- Department of Hematology/Oncology, Staten Island University Hospital, Neuroscience Associates of New York, New York
- Division of Neurosurgery, Staten Island University Hospital, Neuroscience Associates of New York, New York
- Department of Pathology, Staten Island University Hospital, Neuroscience Associates of New York, New York
- Department of Radiology, Staten Island University Hospital, Neuroscience Associates of New York, New York
Division of Neurosurgery, Staten Island University Hospital, Neuroscience Associates of New York, New York
DOI:10.4103/2152-7806.69375© 2010 Nakhl F This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Nakhl F, Chang EM, Shiau JS, Alastra A, Wrzolek M, Odaimi M, Raden M, Juliano JE. A patient with multiple synchronous gliomas of distinctly different grades and correlative radiographic findings. Surg Neurol Int 16-Sep-2010;1:48
How to cite this URL: Nakhl F, Chang EM, Shiau JS, Alastra A, Wrzolek M, Odaimi M, Raden M, Juliano JE. A patient with multiple synchronous gliomas of distinctly different grades and correlative radiographic findings. Surg Neurol Int 16-Sep-2010;1:48. Available from: http://sni.wpengine.com/surgicalint_articles/a-patient-with-multiple-synchronous-gliomas-of-distinctly-different-grades-and-correlative-radiographic-findings/
Background:Multiple gliomas represent approximately 2 to 5% of all high-grade gliomas which are categorized as multifocal or multicentric depending on the timing, location and pattern of spread. We present a patient with bi-hemispheric, noncontiguous, low- and high-grade gliomas proven by biopsy. She underwent surgical excision and radiotherapy, but unfortunately succumbed to her disease shortly thereafter.
Case Description:A 64-year-old female presented to the hospital with confusion, disorientation and retrograde amnesia after an unwitnessed fall. There were no symptoms of headaches or visual disturbances before presentation. Magnetic resonance imaging (MRI) with and without gadolinium revealed a nonenhancing left temporal lobe mass without surrounding edema, an enhancing left frontal lobe mass with surrounding edema, and an enhancing right parietal lobe mass with surrounding edema. The patient underwent a left frontal craniotomy with gross total resection of the left frontal mass and a left temporal craniotomy, anterior temporal lobectomy and sub-total resection of the temporal lobe mass. Intraoperative Brainlab® image-guided navigation was used. Postoperative treatment consisted of radiotherapy.
Conclusion:This is the first reported case of multiple separate glial tumors, each with differing grades in which an MRI can be correlated with the tissue diagnoses. This case also highlights the possible mechanisms of transformation of glial tumors in the continuum from benign to malignant forms, lending insight to the possibility of using advanced genetic analysis in the treatment and diagnosis of these entities.
Keywords: High-grade astrocytoma, glioblastoma multiforme, low-grade astrocytoma, magnetic resonance imaging, multicentric, multifocal
Glial tumors represent 42% of all primary adult CNS neoplasms and over 75% of them are malignant high-grade gliomas. Glioblastoma multiforme, the most common malignant glioma, is a tumor with a high rate of proliferation and recurrence. It can present as multiple synchronous lesions due to its aggressive biological behavior.[
A 64-year-old female with an unremarkable past medical history was found unconscious after sustaining an unwitnessed fall resulting in an abrasion to her forehead.
She was confused, disoriented, and did not recall the circumstances of the event. Retrospectively, a seizure may have been the cause of the fall. The patient denied headaches or visual disturbances before presentation. On neurological physical exam the patient was disoriented, but had no apparent focal neurologic deficit. Her gait was steady and the rest of her physical exam was unremarkable.
MRI of the brain with and without gadolinium was performed. Three lesions were detected. A left temporal lobe lesion was identified and was slightly hypo-intense on T1-weighted imaging and hyper-intense on T2-weighted imaging with no associated gadolinium enhancement or surrounding edema [Figure
The patient was maintained on anti-convulsive medications. Subsequently, she underwent left-sided craniotomies for gross total resection of the left frontal tumor, anterior temporal lobectomy and subtotal resection of the temporal lobe tumor. Intra-operative microscope and BrainLab®, Munich, Germany, image-guided navigational system were utilized for the surgery. There was no attempt to remove or biopsy the right parietal tumor. The left frontal specimen showed primary high grade (WHO grade IV) primary neoplasm with astrocytic and neuronal differentiation [Figure
Postoperatively, the patient had an uneventful recovery except for a postoperative seizure, which was medically controlled. Despite early neurological response to adjuvent radiotherapy to the brain with a total dose of 40 Gy, the patient’s overall condition deteriorated a few months later secondary to the progression of her disease. She refused further treatment options and succumbed to her disease shortly thereafter. Autopsy was not performed.
This patient presented with multiple synchronous primary brain tumors of distinctly different grades correlating radiographically and pathologically. The presence of two distinct low- and high-grade gliomas is a very rare condition with few reported cases occurring in different clinical contexts[
Gliomas can be generally divided into “circumscribed” grade I histologies versus “diffuse” or “infiltrating” higher grade II, III or IV varieties.[
Three different pathways have been presented to characterize the pathophysiology of multifocal/multicentric high-grade gliomas.[
It is known that several mutations may contribute to the formation of secondary high-grade glioma which arise from preexisting low-grade gliomas. This type of tumor is relatively rare, has a female predominance and often has a characteristic mutation of TP53 and RB1, and increased expression of PDGFR.[
Multicentric and multifocal gliomas can be quite difficult to differentiate in most cases with the current available imaging technology and their histological features. True multicentric gliomas would have similar histological appearances. However, despite the heterogeneous nature of gliomas and the difference in their phenotype and grade, data suggest clonality in their origin.[
Although this case favors multicentric glioma arising from cell lines with varying degrees of differentiation suggestive of genetic predisposition or germline mutation, a correlation between the pathological findings and genetic features of the two distinct lesions would eventually be required to improve our understanding of this condition. Ultimately, identification of more genetic alterations resulting in the genesis, proliferation and invasiveness of malignant gliomas will better clarify the mechanism and direct future-targeted therapy in gliomas in general and more so in such atypical cases.
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