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Table of Contents    
Year : 2019  |  Volume : 67  |  Issue : 4  |  Page : 1153-1155

Synchronous Glioma of the Brainstem in a Patient with Recurrent Aggressive Vestibular Schwannoma

1 Department of Neurosurgery, Apollo Health City, Jubilee Hills, Hyderabad, Telangana, India
2 Department of Pathology, Apollo Health City, Jubilee Hills, Hyderabad, Telangana, India

Date of Web Publication10-Sep-2019

Correspondence Address:
Dr. Rajesh R Sannareddy
Department of Neurosurgery, Apollo Institute of Neurosciences, Jubilee Hills, Hyderabad - 500 033, Telangana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.266250

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How to cite this article:
Lath R, Sharma P, Sannareddy RR, Swain M, Ranjan A. Synchronous Glioma of the Brainstem in a Patient with Recurrent Aggressive Vestibular Schwannoma. Neurol India 2019;67:1153-5

How to cite this URL:
Lath R, Sharma P, Sannareddy RR, Swain M, Ranjan A. Synchronous Glioma of the Brainstem in a Patient with Recurrent Aggressive Vestibular Schwannoma. Neurol India [serial online] 2019 [cited 2021 Jan 20];67:1153-5. Available from:


Malignant transformation of vestibular schwannoma is reported after radiation therapy; however, the development of malignant tumors growing in the vicinity without radiation therapy is rare.[1]

A 60-year-old lady presented with decreased hearing in the left ear with altered left hemifacial sensations. Upon examination, there was left sensorineural hearing loss with loss of touch and pain sensation in the ophthalmic and maxillary divisions of the left trigeminal nerve. Left-sided cerebellar signs were also present. MRI brain features were consistent with left vestibular schwannoma [Figure 1]a. Patient underwent microsurgical resection of tumor with anatomical preservation of neural structures [Figure 1]b. Postoperatively, there were transient facial nerve palsy and impaired gag reflex which recovered over a period of time. Histopathological examination was suggestive of vestibular schwannoma [Figure 2].
Figure 1: (a) Contrast T1-weighted images showing well-defined, contrast-enhancing lobulated lesion in left cerebellopontine angle cistern with intrameatal extension and (b) postoperative CT scan showing complete excision of tumor

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Figure 2: (a) 20× Hematoxylin and eosin stain showing hypercellular spindle-shaped cells, (b) 20× S100 positive, (c) 40× GFAP negative, and (d) 20× MIB index 6–8%

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One year following surgery, she presented with progressive stiffness in all four limbs, imbalance while walking, and urinary incontinence of a few weeks duration. MRI brain showed a contrast-enhancing lesion with perilesional edema in the left cerebellopontine angle cistern with compression of the brain stem [Figure 3]. A possibility of tumor recurrence was considered. She underwent a ventriculoperitoneal shunt and redo-surgery for the lesion. Preoperatively, the lesion was vascular and firm in consistency with no tumor–brain interface. Lesional tissue was noted invading into the middle cerebellar peduncle.
Figure 3: (a) T2-weighted MR, (b) FLAIR sequence MR showing bulky pons with edema in brainstem and cerebellar peduncles, and (c) contrast MR showing ill-defined enhancing extra-axial lesion

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Histopathological examination revealed tumor composed predominantly of ovoid spindle cells with areas showing nuclear pleomorphism. Rare mitosis was noted and there was no evidence of necrosis. Foamy histiocyte infiltrates were noted at places. Immunohistochemical examination revealed diffuse and strong GFAP (Glial Fibrillary Acidic Protein) positivity with absent S100 in these areas suggestive of glioblastoma. Other areas showed strong positivity for S100 with MIB (Mitotic Proliferative) index of 20–25% in the spindle cells consistent with aggressive schwannoma [Figure 4]. In retrospect, a MIB stain done on the biopsy sample following the first surgery showed a MIB-1 index of 6–8%. Her clinical condition gradually deteriorated over the next 2 weeks and she succumbed to refractory hypotension and brain stem dysfunction.
Figure 4: (a) 40× Immunohistochemical staining showing area with strong GFAP positive, (b) GFAP negative, (c) S100 positive, and (d) high MIB index

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Recurrence rates following microsurgical treatment or stereotactic radiosurgery for vestibular schwannomas vary. A retrospective review of 2400 cases by Ahmed et al. found 0.05–1.8% recurrence rates following complete excision in 2252 cases.[2] A high proliferative index (MIB-1 >20%), cellularity, and pleomorphism are features of aggressive schwannomas, and such patients require frequent clinicoradiological follow-up.[3] Gonzalez reported a case of true malignant schwannoma with dissemination which was focally positive for S100, negative for GFAP, and had a high MIB index of 15.1%.[4] However, this patient had received postoperative radiotherapy. Hoa reported a patient who did not receive radiation therapy following microsurgical excision of vestibular schwannoma and was found to have glioblastoma of the midbrain during follow-up.[5] The case under discussion is similar to this report. Several cases of collision and synchronous brain tumors with glioblastoma as one of the components have been reported in the literature [Table 1].[15],[16],[17]
Table 1: List of recent publications with synchronous brain tumors, high grade glioma being one of the tumors

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The proposed hypothesis for pathogenesis of tumors of different histology located in juxtaposition is that one tumor might act as an irritating agent for local proliferation and growth of the other. Another proposed mechanism is the stimulation of glial cells in a paracrine manner by platelet-derived growth factor released from adjacent tumors like a meningioma.[11],[14]

A high MIB index in a vestibular schwannoma indicates aggressive behavior with high recurrence rate and poor prognosis, and thereby, these patients would need closer follow-up. One must consider the possibility of a synchronous intra-axial pathology such as glioma if the follow-up imageology suggests rapid regrowth of the tumor with significant edema in the cerebellum or brain stem.

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  References Top

Demetriades AK, Saunders N, Rose P, Fisher C, Rowe J, Tranter R, et al. Malignant transformation of acoustic neuroma / vestibular schwannoma 10 years after gamma knife stereotactic radiosurgery. Skull Base 2010;20:381-7.  Back to cited text no. 1
Ahmad RA, Sivalingam S, Topsakal V, Russo A, Taibah A, Sanna M. Rate of recurrent vestibular schwannoma after total removal via different surgical approaches. Ann Otol Rhinol Laryngol 2012;121:156-61.  Back to cited text no. 2
Hwang SK, Kim DG, Paek SH, Kim CY, Kim MK, Chi JG, et al. Aggressive vestibular schwannomas with post-operative rapid growth: Clinicopathological analysis of 15 cases. Neurosurgery 2002;51:1381-90.  Back to cited text no. 3
Gonzalez LF, Lekovic GP, Eschbacher J, Coons S, Spetzler RF. A true malignant schwannoma of the eight cranial nerve: Case report. Neurosurgery 2007;61:E421-2.  Back to cited text no. 4
Hoa M, Rhiew R, Kupsky WJ, Guthikonda M, Monsell EM. Glioblastoma multiforme after microsurgery for acoustic neuroma without radiotherapy: Limitation of the Cahan criteria. Otolaryngol Head Neck Surg 2008;139:323-4.  Back to cited text no. 5
Fortuniak J, Jaskolski DJ, Zawirski M, Tybor K. Glioblastoma multiforme and meningioma in the same patient – A case report. Neurol Neurochir Pol 2007;41:272-5.  Back to cited text no. 6
Mitsos AP, Konstantinou EA, Fotis TG, Lafazanos SA, Kontogeorgos G, Georgakoulias NV. Sphenoid wing meningioma and glioblastoma multiforme in collision: A case report with review of the literature. Neurol Neurochir Pol 2009;43:479-83.  Back to cited text no. 7
Muragaki Y, Chernov M, Tajika Y, Kubo O, Iseki H, Hori T, et al. Coincidence of central neurocytoma and multiple glioblastomas: A rare case report. J Neurooncol 2009;93:431-5.  Back to cited text no. 8
Chen G, Gao X, Liao Y, Xu B. A glioblastoma adjacent to a meningioma. Br J Neurosurg 2010;24:718-9.  Back to cited text no. 9
Pereira EA, Dabbous B, Qureshi HU, Ansorge O, Bojanic S. Rapid development of glioblastoma at the site of atypical meningioma resection. Br J Neurosurg 2010;24:471-3.  Back to cited text no. 10
Suzuki K, Momota H, Tonooka A, Nguchi H, Yamamoto K, Wanibuchi M, et al. Glioblastoma simultaneously present with adjacent meningioma: Case report and review of literature. J Neurooncol 2010;99:147-53.  Back to cited text no. 11
Haciyakupoglu E, Sav A, Haciyakupoglu S, Walter J. Multiple intracranial tumors: Coexistence of a glioblastoma and null cell pituitary adenoma within the same patient. Clin Neurol Neurosurg 2014;120:120-3.  Back to cited text no. 12
Ruiz J, Capilla E, Diaz JF, Ruiz JA, Andrade J, Hernandez T, et al. Secretory meningioma with KLF4 K409Q mutation in collision with glioma. Clin Neuropathol 2015;34:322-9.  Back to cited text no. 13
Yaghmour W, Kurdi ME, Baeesa SS. De novo glioblastoma in the territory of a recent middle cerebral artery infarction and a residual meningioma: Pathogenesis revisited. World J Surg Oncol 2016;14:112.  Back to cited text no. 14
Sahuc P, Joubert C, Nguyen AT, Fouet B, Wybrecht D, Faivre A, et al. Glioblastoma secondary to meningioma: A case report and literature review. World Neurosurg 2017;98:881.e9-881.e13.  Back to cited text no. 15
Naik H, Vernon V, Gade P, Bhople L, Guha A. Anaplastic astrocytoma and pituitary macroadenoma within the same patient. A rare case of intracranial collision tumor. Neurol India 2018;66:857-60.  Back to cited text no. 16
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Shaikh ST, Chawda MD, Mohanty CB, Deopujari CE. Primary sphenoid wing meningioma in contiguity with a glioblastoma. Neurol India 2018;66:245-8.  Back to cited text no. 17
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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

  [Table 1]


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