|Year : 2018 | Volume
| Issue : 2 | Page : 38-40
Glioblastoma multiforme in a pediatric child
Subhasis Mishra1, Ashis Patnaik2, Saroj Kumar Das Majumdar1, Dillip Kumar Parida1
1 Department of Radiotherapy, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
2 Department of Neurosurgery, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
|Date of Web Publication||21-Jun-2018|
Dr. Dillip Kumar Parida
Department of Radiotherapy, All India Institute of Medical Sciences, Bhubaneswar - 751 019, Odisha
Source of Support: None, Conflict of Interest: None
Glioblastoma multiforme (GBM) is the most aggressive, malignant primary brain tumor with increasing frequency in older age group. The majority of cases occur in the 6th to 8th decade of life, with a male predominance. GBM is unusual in the pediatric age group and accounts for only 3% of all the childhood brain tumors. The treatment of GBM is still challenging in children. We report a case of GBM in an 11-year-old male child located in the right cerebral hemisphere. Right fronto-parietal craniotomy and gross total excision of the tumor were performed successfully.
Keywords: Glioblastoma multiforme, management, pediatric
|How to cite this article:|
Mishra S, Patnaik A, Majumdar SK, Parida DK. Glioblastoma multiforme in a pediatric child. Oncol J India 2018;2:38-40
| Introduction|| |
Glioma is the most common primary malignant brain tumor constituting approximately 80% of the cases. Glioblastoma multiforme (GBM) is the most common type of glioma and is highly aggressive brain tumor with the poor outcome in both children and adults. GBM may manifest at any age with the frequency increases with age, and most of the cases found in between 6th and 8th decade of life.
Brain tumor is the most common type of solid neoplastic disorder in childhood and is the most common primary cancer-related cause of death among children. GBM is unusual in children and constitutes only 3% of all the primary pediatric brain tumors. This case report documents GBM that was located deep in the right cerebral hemisphere of an 11-year-old male child.
| Case Report|| |
An 11-year-old male child presented with complaints of right hemicranial headache, facial muscle twitching sensation, and neck pain for the past 5 months followed by a single episode of seizure, features of irritability, and left-sided hemiparesis for the past 4 months. His performance status (PS) was Eastern Cooperative Oncology Group 2, higher mental functions intact. Sensorimotor function was diminished in both limbs of the left side. Left upper limb had sensory and motor function of 4/5 and 3/5, respectively, and left lower limb had sensory and motor function of 4/5 and 3/5, respectively. Contrast magnetic resonance imaging (MRI) of the brain showed right fronto-parietal lobe intra-axial heterogeneous infiltrating irregularly marginated space occupying lesion measuring approximately 6.7 cm × 5.5 cm × 7.9 cm with variable signal characteristics of T1-weighted and T2-weighted heterogeneous being noted [Figure 1]. It is associated with perifocal edema and mass effect. Magnetic resonance spectroscopy (MRS) showed intralesional persisting raised choline peak with reduced N-acetyl-aspartate [Figure 2]. He underwent right fronto-parietal craniotomy and gross total excision of the tumor. Histopathological examination of the tumor tissue showed the presence of nuclear atypia, vascular endothelial proliferation, and necrosis consistent with GBM (WHO Grade IV) [Figure 3]a and [Figure 3]b. Noncontrast computed tomography scan of the brain performed on the 2nd day of surgery showing edema and internal hemorrhage with air density within the cranial cavity, and there is no definite evidence of any residual mass [Figure 4]. Now, he is under physiotherapy consultation for motor deficit which improved gradually. He is planned for adjuvant radiotherapy with concurrent tablet temozolomide followed by adjuvant temozolomide with proper dose schedule.
|Figure 1: Contrast magnetic resonance imaging of the brain showing right fronto-parietal intra-axial heterogeneous infiltrating irregularly marginated space occupying lesion (size approximately 6.7 cm × 5.5 cm × 7.9 cm) with variable signal characteristics of T1-weighted and T2-weighted heterogeneous and associated with perifocal edema and mass effect|
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|Figure 2: Magnetic resonance spectroscopy showing intralesional persisting raised choline peak with reduced N-acetyl-aspartate|
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|Figure 3: Histopathological examination of the postoperative tumor tissue showing features of nuclear atypia, vascular endothelial proliferation, and necrosis consistent with glioblastoma multiforme (a: ×40; and b: ×400)|
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|Figure 4: Noncontrast computed tomography scan brain showing postoperative edema and internal hemorrhage with air density within cranial cavity, and there is the absence of any residual mass|
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| Discussion|| |
Malignant gliomas are uncommon in children and consist of approximately 6.5% of all intracranial neoplastic disorders in the pediatric age group. Although these cases may occur at any anatomical site in the central nervous system, supratentorial site is the most frequent location and there is a slight male predominance. Histopathologically, GBM is characterized by hypercellularity, nuclear atypia, mytotic figures, vascular proliferation, and necrosis.
Presentation of the childhood GBM varies depending upon the location and size of the tumor and age of presentation. Frontal lobe is the most frequent location for childhood GBM consisting of 25%–35% of the cases. Seizures, headache, slowly progressive neurological deficits, hemiparesis, visual deficit, and symptoms of raised intracranial pressure are the common presenting symptoms. In our case, the first clinical feature was headache which was followed by an episode of seizure and hemiparesis.
MRI of the brain is the investigation of choice for diagnosing GBM. MRS is an advanced noninvasive MRI technique showing metabolic profile of brain and may evaluate cellular metabolism providing more information regarding response to treatment. It can identify active areas of tumor and can detect tumor recurrence before changes in contrast enhancement are evident. MRS showed increased choline levels and reduced NNA levels in most of the central nervous system malignancies including gliomas.
Younger age (<40 years), extent of surgery (completeness of removal), and good PS are the strongest prognostic factors. Because of infiltrative margin of the tumor, complete resection is usually not possible. Hemoglobin level and midline shift are the other risk factors. Complete tumor resection was performed in the present case.
Surgical excision of the tumor followed by chemoradiotherapy and adjuvant chemotherapy by temozolomide is the standard effective treatment for GBM. Despite advances in treatment, the average survival for GBM is about 1 year only, which has not been improved significantly. After the introduction of temozolomide chemotherapy both concurrent with radiotherapy and adjuvant treatment, the survival is improved for only 2 months.
The treatment of malignant gliomas in children is still a challenge. Yacob and Johnston. in a case series and Perkins et al. in a retrospective study for pediatric GBM reported a median survival of 9 and 13.5 months, respectively., According to few recent studies, there is a small increase in survival rates using temozolomide and lomustine to treat pediatric high-grade gliomas.
The role of genetic aberration and immunology in the management of GBM is under investigation. The introduction of bevacizumab and temozolomide in the treatment of GBM is effective in survival prolongation. There is a presence of difference in genetics and biology of GBM between pediatric and adult which may reveal different treatments and differences in treatment outcome. Temozolomide is less effective in pediatric GBM in comparison to adult GBM patients and may be related to O6-methylguanine-DNA methyltransferase gene expression. Mutations of tumor suppressor genes and protooncogenes are the key factors in the early detection of GBM and their pathogenesis and progression.
The different molecular analyses and profiling approaches are necessary to provide novel diagnostic and prognostic perceptions into the biology of GBM. The improvement in outcome of treatment and quality of life in GBM, especially in pediatric age group, requires further evaluation with larger studies.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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