Clinical diagnosis
Case 229
【Progress】
The brain images on MRI & CT showed the stable size of the tumor compared with the previous ones. He was given venous drip infusion of hyper osmotic fluid, inducing relief of headache.
【Discussion】
Brain tumor is the second highest mortality rate in children followed by acute leukemia (1). The most common brain tumor is glioma. Glioma is categorized into three or four types: pilocytic astrocytoma, (diffuse astrocytoma), anaplastic astrocytoma, glioblastoma multiforme (2). Of the gliomas, optic glioma and brain stem glioma are representative in children. The other common brain tumors are pituitary tumor, craniopharyngioma, pinealoma, germinoma, medduloblastoma and ependymoma. Other brain tumor is not common but some are brain tumors which radiologists should be kept in mind.
There are several brain diseases at temporal lobe including brain tumors of causing epilepsy or headache in children. The representing diseases are temporal sclerosis, cortical dysplasia, DNET (dysembryoplastic neuroepithelial tumor), ganglioglioma and meningioangiomatosis.
Meningioangiomatosis is histologically composed of meninge proliferation and vascular network occupying cortical cortex like hamartoma. Although it glows least, large meningioangiomatosis contains cyst formation, leptomeninges calcification and peripheral edema (3,4). In our case, wide-cortex containing small cysts and calcification with peripheral edema are demonstrated on CT and MRI, indicating to meet diagnostic image points of meningioangiomatosis.
DNET arises from cortex and histologically contains glio-neural element, indicating glio-neural neoplasm. Cortical dysplasia is associated with DNET in approximately 80% (5). A large DNET contains cyst and calcification (5,6). DNET is glio-neural tumor, implying unclear margin to normal cortex. In our case, cerebral cortex is widening like hyperplasia of cortex but not like a voluminous nodule or mass on CT and MRI, implying not to refer DNET. Cortical dysplasia is shown merely as widening or hyperplasia of cortex or cortex in deep white matter.
Ganglioglioma arises from temporal lobe in approximately 70% indicative of causing epilepsy but occurs at anywhere (7, 8). Ganglioglioma is a neoplasm composed of ganglion cells and glia cells. It belongs to low grade (Grade 1) tumor, indicating least peritumor edema. It often includes cystic component. Then, when a cystic tumor with least peritumor edema is observed in temporal lobe, ganglioglioma should be listed for differentiation.
Mesial temporal sclerosis is also known as hippocampal sclerosis is visualized as high signal intensity or atrophy of hippocampus on MRI with T2WI and/or FLAIR (8). This disease causes epilepsy and sometimes dementia. The atrophy area is a little different in case of Alzheimer disease. Hippocampal atrophy alone occurs in mesial temporal sclerosis, while atrophy of amygdala and entorhinal cortex proceed hippocampal atrophy in Alzheimer disease.
【Summary】
We presented an eleven-year-old boy suffering from headache. Brain CT and MRI showed a relatively large brain tumor with marked peripheral edema. The tumor composes of massive calcification along with swollen cortex. As the brain diseases which cause epilepsy in temporal lobes, hippocampal sclerosis, cortical dysplasia and relatively rare tumors such as meningioangiomatosis, DNET(dysembryonal neuroepithelial tumor) and ganglioglioma. Meningioangiomatosis compose of disorganized proliferation of meninge and vessels, occupying cortex, inducing cystic formation and calcification with least peripheral edema. DNET is a neoplasm like hamartoma and composes of ganglion and glia. The border between DNET and cortex is unclear because of its arising from cortex. Ganglioglioma occurs in temporal lobe in 70% and composes of unorganized ganglia and glia. It often contains a component of various sized cysts. Ganglioglioma and pilocytic astrocytoma belong to low grade (Grade 1) astrocytoma, sometimes difficult to distinct both tumor on CT and MRI.
【References】
1.Brain Tumor Statistics. American Brain Tumor Association. January 2017.
2.Key Statistics for Brain and Spinal Cord Tumors. American Cancer Society. January 4, 2018.
3.Jamil O, et al. Multifocal meningioangiomatosis in a 3-year-old patient. Journal of Neurosurgery:Pediatrics (Case report). 2012; 10 :486–489.
4.Feng R, et al. Diagnosis and surgical treatment of sporadic meningioangiomatosis. Clinical Neurology and Neurosurgery. 2013;115 : 1407–1414.
5.Takahashi A, et-al. Frequent association of cortical dysplasia in dysembryoplastic neuroepithelial tumor treated by epilepsy surgery. Surg Neurol. 2005;64 : 419-427.
6.Fernandez C, et al. The usefulness of MR imaging in the diagnosis of dysembryoplastic neuroepithelial tumor in children: a study of 14 cases. AJNR Am J Neuroradiol. 2003;24 : 829-34.
7.Lang, F. et al. Central nervous system gangliogliomas. Part 2: Clinical outcome. Journal of Neurosurgery. 1993; 79 : 867–873
8.Shin JH, et al. Neuronal tumors of the central nervous system: radiologic findings and pathologic correlation. Radiographics. 2002;22:1177-89.
2021.4.21