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|LETTERS TO EDITOR
|Year : 2019 | Volume
| Issue : 4 | Page : 1153-1155
Synchronous Glioma of the Brainstem in a Patient with Recurrent Aggressive Vestibular Schwannoma
Rahul Lath1, Pankaj Sharma1, Rajesh R Sannareddy1, Meenakshi Swain2, Alok Ranjan1
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 Publication||10-Sep-2019|
Dr. Rajesh R Sannareddy
Department of Neurosurgery, Apollo Institute of Neurosciences, Jubilee Hills, Hyderabad - 500 033, Telangana
Source of Support: None, Conflict of Interest: None
|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 2020 Jul 4];67:1153-5. Available from: http://www.neurologyindia.com/text.asp?2019/67/4/1153/266250
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.
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. A high proliferative index (MIB-1 >20%), cellularity, and pleomorphism are features of aggressive schwannomas, and such patients require frequent clinicoradiological follow-up. 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%. 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. 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].,,
|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.,
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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[Figure 1], [Figure 2], [Figure 3], [Figure 4]