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  Table of Contents    
CASE REPORT  
Year : 2014  |  Volume : 57  |  Issue : 1  |  Page : 101-104
Pleomorphic xanthoastrocytoma with anaplastic features: A case report


1 Department of Histopathology, SRM Institute of Medical Sciences, Vadapalani, Chennai, India
2 Head of the Department of Neurosurgery, SRM Institute of Medical Sciences, Vadapalani, Chennai, India

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Date of Web Publication17-Apr-2014
 

   Abstract 

Pleomorphic xanthoastrocytoma has been considered as an astrocytic tumor with relatively favorable prognosis. It corresponds to WHO Grade-II neoplasm. Recently, several patterns with relatively poor prognosis have been recorded and a new concept of "PXA with anaplastic features" has been proposed. The present case is about a 9-year-old girl who presented with symptoms of recurrent headache, seizures and poor academic performance. MRI revealed left fronto-parietal irregular enhancing mass lesion with callosal involvement and right mid-brain arteriovenous malformation. Clinical and radiological examination was suggestive of a high grade glial neoplasm/PNET. A diagnosis of high grade glial neoplasm was rendered on the squash smears submitted for frozen sections based on the presence of spindle cells, admixed with pleomorphic bizarre, giant cells with multilobated nuclei showing few atypical mitosis and abundant eosinophilic cytoplasm. Frontal craniotomy with debulking of the tumor was performed and permanent sections revealed a biphasic glial neoplasm with spindle cells arranged in fascicles admixed with bizarre multinucleated giant cells showing abundant vacuolated and lipidized cytoplasm, nuclear hyperchromasia with intranuclear inclusions. Eosinophilic granular bodies, mitosis of 7/10 HPF, micro vascular proliferation, necrosis and invasion into the underlying brain parenchyma were noted. With these histomorphological findings a diagnosis of pleomorphic xanthoastrocytoma with anaplastic features was rendered.

Keywords: Anaplastic features, astrocytic tumor, pleomorphic xanthoastrocytoma

How to cite this article:
Niamathullah S, Sivaselvam S, Ghosh M, Ghosh S. Pleomorphic xanthoastrocytoma with anaplastic features: A case report. Indian J Pathol Microbiol 2014;57:101-4

How to cite this URL:
Niamathullah S, Sivaselvam S, Ghosh M, Ghosh S. Pleomorphic xanthoastrocytoma with anaplastic features: A case report. Indian J Pathol Microbiol [serial online] 2014 [cited 2019 Dec 9];57:101-4. Available from: http://www.ijpmonline.org/text.asp?2014/57/1/101/130913



   Introduction Top


Pleomorphic xanthoastrocytoma (PXA) is an uncommon astrocytic tumor representing <1% of all astrocytic tumors. It arises within the first three decades of life with no gender predilection. Thepostulated cell of origin being subpial astrocytes, multipotential neuroectodermal precursor cells or pre-existing hamartomatous lesion. It is supratentorial in location with predilection to the temporal followed by parietal lobe. PXA belongs to grade II of the WHO histological classification of tumors of the CNS and is therefore considered as a relatively benign entity among the astrocytic tumors. However, 9-20% of PXAs have been reported to undergo malignant transformation [1] and some of them exhibit anaplastic features at the first presentation. [2] Cases having several recurrences with poor prognosis have been reported recently and a newer concept of "PXA with anaplastic features'' has been proposed. We herein report a rare case of PXA with anaplastic features in a 9-year-old girl.


   Case Report Top


A 9-year-old girl presented with the symptoms of recurrent headache, seizures and poor academic performance. Magnetic resonance imaging studies revealed left fronto-parietal irregular enhancing mass lesion with callosal involvement and right mid-brain arteriovenous malformation [Figure 1]. Frozen section and squash preparation revealed spindle cells admixed with variably pleomorphic bizarre giant cells with single to multiple multilobated nuclei abundant cytoplasm and few mitosis [Figure 2]. The diagnosis of high grade glioma was rendered. Left frontal craniotomy with debulking of the tumor was done. Follow-up and permanent sections revealed a biphasic tumor showing multinucleated giant cells with abundant vacuolated lipidized cytoplasm [Figure 3] with intranuclear inclusions [Figure 3] as inset with arrow] admixed with spindle cells arranged in fascicles [Figure 3] with eosinophilic granular bodies [arrow in Figure 3]. Mitosis of 7/10 HPF [ shown by arrow in [Figure 4], necrosis [inset in [Figure 4], endothelial proliferation [Figure 5], with perivascular space involvement by tumor cells [inset in [Figure 5] and invasion of adjacent brain parenchyma [Figure 6], were noted. Reticulin stain done showed focal increased density and fragmentation of reticulin fibers [Figure 7]. Immunohistochemistry done showed diffuse positivity for GFAP [Figure 8a] and focal positivity for CD-34 [Figure 8]b. ki-67 proliferative index was 8% [Figure 8]c and synaptophysin was focally positive [Figure 8]d. Nuclear positivity for p53 was noted in 10% of the cells.
Figure 1: MRI showing left fronto-parietal irregular enhancing mass lesion

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Figure 2: Squash smear showing spindle cells admixed with
multinucleated giant cells


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Figure 3: Biphasic tumor showing multinucleated giant cells with lipidized cytoplasm and inset showing intranuclear inclusion (depicted by an arrow) admixed with spindle-shaped cells and eosinophilic granular bodies (depicted by arrows)

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Figure 4: Tumor with atypical mitosis shown by arrow and inset showing necrosis

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Figure 5: Tumor with endothelial proliferation and inset showing perivascular space involvement by tumor cells

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Figure 6: Tumor showing invasion into adjacent brain parenchyma

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Figure 7: Special stain showing fragmented reticulin

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Figure 8: (a) Diffuse positivity for GFAP. (b) Focal positivity for CD-34. (c) Ki-67 proliferative index of 8%. (d) Focal positivity for synaptophysin

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With the above histomorphological and immunohistochemical findings a diagnosis of PXA with anaplastic features was made, with a note on close follow-up following radiotherapy.


   Discussion Top


Pleomorphic xanthoastrocytoma with anaplastic features is a rarity. [2] A review of the available literature dating back to 1979 revealed only 15 well-documented cases of PXA with subsequent malignant transformation and 11 cases of primary PXA with anaplastic features till date. [3]

PXA (WHO Grade-II) is a biphasic tumor with characteristic histological pattern which includes (1) spindle cells arranged in fascicles, (2) bizarre pleomorphic multinucleated giant cells with lipidized vacuolated cytoplasm and intranuclear inclusions, (3) perivascular patchy lymphocytic infiltrate, (4) eosinophilic granular bodies, (5) dense reticulin stain invested around each cell.

Endothelial proliferation, high MIB-1 proliferative index, mitotic activity >5/10 hpf, and foci of necrosis are associated with an unfavorable prognosis. [4] WHO recommends the designation PXA with anaplastic features to denote lesions that demonstrate high mitotic activity of >5 mitosis/10 hpf and/or areas of necrosis. However, mitotic index is considered as the independent prognostic indicator for survival and recurrence. [5] PXA with anaplastic features has been associated with invasion of the brain parenchyma and with a tendency for perivascular space involvement. [5] The designations anaplastic PXA and PXA Grade III or IV is not recommended by WHO as this may provoke inappropriately aggressive treatment. [5]

The presence of necrosis is a significant feature in PXA that is recognized by WHO as PXA with anaplastic feature. Previous studies indicate that necrotic PXA are not and should not be termed glioblastomas. [6] PXA is an astrocytic tumor distinct from diffuse astrocytomas and to which criteria used in grading astrocytoma should not be applied.

In addition with increasing degree of anaplasia the formerly rich reticulin network becomes fragmented as in the present case or disappears completely.

Immunohistochemical positivity for GFAP supports exclusion of other non-glial neoplasms like malignant fibrous histiocytoma which resembles PXA. GFAP expression in PXA may be faint or focal although complete lack of expression has been described. [7],[8] The degree of immunoreactivity for CD-34 varied from tumor to tumor with WHO Grade II PXA showing widespread immunoreactivity than PXA with anaplastic features. Low fractions of less than 10% immunoreactive tumor cells have been reported in PXA with anaplastic features. [9] Reactivity of focal analysis of p53 and its expression in PXA with anaplastic features have been documented. [8] p53 mutation may predict subsequent anaplastic progression in recurrent tumors. The MIB-1 labeling index for PXA is less than 1%, whereas in PXA with anaplastic features the labeling index may be between 5% and 10%. [10]

The main alternate diagnostic consideration is giant cell glioblastoma; both of these cellular glioma contain variable number of pleomorphic astrocytes, though have significantly different therapeutic and prognostic implication. The histological findings of biphasic tumor with spindle cells admixed with multinucleated giant cells showing lipidized vacuolated cytoplasm, with intranuclear cytoplasmic inclusion, eosinophilic granular bodies and focal positivity for CD-34 and synaptophysin favor a diagnosis of PXA with anaplastic features. PXA with anaplastic features can transform into giant cell glioblastoma. [6]

Based on these findings and recommendations, we use the term PXA with anaplastic features as recommended by WHO in reporting the diagnosis of the current case to indicate the correlation with potentially more aggressive clinical behavior and we chose not to use the term anaplastic PXA as this designation may provoke inappropriate aggressive treatment. The high MIB-1 labeling index in the current case not only indicates more aggressive behavior but also higher incidence of recurrence.


   Conclusion Top


Although mitotic index and extent of resection appear to be the main predictor of recurrence free survival, review of more studied cases and longer period of follow-up will be essential to confirm our findings regarding prognostic factors affecting this unusual tumor. Analysis of p53 in pleomorphic xanthoastrocytoma with anaplastic features needs to be studied in detail due to limited literature.

 
   References Top

1.Chakrabarty A, Mitchell P, Bridges LR, Frank AJ. Malignant transformation in a pleomorphic xanthoastrocytoma-a report of two cases. Br J Neurosurg 1999;13:516-9.   Back to cited text no. 1
    
2.Lubansu A, Rorive S, David P. Cerebralanaplastic pleomorphic xanthoastrocytoma with meningeal dissemination at first presentation. Childs Nerv Syst 2004;20:119-22.  Back to cited text no. 2
    
3.Tekkok IH, Sav A. Anaplastic pleomorphic xanthoastrocytoma. Review of literature with reference to malignant potential. Pediatr Neurosurg 2004;40:171-81.  Back to cited text no. 3
    
4.Sharma A, Sharma D, Julka PK, Rath GK. Pleomorphic xanthoastrocytoma - a clinico-pathological review. Neurol Neurochir Pol 2011;45:379-86.  Back to cited text no. 4
    
5.Giannini C, Scheithauer BW, Burger PC, Brat DJ, Wollan PC, Lach B, et al. Pleomorphic Xanthoastrocytoma. What do we really know about it? Cancer 1999;85:2033-45.  Back to cited text no. 5
    
6.Pahapilli PA, Ramsay DA, Del Maestro RF. Pleomorphic Xanthoastrocytoma: Case report and analysis of the literature concerning the efficacy of resection and significance of necrosis. Neurosurgery 1996;38:822-9.  Back to cited text no. 6
    
7.Gelpi E, Popovic M, Presseur M, Budka H, Hainfellner J. Pleomorphic xanthoastrocytoma with anaplastic features presenting without GFAP immunoreactivity: Implications for differential diagnosis. Neuropathology 2005;25:241-6.  Back to cited text no. 7
    
8.Giannini C, Hebrink D, Scheithauer BW, Dei Tos AP, James CD. Analysis of p53 mutation and expression in pleomorphic xanthoastrocytoma. Neurogenetics 2001;3:159-62.  Back to cited text no. 8
    
9.Reifenberger G, Kaulich K, Wiestler OD, Blümcke I. Expression of CD 34 Antigens in Pleomorphic Xanthoastrocytoma. Acta Neuropathol 2003;105:358-64.  Back to cited text no. 9
    
10.Chang HT, Latorre JG, Hahn S, Dubowy R, Schelper RL. Paediatric Cerebellar pleomorphic xanthoastrocytoma with anaplastic features: A case of long term survival after multimodality therapy. Childs Nerv Syst 2006;22:609-13.  Back to cited text no. 10
    

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Correspondence Address:
Sadiya Niamathullah
No 21, Flat no T-1, Ameer Regency, Balaji Nagar 2nd street, Royapettah, Chennai - 14, India. Pin - 600 014.
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.130913

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]

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