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 Table of Contents  
LETTER TO THE EDITOR
Year : 2014  |  Volume : 10  |  Issue : 3  |  Page : 777-778

Cerebellar glioblastoma multiforme in an adult


1 Department of Neurosurgery, Datta Meghe Institute of Medical Sciences, Sawangi, Wardha, Maharashtra, India
2 Department of Pathology, Datta Meghe Institute of Medical Sciences, Sawangi, Wardha, Maharashtra, India
3 Department of Surgery, Datta Meghe Institute of Medical Sciences, Sawangi, Wardha, Maharashtra, India

Date of Web Publication14-Oct-2014

Correspondence Address:
Amit Agarwal
Department of Neurosurgery, Datta Meghe Institute of Medical Sciences, Sawangi, Meghe, Wardha - 442 004, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.139132

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How to cite this article:
Agarwal A, Bhake A, Kakani A, Hiwale KM, Enam SK. Cerebellar glioblastoma multiforme in an adult. J Can Res Ther 2014;10:777-8

How to cite this URL:
Agarwal A, Bhake A, Kakani A, Hiwale KM, Enam SK. Cerebellar glioblastoma multiforme in an adult. J Can Res Ther [serial online] 2014 [cited 2017 Oct 21];10:777-8. Available from: http://www.cancerjournal.net/text.asp?2014/10/3/777/139132

Dear Sir,

Although glioblastomas constitute approximately 15-20% of the entire intracranial tumors, [1] occurrence of primary cerebellar glioblastoma multiforme (GBM) in adults is extremely rare (about 1% of all intracranial glioblastomas) with only few cases reported in the literature. [2],[3],[4] A 47-year-old gentleman presented with the complaints of decreased hearing in right ear of 6 month's duration and giddiness of similar duration. He also noticed decreased eyelid movements on right side and decrease movement of face on right side with drooling of saliva while eating food. He was able to walk with support. He took some treatment at a local hospital without any relief. He was a chronic alcoholic and was treated for tuberculosis in 1994 and took antitubercular treatment for 6 months. Physical examination and laboratory tests were within normal limits. Neurological examination revealed nystagmus, right-sided 7 th nerve lower motor neuron type of palsy, decreased hearing in the right ear, and impaired gag reflex on right side. There was presence of right-sided cerebellar signs including cerebellar ataxia, tandem gait, dysmetria, and dysdiadochokinesia. An investigation with brain computed tomography (CT) and magnetic resonance imaging (MRI) scan showed a heterogenic contrast-enhancing expanding process in the right cerebellar hemisphere [Figure 1]. The MRI further showed an intraaxial, infiltrative, heterogenic mass localized in the right cerebellar hemisphere with extension into the right middle and superior cerebellar peduncle and brainstem. The lesion was hypointense on T1-weighted (T1W) images and hyperintense on T2W and fluid-attenuated inversion recovery (FLAIR) images with poorly-defined borders and mild peritumoral edema, and after the gadolinium administration there was ill-defined, heterogeneous enhancement. There was evidence of mass effect on the fourth ventricle, but no hydrocephalus was present. To rule out the metastatic lesion, the patient underwent chest X-ray and abdominal sonography; but these studies were normal. The patient underwent right retromastoid suboccipital craniectomy. During surgery a yellowish tumor with necrotic change and abnormal vessels that had indistinct margins from adjacent normal white matter in the deep part of cerebellum was identified. A partial decompression of the tumor was performed. The postoperative course was uneventful and his symptoms and neurologic signs gradually improved. Histological examination showed a cellular tumor, consistent with glioblastoma, composed of elongated, spindle-shaped cells with irregular, moderately pleomorphic nuclei, as well as proliferative blood vessels and necrosis [Figure 2]. Upon recovery from surgery, the patient underwent received radiotherapy and chemotherapy (temozolomide). However, he survived only for 8 months.
Figure 1: Magnetic resonance imaging (MRI) axial images revealed the presence of hypo- and hyperintense lesions in the right cerebellum in T1-weighted (a), T2-weighted (b), and fluid-attenuated inversion recovery (FLAIR) images (c). Note the enhancement of the lesion after contrast administration (d)

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Figure 2: Histological examination of the removed tumor specimen suggestive of glioblastoma multiforme

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Cerebellar glioblastoma is a very malignant tumor, with rapid onset of symptoms and has a poor prognosis with median overall survival of approximately 10 months, with a very less 1-year overall survival. [3] The majority of the cerebellar glioblastomas occur in adult age range with male preponderance. [3] It has been estimated that by tissue volume, if the average human cerebrum is taken as 1,034 mL and cerebellum as 114 mL, then relative cerebellar volume is 11% of cerebral volume, [5] if all other factors were equal then it is expected that 11% of all glioblastomas would occur in the cerebellum; however, the incidence of primary cerebellar glioblastomas is less than 0.5% of all cases (20 times lower than expected). [3],[4] In most of the studies, the authors failed to show the explanation for the rarity of cerebellar GBM, [6] but it has been proposed that there is a lesser tendency of cerebellar astrocytes to suffer malignant transformation description. [3],[4] Primary glioblastomas usually develop in older patients, while secondary glioblastomas develop in younger patients and contain TP53 mutations as the earliest detectable change. [3] Recently, there is evidence pointing to signaling pathwayas an initiating and growth-enhancing path in glioblastomas. [4] The major clinical manifestations relates to cerebellar dysfunction and the existence of a mass lesion in the posterior fossa (i.e. unsteady gait, imbalance, headache, dizziness, slurred speech nausea, and vomiting); none of these are specific for glioblastoma. [3],[6] CT scan can be used for the initially evaluation for these mass lesions; [7] however, because of good tissue resolution, lack of major bone artifacts, and multiplanar capabilities; MRI is far superior to CT for evaluation of these patients. [8] On MRI cerebellar glioblastomas usually appear hypointense in T1 and hyperintense in T2 and reveal heterogeneous and irregular enhancement after contrast, mild-to-moderate perilesional edema and deformity of the fourth ventricle. [8] MR diffusion/perfusion imaging and MR spectroscopy examinations would further facilitate the characterization of the lesions and the differential diagnosis. [8] Subtotal excision of the lesion and pathological analysis provides the definite diagnosis of GBM. [8],[9] This can be followed by radiotherapy and the temozolomide chemotherapy. [10]



 
 > References Top

1.
Bigner DD, McLendon RE, Bruner JM. Russell and Rubinstein's Pathology of Tumors of the Nervous System. 7 th ed. London: Hodder Arnold; 2006.  Back to cited text no. 1
    
2.
Hur H, Jung S, Jung TY, Kim IY. Cerebellar glioblastoma multiforme in an adult. J Korean Neurosurg Soc 2008;43:194-7.  Back to cited text no. 2
    
3.
Grahovac G, Tomac D, Lambasa S, Zoric A, Habek M. Cerebellar glioblastomas: Pathophysiology, clinical presentation and management. Acta Neurochir (Wien) 2009;151:653-7.  Back to cited text no. 3
    
4.
Kast RE. Why cerebellar glioblastoma is rare and how that indicates adjunctive use of the FDA-approved anti-emetic aprepitant might retard cerebral glioblastoma growth: A new hypothesis to an old question. Clin Transl Oncol 2009;11:408-10.  Back to cited text no. 4
    
5.
Ekinci N, Acer N, Akkaya A, Sankur S, Kabadayi T, Sahin B. Volumetric evaluation of the relations among the cerebrum, cerebellum and brain stem in young subjects: A combination of stereology and magnetic resonance imaging. Surg Radiol Anat 2008;30:489-94.  Back to cited text no. 5
    
6.
Chamberlain MC, Silver P, Levin VA. Poorly differentiated gliomas of the cerebellum. A study of 18 patients. Cancer 1990;65:337-40.  Back to cited text no. 6
    
7.
Bhimani S, Virapongse C, Spencer D, Kim J. CT appearance of cerebellar glioblastoma multiforme. J Comput Assist Tomogr 1983;7:889-91.  Back to cited text no. 7
    
8.
Demir MK, Hakan T, Akinci O, Berkman Z. Primary cerebellar glioblastoma multiforme. Diagn Interv Radiol 2005;11:83-6.  Back to cited text no. 8
    
9.
Gupta V, Goyal A, Sinha S, Singh AK, Tatke M, Kumar S, et al. Glioblastoma of the cerebellum. A report of 3 cases. J Neurosurg Sci 2003;47:157-64.  Back to cited text no. 9
    
10.
Saito T, Hama S, Kajiwara Y, Sugiyama K, Yamasaki F, Arifin MT, et al. Prognosis of cerebellar glioblastomas: Correlation between prognosis and immunoreactivity for epidermal growth factor receptor compared with supratentorial glioblastomas. Anticancer Res 2006;26:1351-7.  Back to cited text no. 10
    


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