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Neurology India, Vol. 58, No. 4, July-August, 2010, pp. 651-653 Case Report Cerebellar glioblastoma multiforme with non-contiguous grade 2 astrocytoma of the temporal lobe in the same individual Salunke Pravin, Badhe Prerna, Sharma Alok Department of Neurosurgery, PGIMER, Chandigarh Correspondence Address:Department of Neurosurgery, PGIMER, Chandigarh-160 012, drpravin_salunke@yahoo.co.uk Date of Acceptance: 23-Oct-2009 Code Number: ni10172 PMID: 20739816 DOI: 10.4103/0028-3886.68696 Abstract Multicentric Gliomas, both supratentorial and infratentorial, with varying histopathological picture is extremely rare. We report a unique occurrence of such a combination in a 50-year-old man who presented with features of elevated intracranial pressure, ataxia and vertigo. Magnetic resonance imaging showed a diffuse non-enhancing lesion in the temporal lobe and insula and another non-contigous well defined enhancing lesion in the cerebellum. Both the lesions had mass effect. The lesions were decompressed; first the temporal lesion and then the cerebellar lesion. Histopathology revealed grade II astrocytoma in the temporal lobe and glioblastoma multiforme in the cerebellum. He recieved whole brain radiotherapy with which he showed symptomatic improvement and survived for 1.5 years.Keywords: Cerebellar glioblastoma, multicentric glioma, glioma surgery Introduction Cerebellar glioblastoma is an uncommon tumor [1],[2],[3] and its association with another noncontiguous tumor of a different histopathology grade makes it even more rare. [4] We report a patients with cerebellar glioblastoma and temporal grade II diffuse astrocytoma. Case Report A 50-year-old male presented with episodic vertex headache of five months duration. For eight days he had visual blurring associated with vomiting and vertigo associated and increase in headache. Patient was unable to sit up or walk without support. Higher mental functions were normal. On examination he had bilateral papilledema, gaze-evoked nystagmus, and truncal ataxia. Magnetic resonance imaging (MRI) of brain showed a diffuse infiltrating lesion in the right temporal lobe and insular region, hyperintense on T2 and hypointense on T1 with no contrast enhancement [Figure - 1]a. There was mass effect and evidence uncal herniation. Another well defined irregular contrast enhancing lesion (2.2 x 3.5 cms) with central area of necrosis was seen in the cerebellum close to the vermis pushing the fourth ventricle anteriorly and to the left side. The lesion was hypointense on T1, iso- to hyper-intense on T2 [Figure - 1]b and c. Non-contiguity was evident on T2 images, there was no spread of the temporal lesion to the cerebellar lesion. The hyperintensity was limited to temporal lobe and insula [Figure - 1]d He was operated first through a right temporal craniotomy and decompression of the temporal lesion was done. The lesion was soft, friable and grayish with mild to moderate vascularity. The tumor had a poor plane of cleavage from the surrounding parenchyma. The tentorial edge was seen clearly and there was not enough space to prevent herniation. A week later he was operated for the infratentorial lesion through midline suboccipital craniectomy. The mass was adherent to the dura at the level of foramen magnum. It was yellowish gray in color, firm at places with most of it being friable, vascular. There were places where it could be easily separated from the surrounding parenchyma. The fourth ventricle got opened during operation. The histopathology of the temporal mass revealed diffuse astrocytoma grade II with well differentiated fibrillary astrocytes in a background of loosely structured, microcystic tumor matrix. Tumor was moderately cellular without any mitotic activity, necrosis or vascular endothelial proliferation [Figure - 2]a. Cerebellar tumor was cellular, composed of poorly differentiated and pleomorphic cells with marked nuclear atypia and brisk mitotic activity. There were large areas of necrosis with pseudo palisiding of tumor cells around them [Figure - 2]b. Postoperatively, he received whole brain radiotherapy without boost to the posterior fossa (60-Gy dose in total per 30 fractions). He was able to walk with minimal support. The possible role of temozolomide was explained. However, he could not pursue the adjuvant therapy because of financial constraints. Imaging at one-year post surgery revealed some residual tumor in the temporal lobe without significant mass effect and no recurrence He continued to be in the same clinical status for 1.5 years and finally succumbed to chest infection and the exact cause of death could not be ascertained, as he could neither undergo radiological investigations at that time nor was an autopsy performed later. Discussion Multicentric glial lesions and multifocal gliomas are different entities. The latter results from dissemination or growth by established routes i.e., direct extension or metastasis through either cerebrospinal fluid pathway or hemotogenic spread. Multicentric gliomas should have location in different regions of the brain and have no connections or way of dissemination. [5],[6] Multiple sclerosis and von Recklinghausen′s disease predisposing risk factors for multiple gliomas. [5],[7] None of these diseases were evident in our patient. True multicentric gliomas are usually of the same histology type. However, in our patient the histology was different. Only ten such cases have been reported in the literature. [4] Further, multicentric gliomas in supra- and infratentorial regions are uncommon [7],[8] and cerebellar glioblastomas are quite uncommon. [1] With the non contiguous lesions in supra and infratentorial region, it will be appropriate to biopsy both the lesions. This helps in not only establishing the diagnosis but also reducing the tumor load for chemo-radiation. [6] For patients with both infra and supratentorial lesions it is difficult to decide which lesion should be operated first. Operating the infratentorial lesion first may have the risk of transtentorial herniation of the supratentorial lesion, especially in the temporal lobe. Radiotherapy to control infratentorial glioblastoma is controversial as compared to supratentorial gliomas. The extent of radiotherapy is not yet defined. Salazar is of the opinion that craniospinal irradiation with posterior fossa boost should be given as malignant cerebellar gliomas, particularly in children, behave somewhat similar to medulloblastomas. [9] In contrast, Kopelson has shown no benefit with radiotherapy to the entire neuroaxis in infratentorial glioblastomas, majority of his patients were adults. He suggested whole brain irradiation with or without posterior fossa boost. [2],[10] Craniospinal irradiation is a reasonable decision if cerebrospinal fluid (CSF) dissemination is considered as in children, who seem to have higher chances of CSF dissemination than adults. [3],[11] There is a tendency for limited posterior fossa radiation. The craniospinal axis dissemination is usually seen in the setting of local recurrences. This fact has been argued for advocating higher radiation doses around 5 Gy limited to posterior fossa. [3] Chemotherapy is not well established but is the only reasonable adjuvant treatment for very young children. [3],[11] However, there is a role for adjuvant and concomitant temozolomide with radiotherapy in prolonging the survival by few months in patients harboring glioblastoma. [12] When imaging reveals multiple brain tumors it is prudent to biopsy both the lesions before considering the diagnosis of metastatic brain tumors. In the co-occurrence of both infra and supratentorial lesions with mass effect it is appropriate to decompress the supratentorial lesion first. Whole brain radiotherapy with posterior fossa boost along with concomitant and adjuvant temozolomide is a good option and may help to increase the recurrence-free survival. References
Copyright 2010 - Neurology India The following images related to this document are available:Photo images[ni10172f2a.jpg] [ni10172f1c.jpg] [ni10172f2b.jpg] [ni10172f1b.jpg] [ni10172f1d.jpg] [ni10172f1a.jpg] |
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