pressure. GBMs should be kept in mind in differential diagnosis of tumors in the pineal region.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Al-Hussaini M, Sultan I, Abuirmileh N, Jaradat I, Qaddoumi I Pineal gland tumors: Experience from the SEER database. J Neurooncol 2009;94:351-8.  Back to cited text no. 1
    
2.
Dahiya S, Perry A Pineal tumors. Adv Anat Pathol 2010;17:419-27.  Back to cited text no. 2
    
3.
Rickert CH, Sträter R, Kaatsch P, Wassmann H, Jürgens H, Dockhorn-Dworniczak B, et al. Pediatric high-grade astrocytomas show chromosomal imbalances distinct from adult cases. Am J Pathol 2001;158:1525-32.  Back to cited text no. 3
    
4.
Thakkar JP, Dolecek TA, Horbinski C, Ostrom QT, Lightner DD, Barnholtz-Sloan JS, et al. Epidemiologic and molecular prognostic review of glioblastoma. Cancer Epidemiol Biomarkers Prev 2014;23:1985-96.  Back to cited text no. 4
    
5.
Amini A, Schmidt RH, Salzman KL, Chin SS, Couldwell WT Glioblastoma multiforme of the pineal region. J Neurooncol 2006;79:307-14.  Back to cited text no. 5
    
6.
Matsuda R, Hironaka Y, Suigimoto T, Nakase H Glioblastoma multiforme in the pineal region with leptomeningeal dissemination and lumbar metastasis. J Korean Neurosurg Soc 2015;58:479-82.  Back to cited text no. 6
    
7.
Orrego E, Casavilca S, Garcia-Corrochano P, Rojas-Meza S, Castillo M, Castaneda CA Glioblastoma of pineal region: Report of four cases and literature review. CNS Oncol 2017;6:251-9.  Back to cited text no. 7
    
8.
Stowe HB, Miller CR, Wu J, Randazzo DM, Ju AW Pineal region glioblastoma, a case report and literature review. Front Oncol 2017;7:123.  Back to cited text no. 8
    
9.
Nikitović M, Stanić D, Pekmezović T, Gazibara MS, Bokun J, Paripović L, et al. Pediatric glioblastoma: A single institution experience. Childs Nerv Syst 2016;32:97-103.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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