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CASE REPORT
Year : 2006  |  Volume : 1  |  Issue : 1  |  Page : 21-23
 

Cerebellar glioblastoma multiforme presenting as a cerebellopontine angle mass


1 Department of Pathology, Lady Hardinge's Medical College, Shaheed Bhagat Singh Road, New Delhi - 110 001, India
2 Departments of Neurosurgery and Neuropathology, Neurosciences Centre, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110029, India

Correspondence Address:
Awadhesh Kumar Jaiswal
Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow - 226 014, UP
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1817-1745.22943

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  Abstract 

Cerebellar glioblastoma multiforme (GBM) is a highly malignant brain tumour, which is exceedingly rare and such tumour presenting as cerebellopontine angle (CPA) mass is even rarer. We here discuss the case of a 15-year-old girl who had cerebellar GBM presenting as CPA mass that resembled meningioma on CT scan and was managed successfully with minimal problems.


Keywords: Cerebellum, CPA lesion, glioblastoma multiforme


How to cite this article:
Jindal A, Jaiswal AK, Jaiswal S, Pandey P, Mahapatra AK, Sharma MC. Cerebellar glioblastoma multiforme presenting as a cerebellopontine angle mass. J Pediatr Neurosci 2006;1:21-3

How to cite this URL:
Jindal A, Jaiswal AK, Jaiswal S, Pandey P, Mahapatra AK, Sharma MC. Cerebellar glioblastoma multiforme presenting as a cerebellopontine angle mass. J Pediatr Neurosci [serial online] 2006 [cited 2017 Mar 22];1:21-3. Available from: http://www.pediatricneurosciences.com/text.asp?2006/1/1/21/22943


The most common type of cerebellar neoplasm is the pilocytic astrocytoma; anaplastic astrocytoma and GBM in the cerebellum are rare, which have appeared only in case reports. Of these, those presenting as CPA mass are even more uncommon. [1],[2],[3] Primarily the tumours presenting as CPA are extra-axial; and rarely, a tumour arising from the brain parenchyma may become exophytic and manifest in the CPA. Acoustic tumours comprise 80-90% of CPA lesions and the largest portion of the remaining are meningiomas, epidermoids, lipomas, metastasis and other uncommon tumours. [5],[6],[7],[8] Regardless of the site of origin, tumours in the CPA present with sign and symptoms resulting from compression of fifth, seventh and eighth cranial nerves and lateral aspect of pons and cerebellar peduncle. Intra-axial lesions of posterior fossa generally differ from extra-axial lesions by having more profound deficits (e.g, hydrocephalus, long tract signs, rapid onset and progression of multiple cranial nerves). Imaging by MRI is usually of great help in differentiating extra-axial and intra-axial lesions. As the surgical and adjunctive management differs in both types, preoperative diagnosis is of great importance.


  Case Report Top


A 15-year-old girl presented with a 2-month history of giddiness, headache and vomiting for the last one month. She had rapid deterioration thereafter and was admitted to the hospital in emergency. On examination, she was conscious but complained of severe headache and fundi showed papilloedema bilaterally. Cranial nerve examination revealed bilateral sixth paresis with left seventh minimal paresis and reduced hearing on the left side. In addition, she had bilateral cerebellar signs, left more than right. Although her vitals were stable, she was dehydrated and her clinical examination fitted the working diagnosis of CPA meningioma except for the short duration of complaints. She had come with a CT scan done outside, which showed a rounded, apparently dural-based, lesion that was hyper-dense on the non-contrast CT and enhanced homogeneously on contrast [Figure - 1]. Considering her sick condition, she was not investigated further and after correcting the dehydration with intravenous fluids, she was taken up for surgery next morning. Left retromastoid craniectomy was done; brain became lax after draining CSF. After gentle retraction of cerebellum, a pink, fleshy, moderately vascular tumour was seen, which was apparently encapsulated on the lateral aspect but had indistinct margins on the cerebellar side. To our surprise, seventh and eighth nerve complex was seen separate from the tumour. At this time, a frozen section was sent, which was reported as medulloblastoma (?). A gross total resection was done using microsurgical technique preventing with minimal handling of cranial nerves. Microscopic examination of the tumour showed oval and polygonal cells with moderate amount of cytoplasm, admixed with tumour giant cells. There were areas of necrosis and moderate endothelial proliferation, suggestive of glioblastoma multiforme [Figure - 2]. Immunohistochemistry for GFAP was positive but negative for synaptophysin.

Postoperative course was uneventful and her hearing and facial paresis improved immediately and sixth nerve paresis improved over the next few days. Cerebellar signs worsened on the left side, as expected, but improved gradually over a period of 2 w. She was referred to the Department of Radiotherapy for further management. She was doing well at follow-up of 3 months.


  Discussion Top


This case demonstrates that an exophytic cerebellar tumour can closely resemble both clinical and radiological character of the extra-axial mass in the CPA. It is of great importance to differentiate the two before surgery. For extra-axial lesions like acoustic, complete excision is curative, but for intra-axial tumours like medulloblastomas and GBM, surgical approach varies from gross total to subtotal resection followed by postoperative radiotherapy and chemotherapy. Aggressive surgery particularly in exophytic brain stem masses may be devastating.[3]

Primary cerebellar or brainstem tumours may occupy CPA through either of the two paths: lateral extension from the fourth ventricle through foramen of Luschka or direct exophytic growth from the site of origin.[3] It is for the prudent clinician to realize the subtle clinical features of CPA lesions that suggest an intra-axial mass. A short duration of symptoms and rapid progression to brainstem dysfunction and hydrocephalus (like in the present case) are suggestive of a parenchymal lesion rather than extra-axial mass. Also an intra-axial lesion should be suspected when hydrocephalus accompanies a moderate-size CPA tumour. There remains a substantial overlap in the clinical presentation of intra-axial and extra-axial lesions in the CPA and radiological investigations may prove to be of great help. Irregularity of the interface between the mass and adjacent brain is the most important feature on MRI to distinguish the two; however, one should remember that with longstanding extra-axial lesion, the interface might be blurred because of encephalomalacia.[3],[4] Bright signal intensity within the adjacent brain and dilation of lateral recess of fourth ventricle may indicate intra-axial lesion. Kulkarni[1] et al reported four cases of cerebellar GBM and in their patients, the CT and MRI showed relatively little oedema and invariable discrete border with normal brain. Precontrast CT density was also variable and not specific. They showed that malignant tumours are of large size at the time of presentation (>40 mm in their series) and have short duration of symptoms. Radiological picture alone may not be a perfect guide to the diagnosis. However in our case, we did not have the advantage of MRI as the child was sick and routine MRI would have delayed surgical treatment and we thought that the clinical picture corresponds to the radiological diagnosis and probably, MRI was thought not necessary. This case gives a lesson not to ignore the subtle clinical features of malignancy and also not to underestimate our clinical skills when confronted with strong radiological evidence.

 
  References Top

1.Kulkarni AV, Becker LE, Jay V, Armstrong DC, Drake JM. Primary cerebellar glioblastoma multiforme in children. Report of four cases. J Neurosurg 1999;90:546-60.  Back to cited text no. 1  [PUBMED]  
2.Bristot R, Raco A, Vangelista T, Delfini R. Malignant cerebellar astrocytomas in childhood. Experience of four cases. Childs Nerv Syst 1998;14:532-6.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.Ahn MS, Jackler RK. Exophytic brain tumors mimicking primary lesions of the cerebellopontine angle. Laryngoscope 1997;107:466-71.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Kuriowa T, Numaguchi Y, Rothman MI, Zoarski GH, Morikawa M, Zagardo MT, et al. Posterior fossa glioblastoma multiforme: MR Findings. AJNR AM J Neuroradiol 1995;16:583-9.  Back to cited text no. 4    
5.Brackmann DE, Bartels LJ. Rare tumors of the cerebellopontine angle. Otolarygngol Head Neck Surg 1980;88:555-9.  Back to cited text no. 5  [PUBMED]  
6.Moffat DA, Saunders JE, Mcelveen JT, Mcferran, Hardy DG. Unusual cerebellopontine angle tumors. J Laryngol Otol 1993;107:1087-98.  Back to cited text no. 6    
7.Lalwani AK. Menningiomas, epidermoids and other nonacoustic tumors of the cerebellopontine angle. Otolaryngol Clin North Am 1992;25:707-28.  Back to cited text no. 7  [PUBMED]  
8.Bartels LJ, Arrington JR. Rare tumors of the cerebellopontine angle. In : Jackler RK, Brackmann DE, ediutors. Neurotology. Mosby-Year Book: Chicago; 1994. p. 835-61.  Back to cited text no. 8    


    Figures

[Figure - 1], [Figure - 2]


This article has been cited by
1 A retrospective study of primary cerebellar glioblastoma multiforme in adults
Gopalakrishnan, C.V. and Dhakoji, A. and Nair, S. and Menon, G. and Neelima, R.
Journal of Clinical Neuroscience. 2012; 19(12): 1684-1688
[Pubmed]
2 A retrospective study of primary cerebellar glioblastoma multiforme in adults
C.V. Gopalakrishnan,Amit Dhakoji,Suresh Nair,Girish Menon,R. Neelima
Journal of Clinical Neuroscience. 2012; 19(12): 1684
[Pubmed] | [DOI]



 

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