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Year : 2011  |  Volume : 54  |  Issue : 1  |  Page : 201-203
Giant cell ependymoma of a cervical spinal cord


1 Department of Pathology, The Gujarat Cancer and Research Institute, M.P. Shah Cancer Hospital, Ahmedabad - 380 016, Gujarat, India
2 Department of Neurosurgery, The Gujarat Cancer and Research Institute, M.P. Shah Cancer Hospital, Ahmedabad - 380 016, Gujarat, India

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Date of Web Publication7-Mar-2011
 

How to cite this article:
Trivedi P, Gupta A, Pasricha S, Patel D. Giant cell ependymoma of a cervical spinal cord. Indian J Pathol Microbiol 2011;54:201-3

How to cite this URL:
Trivedi P, Gupta A, Pasricha S, Patel D. Giant cell ependymoma of a cervical spinal cord. Indian J Pathol Microbiol [serial online] 2011 [cited 2019 Nov 12];54:201-3. Available from: http://www.ijpmonline.org/text.asp?2011/54/1/201/77406


Ependymomas are neuro-epithelial tumors of the central nervous system (CNS), which originate from ependymal cells lining the ventricles, the choroid plexus, the central canal of spinal cord, and the filum terminale. Apart from the four ependymal patterns and its four histologic types, the world health organization (WHO) recognizes several other rare patterns with variable differentiation including an exceptionally rare variant, giant cell ependymoma (GCE). [1] To the best of our knowledge, only few cases of GCE have been reported in the literature. [2],[3],[4],[5] A 47-year-old male presented with a 3 year history of tingling and numbness in the left side of body and with progressive weakness in the left side of body since past 15 days. Neurological examination revealed reduced motor strength in the left side of body. Magnetic resonance imaging (MRI) revealed a heterogeneous intensity in a homogeneously enhancing lesion measuring 73×18×13 mm involving an upper cervical spinal cord [Figure 1]. Suboccipital craniectomy below the inferior nuchal line was performed. Tumor was well circumscribed and easily dissected from the surrounding spinal cord [Figure 2]. At 16 months follow-up, progressive neurological improvement is registered. MRI scan confirmed complete resection of the tumor and the absence of recurrence. Grossly, the specimen consisted of a well-circumscribed single soft grey white tissue piece measuring 6.0 cm in diameter. Hematoxylin and eosin (H and E) stained sections revealed a highly cellular biphasic neoplasm comprising chiefly neoplastic large pleomorphic giant cells with abundant amount of eosinophilic cytoplasm disposed in sheets as well as forming perivascular pseudorosettes. Individual cells were large pleomorphic having vesicular to hyperchromatic nucleus and single prominent nucleoli. Nuclear pseudo-inclusions were also present. The second component comprised smaller round to oval neoplastic cells forming ependymal pseudorosettes. Occasional true ependymal rosettes were also identified. The tumor contained numerous thin-walled small vessels. Per-iodic acid Schiff stain (PAS) positive extracellular hyaline bodies were also present [Figure 3] and [Figure 4]. Areas of necrosis, mitotic figures, or microvascular proliferation were not identified which favored low-grade ependymoma. On immunohistochemical (IHC) staining, tumor cells showed positivity for glial fibrillary acidic protein (GFAP), S-100, and vimentin. The tumor cells demonstrated a low Ki-67 labeling index (<1%) [Figure 5].
Figure 1: (a) Sagittal T2W MRI demonstrating a heterogeneously hyperintense intramedullary lesion from medulla to C4-C5 level with focal expansion of cord and an exophytic cystic component posterior to medulla. (b) Sagittal T1W, gadolinium enhanced MRI showing inhomogeneously enhancing lesion extending from medulla to C4-C5 level with a non-enhancing exophytic cystic component posterior to medulla

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Figure 2: (a) Intra-operative photograph obtained before resection of GCE. (b) Gross appearance of the solid tumor after resection

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Figure 3: Photomicrograph of GCE. (a) Proliferation of neoplastic cells with perivascular pseudorosettes (H and E, ×100). (b) Pleomorphic giant cells with abundant eosinophilic cytoplasm (H and E, ×200)

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Figure 4: (a) Neoplastic cells with giant cells (arrow) forming pseudorosettes (H and E, ×200).
(b) Neoplastic cells with giant cells, thin walled vessels, hyaline globules (arrow) (H and E, ×200)


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Figure 5: Immunohistochemical stain: (a) Positive GFAP staining in tumor cells (IHC stain, ×100). (b) Ki-67 (MIB-1) positive tumor cells (arrow) (IHC stain, ×400)

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In 1996, Zec et al.[2] first described two cases of GCE of filum terminale. Of the cases reported in literature, few showed features of low grade ependymoma and few showed anaplastic features. [3] Our case satisfied the WHO criteria for low-grade ependymoma despite the pleomorphic nature of tumor cells as significant mitosis, microvascular proliferation or necrosis was not present. Differential diagnosis of GCE includes anaplastic oligodendroglioma, pleomorphic xanthoastrocytoma (PXA), subependymal giant cell astrocytoma (SEGA) and giant cell glioblastoma. The presence of giant cells does not seem to worsen the prognosis of GCE as is also evident by the good prognosis of SEGA and PXA, the two other glial tumors containing pleomorphic giant cells. [4] Light microscopic and IHC findings in our case provided unequivocal evidence of an ependymal origin albeit with an unusual gigantocellular morphology. A low MIB1 index (<1%), which is an adjunctive prognostic indicator for ependymoma, [4] corresponds well to the excellent outcome in our case. Pathologists and surgeons should be aware of this newer entity as the worrisome pleomorphic giant cells could easily be misinterpreted as a higher grade neoplasm.


   Acknowledgements Top


The authors wish to express their thanks to Dr. P.M. Shah, Honorary Director, Dr. K.M. Patel, Dr. S.N. Shukla Deputy Directors and Dr. M. J. Shah, head of the Pathology Department of The Gujarat Cancer and Research Institute for allowing to publish this original article.

 
   References Top

1.Louis DN, Ohgaki H, Wiestler OD, Cavenee WK. WHO classification of tumours of the central nervous system. Lyon: IARC; 2007.  Back to cited text no. 1
    
2.Zec N, De Girolami U, Schofield De, Scott R M, Anthony DC. Giant cell ependymoma of the filum terminale: A report of two cases. Am J Surg Pathol 1996;20:1091-101.   Back to cited text no. 2
    
3.Sangoi AR, Lim M, Dulai MS, Vogel H, Chang SD. Suprasellar giant cell ependymoma: A rare neoplasm in a unique location. Hum Pathol 2008;39:1396-40.   Back to cited text no. 3
    
4.Barbagallo GM, Caltabiano R, Parisi G, Albanese V, Lanzafame S. Giant cell ependymoma of the cervical spinal cord: Case report and review of the literature. Eur Spine J 2009;18:S186-90.  Back to cited text no. 4
    
5.Shamji MF, Benoit BG, Perry A, Jansen GH. Giant cell ependymoma of the thoracic spine: Pathology case report. Neurosurgery 2009;64: 566-7.  Back to cited text no. 5
    

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Correspondence Address:
Priti Trivedi
Room # 412, Department of Pathology, The Gujarat Cancer and Research Institute, NCH Campus, Asarwa, Ahmedabad - 380 016, Gujarat
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.77406

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

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