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Year : 2010  |  Volume : 58  |  Issue : 5  |  Page : 771-773

Primary spinal pleomorphic xanthoastrocytoma

1 Department of Neurosurgery, Army Hospital (R&R), Delhi Cantt., New Delhi, India
2 Department of Pathology, Army Hospital (R&R), Delhi Cantt., New Delhi, India
3 Department of Oncology, Army Hospital (R&R), Delhi Cantt., New Delhi, India

Date of Acceptance22-Jul-2010
Date of Web Publication28-Oct-2010

Correspondence Address:
Maneet Gill
Department of Neurosurgery, Army Hospital (R&R), Delhi Cantt., New Delhi - 110 010
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.72193

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 » Abstract 

Pleomorphic xanthochromic astrocytoma primarily of the spinal cord is a rare entity. The case is possibly the fifth such report. Complete surgical excision is the essential requirement for good survival. In the absence of any clearly laid down protocols of adjuvant treatment, anecdotal reports support treatment with chemotherapy alone or both chemotherapy and radiotherapy.

Keywords: Glial fibrillary acidic protein, leptomeninges, pleomorphic xanthoastrocytoma

How to cite this article:
Gill M, Pathak H C, Madan R, Bhattacharya S, Choudhary G S. Primary spinal pleomorphic xanthoastrocytoma. Neurol India 2010;58:771-3

How to cite this URL:
Gill M, Pathak H C, Madan R, Bhattacharya S, Choudhary G S. Primary spinal pleomorphic xanthoastrocytoma. Neurol India [serial online] 2010 [cited 2022 Nov 28];58:771-3. Available from: https://www.neurologyindia.com/text.asp?2010/58/5/771/72193

 » Introduction Top

Pleomorphic xanthoastrocytomas (PXAs) are relatively rare central nervous system (CNS) tumors. Though first described in 1979 by Kepes et al.,[1] these tumors are added to the WHO classification of tumors of the CNS only in 1993. [2],[3] Within the CNS, they are primarily supratentorial in location, most commonly in the temporal lobe, followed by the cerebellum. [1],[3],[4],[5] Spinal location of PXA is extremely rare. The first case of spinal PXA was reported in 1994. [6] This was followed by the second case in 2006. [7] In this report we describe probably the fifth such case.

 » Case Report Top

A 23-years-old female, who had been operated for a spinal tumor at another hospital , presented to our center with complaints of gradually increasing weakness in both lower limbs and loss of bladder and bowel control. She had been operated upon seven months prior to reporting to us and was symptomatic for a month prior to the first surgery. Clinically she had grade 0/5 paraplegia, decreased sensation below D12 dermatomal level and bladder-bowel incontinence. Magnetic resonance imaging (MRI) of dorsolumbar spine revealed an intramedullary lesion extending from D11-12 space to L1-2 space [Figure 1]a. Operative findings from the first surgery were not available, and comparison of the pre-operative and post-operative MRIs showed that the first excision was a minimal one. She was taken up for excision, and the tumor was exposed through a laminectomy D12 to L2. Intraoperatively the tumor was firm, adherent to the meninges and without a very distinct peritumoral plane. The tumor was amenable to dissection with Cavitron Ultrasonic Suction Apparatus (CUSA) and was totally removed, as confirmed by post-operative scan [Figure 1]b. Histopathology revealed large pleomorphic spindle and giant cells with bizarre multinucleated forms [Figure 2]a and sarcomatoid features with monstrous nuclei and hyalinized cytoplasm [Figure 2]b. Necrotic and mitotic features were absent. Immunohistochemical studies were positive for glial fibrillary acidic protein (GFAP), and the diagnosis given was pleomorphic xanthoastrocytoma . After discussion in the tumor board, the patient was administered temozolamide based chemotherapy. She is on regular follow-up; and at the last visit 6 months after surgery, she had regained sphincter control, and repeat MRI did not show any significant residual/recurrent lesion, but only post-operative changes.
Figure 1: a: Post-operative MRI after first surgery showing significant residual tumor from D11-12 space to L1-2 space.
b: MRI after second surgery showing tumor excision.

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Figure 2: a: Low-power view (× 10) of H and E stained section of tumor showing spindle and giant cells, including bizarre, multinucleated forms.
b: High-power view (× 40) of H and E stained section of the same tumor, highlighting the sarcomatoid features with monstrous nuclei and hyalinized cytoplasm due to lipid accumulation.

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 » Discussion Top

The age and sex distribution and location of the lesion in the previous reported four patients [6],[7],[8],[9] and the present patient are given in [Table 1]. Our patients seems to be the fifth case of primary spinal PXA being reported. Of the five patients, in four the spinal segmental location of the tumor was dorsal spine.
Table 1: Summary of literature review of reported cases of primarily spinal PXA

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Debate regarding the pathological nature of PXA continues. [5] However, most of the literature suggests a subpial astrocytic based origin. [10],[11] Typical histological features on hematoxylin and eosine stain include large pleomorphic giant and multinucleated cells with vacuolization and xanthomatous changes in some cells. There may also be some fusiform cells showing nuclear hyperchromatism. However, necrosis, vascular proliferation and mitotic figures are typically absent. A reticulin stain shows granular deposits between the tumor cells and continuous fibers around the blood vessels. Immunohistochemical studies show strong positivity for GFAP. [10],[11] Typically the tumor occurs between the ages of 7 and 25 years, but older patients have also been reported. [6] The tumors are most commonly supratentorial in location , with the temporal lobe being the commonest site. [1],[3],[4],[5] The commonest symptoms are seizures and headache. [8] Spinal PXAs are very rare. [6],[7] Other rare sites, like the retina, have also been reported. [12] Aggressive and anaplastic variants with extensive craniospinal leptomeningeal dissemination to sites such as the optic chiasma and infundibulum have also been reported. [13],[14],[15] Malignant transformation into glioblastoma with spinal dissemination in cases of partially resected cranial tumors is known. [16] Radiological evaluation reveals a hypodense or isodense lesion on the computerized tomography scan. MRI shows predominantly hypo-intense or iso-intense T1 and hyperintense T2 images. Contrast enhancement is the norm on both CT and MRI. [9]

Available literature gives sparse information regarding treatment protocols for spinal cord PXAs, primarily because of anecdotal nature of such reported cases. However more information is available regarding treatment modalities for cranial PXAs. Typically these tumors are astrocytic with favorable prognosis, especially early surgical removal has been good. [5],[7],[8] Recurrence is reported in around 20% of cases. [7] Early total surgical removal is imperative for good prognosis because recurrent lesions are more diffusedly infiltrative and more difficult to remove. [3],[17] Moreover, residual lesions are known to transform into aggressive anaplastic variants and to have extensive leptomeningeal dissemination. [16] Though some authors report good disease-free survival, about 85%, with gross total resection without adjuvant therapy, [8] others recommend adjuvant chemotherapy with 12 cycles of carboplatin and vincristine [13] or both chemotherapy and radiotherapy [16] for good survival. This is more relevant in disseminated or recurrent lesions or anaplastic variants of the disease. Survival figures for spinal lesions are not reported; but for supratentorial lesions, survival varying from 7 months to 25 years is reported.

 » References Top

1.Kepes JJ, Rubinstein LJ, Eng LF. Pleomorphic xanthoastrocytoma: A distinctive meningocerebral glioma of young subjects with relatively favorable prognosis. A study of 12 cases. Cancer 1979;44:1839-52.  Back to cited text no. 1
2.Kleihues P, Burger PC, Scheithauer BW. Astrocytic tumors, in Histological typing of tumors of the Central Nervous System. 2 nd ed. Berlin: Springer-Verlag; 1993. p.11-5.  Back to cited text no. 2
3.Kepes JJ. Pleomorphic xanthoastrocytoma: The birth of a diagnosis and a concept. Brain Pathol 1993;3:269-74.  Back to cited text no. 3
4.Dave KG, Maxwell RE, Seljeskog E, Sung JH. Pleomorphic xanthoastrocytoma-report of 4 cases, with MRI appearances and literature review. Br J Neurosurg 1994;8:681-9.  Back to cited text no. 4
5.Gianninni 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.Herpers MJ, Freling G, Beuls EA. Pleomorphic xanthoastrocytoma in the spinal cord. Case report. J Neurosurg 1994;80:564-9.  Back to cited text no. 6
7.Masaya N, Kazuhiro C, Morio M, Eiji I, Yoshiaki T. Pleomorphic xanthoastrocytoma of the spinal cord. Case report. J Neurosurg Spine 2006;5:72-5.   Back to cited text no. 7
8.Fouladi M, Jenkins J, Burger P, Langston J, Merchant T, Heidman R, et al. Pleomorphic xanthoastrocytoma: Favorable outcome after complete surgical resection. Neuro Oncol 2001;3:184-92.  Back to cited text no. 8
9.Van Roost D, Kristof R, Zentner J, Wolf HK, Schramm J. Clinical, radiological, and therapeutic features of pleomorphic xanthoastrocytoma: Report of three patients and review of the literature. J Neurol Neurosurg Psychiatry 1996;60:690-2.  Back to cited text no. 9
10.Iwaki T, Fukui M, Kondo A, Matsushima T, Takeshita I. Epithelial properties of pleomorphic xanthoastrocytomas determined in ultrastructural and immunohistochemical studies. Acta Neuropathol 1987;74:142-50.  Back to cited text no. 10
11.Hosokawa Y, Tsuchihashi Y, Okabe H, Toyama M, Namura K, Kuga M, et al. Pleomorphic xanthoastrocytoma. Ultrastructural, immunohistochemical and DNA cytoflourometric study of a case. Cancer 1991;68:853-9.  Back to cited text no. 11
12.Zarate JO, Sampaolesi R. Pleomorphic xanthoastrocytoma of the retina. Am J Surg Pathol 1999;23:79-81.  Back to cited text no. 12
13.Okazaki T, Kageji T, Matsuzaki K, Horiguchi H, Hirose T, Watanabe H, et al. Primary anaplastic pleomorphic xanthoastrocytoma with widespread neuraxis dissemination at diagnosis - a pediatric case report and review of literature. J Neurooncol 2009;94:431-7.  Back to cited text no. 13
14.Passone E, Pizzolito S, D'Agostini S, Skrap M, Gardiman MP, Nocerino A, et al. Non-anaplastic Pleomorphic Xanthoastrocytoma with neuroradiological evidences of leptomeningeal dissemination. Childs Nerv Syst 2006;22:614-8.  Back to cited text no. 14
15.Labansu A, Rorive S, David P, Sariban E, Seligmann R, Brotchi J, et al. Cerebral anaplastic pleomorphic xanthoastrocytoma with meningeal dissemination at first presentation. Child Nerv Syst 2004;20:119-22.  Back to cited text no. 15
16.Nakajoma T, Kumabe T, Shamoto H, Watanabe M, Suzuki H, Tominaga T. Malignant transformation of pleomorphic xanthoastrocytoma. Acta Neurochir 2006;148:67-71.  Back to cited text no. 16
17.Pahapill PA, Ramsay DA, Del Maestro RF. Pleomorphic xanthoastrocytoma: Case report and analysis of the literature concerning the efficacy of resection and the significance of necrosis. Neurosurgery 1996;38:822-9.  Back to cited text no. 17


  [Figure 1], [Figure 2]

  [Table 1]

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