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Indian Journal of Medical and Paediatric Oncology
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LETTER TO EDITOR
Year : 2011  |  Volume : 32  |  Issue : 2  |  Page : 123-124  

Extra-axial cerebello pontine angle medulloblastoma: A rare site of tumor


Department of Neurosurgery, Voluntary Health Services, Adyar, Chennai, Tamil Nadu, India

Date of Web Publication15-Nov-2011

Correspondence Address:
Manish Singh
Department of Neurosurgery, Voluntary Health Services, Adyar, Chennai - 600113, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-5851.89801

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How to cite this article:
Cugati G, Singh M, Symss NP, Pande A, Chakravarthy VM, Ramamurthi R. Extra-axial cerebello pontine angle medulloblastoma: A rare site of tumor. Indian J Med Paediatr Oncol 2011;32:123-4

How to cite this URL:
Cugati G, Singh M, Symss NP, Pande A, Chakravarthy VM, Ramamurthi R. Extra-axial cerebello pontine angle medulloblastoma: A rare site of tumor. Indian J Med Paediatr Oncol [serial online] 2011 [cited 2019 Jul 17];32:123-4. Available from: http://www.ijmpo.org/text.asp?2011/32/2/123/89801

Sir,

Medulloblastoma is a common tumor of the posterior fossa, representing 20-25% of all pediatric neoplasms. [1]

The tumor often occurs in the cerebellar vermis and at the apex of the fourth ventricle. [1],[2] There are only a few reported cases of cerebellopontine (CP)-angle medulloblastoma in the literature, with most being intra-axial. The extra-axial site of this tumor remains a rarity. [1],[3]

This 4-year-old girl presented with left hemicranial headache followed by facial asymmetry with deviation of angle of mouth for 1 month. There was no other significant history.

On clinical examination, higher intellectual functions were normal, both pupils were equal and reacting to light, visual acuity/visual fields were normal, fundus-no papilloedema, left lower motor neuron facial paresis, and left-sided sensory neural hearing loss, other cranial nerves normal. No stigmata of neurofibromatosis was noted. A computerised tomography (CT) scan of the brain showed contrast enhancing extra-axial lesion in the left CP angle centered around internal acoustic meatus [Figure 1]. CT bone window did not show enlargement of the internal acoustic meatus or hyperostosis [Figure 1]. Magnetic resonance imaging (MRI) of the brain showed CP angle lesion which was hypointense on T1W and hyperintense on T2W image [Figure 2]. The lesion was brilliantly enhancing with contrast, and no dural tail or canalicular component noticed [Figure 3]. She underwent left retromastoid craniectomy and total excision of the lesion. It was grayish, moderately vascular, and soft. There was a clear plane between the tumor and cerebellum, whereas it was adherent to dura and tent laterally. The HPE was confirmed as desmoplastic medulloblastoma [Figure 4] with the high MIB-1 labeling index and S-100 negativity.
Figure 1: CT scan brain plain with contrast shows left extra-axial contrast enhancing lesion

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Figure 2: MRI of the brain showed the CP angle lesion which was hypointense on T1W and hyperintense on T2W image

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Figure 3: MRI of the brain contrast study axial, sagital, and coronal section shows contrast enhancing extra-axial lesion

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Figure 4: HPE suggestive of desmoplastic medulloblastoma

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Medulloblastoma usually occurs in inferior medullary velum in the midline. [2] However rarely it may occur laterally in the cerebellar hemisphere in the pediatric and adult age group [1],[3],[4],[5] with most being intra-axial. The extra-axial site of this tumor remains a rarity. [1],[3] Origin of medulloblastoma may be either from germinal cells or their remnants situated at the end of the posterior medullary velum or from remnants of the external granular layer. [3],[6] Their development in the CPA may be from the remnants of the external granular layer in the cerebellar hemisphere, including the flocculus which faces the CP angle. [3],[6] In the CP angle, medulloblastomas though fifth, sixth, and eighth cranial nerves are frequently involved, these nerves were spared in this patient. [5],[7] CP angle medulloblastomas are very rare with nearly 36 cases published in the literature [1],[3],[5] of which only 10 are in adults. [1],[3],[5],[7] The lack of association with any cerebellar tissue and the extra-axial location of the tumor made our patient's case quite rare. However, they are likely under-reported owing to publication bias and must be considered in the differential diagnosis of extra-axial CP angle lesions.[8]

 
  References Top

1.Fallah A, Banglawala SM, Provias J, Jha NK. Extra-axial medulloblastoma in the cerebellopontine angle. Can J Surg 2009;52:E101-2.  Back to cited text no. 1
[PUBMED]  [FULLTEXT]  
2.Mohan M, Pande A, Vasudevan MC, Ramamurthi R. Pediatric medulloblastoma: A review of 67 cases at a single institute. Asian J Neurosurgery 2008;2:63-69.  Back to cited text no. 2
    
3.Singh M, Cugati G, Symss NP, Pande A, Vasudevan MC, Ramamurthi R. Extra axial adult cerebellopontine angle medulloblastoma: An extremely rare site of tumor with metastasis. Surg Neurol Int 2011;2:25.  Back to cited text no. 3
[PUBMED]  Medknow Journal  
4.Available from: http://www.surgicalneurologyint.com/text.asp?2011/2/1/25/77178 [Last Cited on 2011 Feb 26].  Back to cited text no. 4
    
5.Hubbard JL, Scheithauer BW, Kispert DB, Carpenter SM, Wick MR, Law ER Jr. Adult cerebellar medulloblastomas: The Pathological radiological and clinical disease spectrum. J Neurosurg 1989;70:536-44.  Back to cited text no. 5
    
6.Kumar R, Bhowmick U, Kalra SK, Mahapatra AK. Pediatric cerebellopontine angle medulloblastomas. J Pediatr Neurosci2008;3:127-30.  Back to cited text no. 6
  Medknow Journal  
7.Stevenson L, Echlim F. Nature and origin of some tumors of cerebellum: Medullobastoma. Arch Neurol Psychiatry 1934;31:93-109.  Back to cited text no. 7
    
8.Jaiswal AK, Mahapatra AK, Sharma MC. Cerebelloponitne angle medulloblastoma. J Clin Neurosci 2004;11:42-5.  Back to cited text no. 8
[PUBMED]  [FULLTEXT]  


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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