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Table of Contents    
CASE REPORT
Year : 2021  |  Volume : 69  |  Issue : 2  |  Page : 470-474

Management Dilemma in A Rare Case of Bilateral Temporo-Insular Glioma


1 Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India
2 Department of Neuropathology, All India Institute of Medical Sciences, New Delhi, India

Date of Submission29-Mar-2020
Date of Decision20-Jul-2020
Date of Acceptance10-Jul-2020
Date of Web Publication24-Apr-2021

Correspondence Address:
Ramesh S Doddamani
Department of Neurosurgery, All India Institute of Medical Sciences (AIIMS), New Delhi - 110 049
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.314559

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 » Abstract 


Multicentric gliomas are uncommon pathological entities, although well described in the literature. The aim of this study was to highlight the management issues in a rare case of multicentric glioma occurring in a bilateral temporo-insular region. A 35-year-old farmer with no comorbid illness presented with a history of progressively increasing holocranial headaches, intermittent episodes of vomiting, and memory disturbances from the last 3 months. Radiological findings were suggestive of multicentric glioma involving bilateral temporo-insular regions. Patient underwent tumor decompression first on the left side followed by the right side. Postoperative course was uneventful. Management of multicentric gliomas is challenging.


Keywords: Bilateral, insular, Mirror image gliomas, multicentric gliomas, temporal
Key Message: Multicentric mirror image gliomas involving bilateral temporo-insular region are extremely rare. The management is challenging and literature is non-existent. Tissue diagnosis should be attempted from all accessible lesions in view of histological heterogeneity. Maximal safe resection of both the lesions may be a reasonable strategy in this group of patients. Through this report, we intend to provide a management perspective of mirror image temporoinsular gliomas.


How to cite this article:
Meena R, Doddamani RS, Chipde H, Agrawal D, Mahajan S, Chandra P S. Management Dilemma in A Rare Case of Bilateral Temporo-Insular Glioma. Neurol India 2021;69:470-4

How to cite this URL:
Meena R, Doddamani RS, Chipde H, Agrawal D, Mahajan S, Chandra P S. Management Dilemma in A Rare Case of Bilateral Temporo-Insular Glioma. Neurol India [serial online] 2021 [cited 2021 May 15];69:470-4. Available from: https://www.neurologyindia.com/text.asp?2021/69/2/470/314559




Two or more gliomas occurring in an individual although uncommon but is well documented in the literature as multiple gliomas. These are classified as multifocal or multicentric. Multifocal gliomas are characterized by dissemination or spread via CSF, local metastasis, or commissural fibers.[1],[2] Gliomas that flout the above-mentioned pathways or mechanisms of dissemination, and that are distantly placed in the different lobes or hemispheres are known as multicentric gliomas.[1],[2],[3] The multicentric gliomas can be further divided into synchronous (if the tumors are detected on initial evaluation), or metachronous (if detected during treatment). In this case report, we will highlight a very rare case of multicentric glioma, in bilateral temporo-insular location and discuss its management dilemmas.


 » Case Report Top


A 35-year- old farmer with no comorbid illness presented with a history of progressively increasing holocranial headaches, intermittent episodes of vomiting, and memory disturbances from the last three months. On clinical examination higher mental functions were normal except for memory disturbances. Patient had no focal neurological deficits. There was no history of trauma, fever, radiation exposure, or intravenous drug abuse. Routine laboratory investigations were within normal limits. Computed tomography (CT) and Magnetic Resonance Imaging (MRI) scans of the patient showed two relatively ill demarcated mass lesions in bilateral temporo-insular region [Figure 1] and [Figure 2]. Left-sided lesion was larger and it contiguously involved most of the superior & middle temporal gyrus, insula, and left peritrigonal white matter part of the parietal lobe. Right-sided lesion contiguously involved anterior & middle part of superior & middle temporal gyrus, and insula. Both lesions were hypo-intense on T1 weighted and hyper-intense on T2 weighted imaging with the left-sided lesion also showing foci of micro-hemorrhages. In contrast administration, multiple irregular heterogeneous areas of enhancement were noted in the left-sided lesion [Figure 3]. Mass effect was noted on the left lateral ventricle, and there was medial displacement of uncus over the free edge of tentorium on both sides causing effacement of supra-sellar cisterns suggestive of bilateral uncal herniation.
Figure 1: Preoperative computed tomography (CT) scan of the patient (a) noncontrast (b) Contrast

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Figure 2: Preoperative magnetic resonance imaging (MRI) scan of the patient (a) Axial T1W1 sequence. (b) Axial T2W1 sequence. (c) Axial contrast T1W1 sequence

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Figure 3: Preoperative magnetic resonance imaging (MRI) scan of the patient. (a) Coronal T1W1 sequence with contrast. (b) Right sagittal T1W1 sequence with contrast. (c) Left sagittal T1W1 sequence with contrast

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In view of raised intracranial pressure, the patient was planned for semi-urgent surgery. Since there was evidence of uncal herniation bilaterally, it was decided to operate the tumor on both sides, simultaneously as opted by the patient and his relatives. Patient underwent tumor removal first on the left side following which the right-sided tumor was removed. Tumor was greyish white, moderately vascular, mostly soft in consistency with intermittent areas of firmness, and had an ill-defined plane with the normal brain. With the intent of speech preservation and good functional outcome, the left-sided tumor was partially decompressed. However, gross total removal of the tumor was done on the right side. Neuro-monitoring was used intraoperatively in the form of subcortical mapping to remove the tumor near the internal capsule region. Postoperative course was uneventful. Postoperative scan showed good operative cavity [Figure 4]. Biopsy of the tumor from both hemispheres came out to be anaplastic astrocytoma WHO grade III, IDH mutant type with MIB Index of 18%. Tumor cells were positive for p53, ATRX suppression was lost [Figure 5]. Subsequently, the patient received adjuvant whole-brain radiotherapy and chemotherapy. Contrast-enhanced MRI of the patient 6 months after surgery showed no evidence of recurrence or residual tumor on either side, suggesting that residual tumor on left side disappeared with adjuvant therapy [Figure 6]. One year after surgery the patient presented with progressively increasing right-sided weakness. The CEMRI brain showed contrast enhancing lesions in the left hemisphere involving the left internal capsule [Figure 7]. Patient was managed conservatively and counseled regarding the nature of disease. He was advised to follow-up with perfusion scans of the brain to differentiate recurrence from radiation necrosis, however the patient did not turn up and lost to follow-up.
Figure 4: Postoperative non-contrast computed tomography (CT) scan of the patient

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Figure 5: Photomicrographs of the tumor sections stained with HE (a and b) and immunostained for IDH1(c), p53(d), ATRX (e), and MIB1 (f). The tumor is moderately cellular and shows diffuse infiltrating pattern (a; HE, X100) with tumor cells showing moderate pleomorphism and mitosis (b; HE X200). The tumor cells show immunoreactivity for IDH1(c; IHC X200), P53(d; IHC X200), and loss of ATRX immunoexpression (e; IHC X200). MIB1 labelling index is 18% (f; IHC X200)

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Figure 6: Follow-up contrast-enhanced MRI 6 months after surgery shows no evidence of residual or recurrent lesion

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Figure 7: Follow-up contrast-enhanced MRI 12 months after surgery shows evidence of recurrent lesion in the left hemisphere

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 » Discussion Top


Multi-centric gliomas are a rare entity and their occurrence in the bilateral temporo-insular region has rarely been reported. A thorough review of the English literature, revealed only a single case report presenting as mirror image gliomas involving bilateral temporo-insular regions. This patient underwent surgical decompression of right-sided lesion only. Biopsy was anaplastic astrocytoma.[4]

However, hyperintensity in bilateral temporal lobe is a commonly encountered MRI finding. It can be seen in a wide variety of medical conditions like infections (herpes infection, congenital cytomegalovirus infection, epileptic syndromes (mesial temporal sclerosis), neurodegenerative diseases (Alzheimer's disease, Fronto-temporal dementia, Myotonic dystrophy), metabolic disorders (Wilson's disease, Hyperammonemia), paraneoplastic disorders (limbic encephalitis), radiation necrosis, trauma, and many others.[2] As bilateral temporal lobes are interconnected via anterior commissure, corpus callosum, and hippocampal commissure. These connections are one of the mechanisms leading to bilateral temporal lobe diseases.[2],[5]

Similarly, neoplasms can also spread from one temporal to another through these interconnecting fibers. This pattern of spread is frequently encountered in glial cell tumors especially astrocytomas.[2],[4] Anterior spread is the most common pattern (via anterior commissure), followed by posterior (via corpus callosum).[1],[2],[5] However, isolated involvement or infiltration of the temporal lobes without any evidence of involvement of these connections is very unlikely.

The pathogenesis of multicentric tumors is largely unknown. Several theories have been proposed for the unlinked proliferation of neoplastic cells at different topographic locations like de novo growth of multiple foci of diseases from separate areas of the brain. According to two-stage theory, multicentricity is the end result of two sequential steps. The first stage (Initiation) is characterized by the neoplastic transformation of a large area of brain or whole brain, which becomes more prone to neoplastic growth. Subsequently, in the second stage (Promotion) different stimuli such as hormonal, biochemical, or even viral lead to neoplastic proliferation at multiple sites. Several other theories like dissemination of glioma cells along interstitial fluid flow along the white matter tracts, unique propensity of glioma cells to invade normal brain and migrate long distances (invading individually or in small groups and abusing preexisting supply lines like guerilla warriors), development of secondary structures of glioma growth along with existing cytoarchitectural elements and metastasis along some pathways presently unknown have been proposed.[1],[2],[3]

Although the histological characteristics were similar bilaterally in our case, neuropathological heterogeneity in multi-centric gliomas have also been described in several other case reports.[3] Salavati et al. in their series of 25 patients with multicentric gliomas, reported this rare occurrence of neuropathological heterogeneity in six patients.[2] Therefore, for optimal therapeutic management, which could be different for each tumor in multicentric gliomas, histopathological diagnosis should be attempted from all accessible sites.

Appropriate management of multicentric gliomas is challenging and controversial. Some authors advocate an aggressive approach with surgical resection of tumor for longer and better survival, considering adjuvant therapy (radiotherapy and/or chemotherapy) to be more useful, when the tumor bulk is already reduced, while some authors recommend biopsy as the initial step, citing that extensive resection increases the risk of hemorrhage and neurological deficit without influencing survival.[1],[2],[3],[5] However, when surgical resection of the tumor has to be done, the goal should be maximal safe resection. Intraoperative neuro-monitoring may be a useful adjunct with preservation of the quality of life while maximizing the extent of resection in such multicentric gliomas.[1],[3] In our case, we considered the following treatment options and the same was discussed in detail with the patient.

  1. Two-stage procedure - Given that this most likely represented a high-grade glial neoplasm, and was bilateral in the notoriously challenging insular region. Decompression of the left insular tumor, awake for speech preservation given the extensive involvement of speech areas. Also, sampling of enhancing tissue to optimize the chance of capturing the highest-grade diagnosis. Subsequently, resection on the contralateral side would be guided by the histopathological report of the first surgery
  2. Single-stage procedure as tumor removal of the unilateral lesion might lead to unopposed herniation from the contralateral side. Therefore, first left-sided craniotomy and tumor removal in view of its larger size and greater mass effect subsequently followed by the right-side tumor removal in the same sitting.


However, patient and patient relatives opted for the second option, i.e. single staged tumor removal bilaterally. 56

In the review by Russo et al. on the management of multicentric gliomas, the median overall survival for the patients receiving surgery and adjuvant treatment was 12 ± 1.2 months and for patients receiving only radiotherapy and/or chemotherapy was 4 ± 1.7 months.[5] Although cytoreduction plays an important role in the outcome of these patients, it should be carefully balanced between decreased overall survival or worsened functional outcome versus good functional outcome.


 » Conclusion Top


Multicentric gliomas involving bilateral temporo-insular location are extremely rare with only one case reported till date. Management of such cases is challenging. Tissue diagnosis should be attempted from all accessible lesions; as histological heterogeneity can be present. The goal of surgery should be maximal safe resection, without compromising functional outcome.

Declaration of patient consent

Patient guardian has consented to the submission of the case report for submission to the journal.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 » References Top

1.
Patil CG, Eboli P, Hu J. Management of multifocal and multicentric gliomas. Neurosurg Clin N Am 2012;23:343-50.  Back to cited text no. 1
    
2.
Salvati M, Caroli E, Orlando ER, Frati A, Artizzu S, Ferrante L. Multicentric glioma: Our experience in 25 patients and critical review of the literature. Neurosurg Rev 2003;26:275-9.  Back to cited text no. 2
    
3.
Sureka J, Jakkani RK. Clinico-radiological spectrum of bilateral temporal lobe hyperintensity: A retrospective review. Br J Radiol 2012;85:e782-92.  Back to cited text no. 3
    
4.
Borkar SA, Tandon V, Kale SS, Mahapatra AK. Mirror-image insular glioma. Neurol India 2009;58:978-9.  Back to cited text no. 4
    
5.
di Russo P, Perrini P, Pasqualetti F, Meola A, Vannozzi R. Management and outcome of high-grade multicentric gliomas: A contemporary single-institution series and review of the literature. Acta Neurochir (Wien) 2013;155:2245-51.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]



 

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