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Year : 2003  |  Volume : 51  |  Issue : 2  |  Page : 246-247

Cerebellar hemisphere, an uncommon location for pleomorphic xanthoastrocytoma and lipidized glioblastoma multiformis

Department of Neuropathology, Sree Chitra Tirunal Institute for Medical Sciences & Technology, Thiruvananthapurum-695011

Correspondence Address:
Department of Neuropathology, Sree Chitra Tirunal Institute of Medical Sciences & Technology. Thiruvananthapurum. Kerala
[email protected]

  »  Abstract

Lipidized glioblastoma multiformis (LGB) and pleomorphic xanthoastrocytoma (PXA) are often supratentorial in location and occur in the second to fourth decade. This report presents two young patients, one having LGB and the other having PXA in the cerebellum. Histological differentiation between LGB and PXA is discussed.

How to cite this article:
Kumar S, Retnam T M, Menon G, Nair S, Bhattacharya R N, Radhakrishnan V V. Cerebellar hemisphere, an uncommon location for pleomorphic xanthoastrocytoma and lipidized glioblastoma multiformis . Neurol India 2003;51:246-7

How to cite this URL:
Kumar S, Retnam T M, Menon G, Nair S, Bhattacharya R N, Radhakrishnan V V. Cerebellar hemisphere, an uncommon location for pleomorphic xanthoastrocytoma and lipidized glioblastoma multiformis . Neurol India [serial online] 2003 [cited 2023 Jan 29];51:246-7. Available from: https://www.neurologyindia.com/text.asp?2003/51/2/246/1096

Lipidized glioblastoma multiformis (LGB) and pleomorphic xanthoastrocytoma (PXA) are two distinct clinicopatbological entities. Both the tumors occur in the second to fourth decade and are often supratentorial in location. Cerebellar location is extremely uncommon. This report describes the characteristic histopathological features and emphasizes the distinguishing histopathological features between PXA and LGB.

   »   Case Report

Case 1
A 15-year-old male was admitted with history of headache and vomiting of one-month duration. Other than papilloedema, there was no neurological deficit. Investigations revealed a right cerebellar solid mass with cystic degeneration. There was obstructive hydrocephalus. Right retro-mastoid craniectomy and subtotal resection of the tumor was done. The tumor was moderately vascular and the cyst contained xanthochromic fluid. Histological examination showed an admixture of spindle cells and mono- or multi-nucleated giant cells. Neoplastic cells had nucleoli of variable size and shape, some of the cells showed characteristic intracellular accumulation of lipid vesicles [Figure - 1]. Eosinophilic granular bodies were also seen in between the neoplastic cells. Characteristic xanthomatous cells surrounded by basement membrane were stained positive for reticulin. There was no necrosis, microvascular proliferation, or mitotic figures. Glial fibrillary acidic protein (GFAP) immunostaining showed varying degree of immunoreactivity in the neoplastic cells. Based on these histopathological features a diagnosis of PXA was made.

Case 2
A 12-year-old child was admitted with a history of headache, vomiting and ataxia for two weeks. Neurological examination revealed papilloedema and left cerebellar signs. MRI showed a hypointense mass lesion having areas of necrosis with surrounding edema in the left cerebellar hemisphere. The patient underwent midline sub-occipital craniectomy and partial resection of the tumor. The tumor was moderately vascular with areas of necrosis and hemorrhage. Leptomeninges over the tumor were thickened and vascular. Hematoxylin and eosin-stained sections showed a cellular tumor with striking nuclear pleomorphism. The cell nuclei showed variation in size and shape [Figure - 2]. The lesion also showed an admixture of spindle cells, multi-nucleated giant cells, as well as lipidized and non-lipidized neoplastic cells. However, the most characteristic features were satellite necrosis, pseudo-palisading and numerous mitotic figures. Microvascular proliferation was also present. Neoplastic cells showed varying degree of immunoreactivity for GFAP. The reticulin stain showed deposition of reticulin fiber in the peri-advential region of the blood vessels. Based on histopathology features a diagnosis of lipidized GBM was made.

   »   Discussion Top

Pleomorphic xanthoastrocytoma (PXA) was recognized as a distinctive astrocytic tumor in 1979.[1] The vast majority of PXA, occur in a supratentorial location with a striking predilection for the temporal lobe. Location outside the supratentorial compartment is extremely uncommon. Only five patients with cerebellar location have been reported.[2],[3],[4],[5],[6] Often the tumor occurs in the second and third decade and the typical clinical presentation is with protracted seizures. Histological characteristics include marked cellular pleomorphism, multi-nucleated cells with bizarre hyperchromatic nuclei and fusiform cells. However, the most characteristic feature is the presence of intracellular multi-lobulated lipid vesicles. The amount of lipidized cells is variable. Other features include lymphocytic perivascular cuffing, granular eosinophilic droplets and reticulin-rich stroma. Mitotic figures and necrosis are usually absent. PXA is generally regarded as a lower grade tumor[7] and following surgery the prognosis is generally good with good functional outcome.[8] However, recurrence and malignant transformation have also been reported.[9],[10]
Glioblastoma multiforme is the most common supratentorial malignant astrocytic tumor in adults. The cerebellum is an uncommon location. The salient histopathological features include cellular pleomorphism, nuclear atypia, brisk mitotic activity, multi-nucleated cells, microvascular proliferation and necrosis. Occasional lipidization of the neoplastic cells in glioblastoma has been reported.[11],[12] These features may be mistaken for PXA when lipidization is extensive and the location of the tumor is superficial, more so if the patient is young. The salient distinguishing features that differentiate LGB from PXA are: a) presence of brisk mitotic activity; b) necrosis; c) absence of characteristic reticulin-rich stroma in and around tumor cells. Malignant changes including mitotic figures and necrosis occur in recurrent PXA and may mimic an anaplastic astrocytoma or even a glioblastoma multiforme[13] and a clear distinction may be difficult on histopathological grounds alone. 

  »   References Top

1.Kepes JJ, Rubinstein LJ, Eng LF. Pleomorphic xanthoastrocytoma: a distinctive meningocerebral glioma of young subjects with relative favorable prognosis. A study of 12 cases. Cancer 1979;44:1839-52.  Back to cited text no. 1    
2.Glasser RS, Rojiani AM, Parker MJ, Bakini TA. Delayed occurrence and cerebellar pleomorphic xanthoastrocytoma after supratentorial pleomorphic xanthoastrocytoma removal. Case report. J Neurosurg 1995;82:116-8.  Back to cited text no. 2    
3.Luidnoe CF, Cappelen J, Keper JJ. Pleomorphic xanthoastrocytoma as-a component of cerebellar ganglioglioma. A case report. Neurosurgerv 1992;31:353-5.  Back to cited text no. 3    
4.Perry A, Giamimi C, Scheithauer BW, Rosiami AM, et al. Composite pleomorphic xanthoastrocytoma and gangliostoma. Report of four cases and review of literature. Am J Sung Pathol 1997;21:763-71.  Back to cited text no. 4    
5.Powell SZ, Yachanis AT, Rorke Lb, et al. Divergent differentiation in pleomorphic xanthoastrocytoma evidence for a neuronal element and possible relationship to ganglion cell tumours. Am J Surg Pathol 1996;20:80-5.  Back to cited text no. 5    
6.Wasdohil XA, Scheithauer BW, Andrews BT, et al. Cerebellar pleomorphic xanthoastrocytoma Case report. Neurosurg 1994;35:947-51.  Back to cited text no. 6    
7.Kleihues P, Burger PC, Scheithauer BW. New WHO classification of brain tumours. Brain Pathol 1993;3:255-68.  Back to cited text no. 7    
8.Palma L, Maleci A, Lauro GM. Pleomorphic xanthoastrocytoma with 18year survival. A case Report. J Neurosurg 1985;63:808-10.  Back to cited text no. 8    
9.Bayindir CKN, Kanasu A, Kasasoglu D. Anaplastic pleomorphic xanthoastrocytoma: Child Nervs System 1997;13:50-6.  Back to cited text no. 9    
10.Prayson RA, Monnis HH, Anaplastic pleomorphic xanthoastrocytoma. Arch Pathol Lab Mod 1998;122:1082-7.  Back to cited text no. 10    
11.Kepes JJ, Rubinstein LJ. Malignant gliomas with heavily lipidized (foamy) tumor cells: a report of three cases with immunoperoxidase study. Cancer 1981;47:2451-9.   Back to cited text no. 11    
12.Rosenblnm MK, Erolandson RA, Budzilogsch GN. The lipid rich epitheliod glioblastoma. Am J Surg Pathol 1991;15:925-34.  Back to cited text no. 12    
13.Kepes JJ, Rubinstein LJ, Ansbarder L, Schreiber DJ. Histopathological features of recurrent xanthoastrocytoma-further corroboration of the filial nature of the neoplasm. A study of three casgs. Acta Neuropathol 1989;78:585-93.  Back to cited text no. 13    


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