Abstract
We report a new case of giant cell ependymoma (GCE) of the thoracic spinal cord. Ependymomas predominate in children and young adults and are frequently intracranial and infra-tentorial. However, a second age peak at 30–40 years is reported for spinal tumours. Microscopically, ependymomas show a large variety of histological features, among which a rare variant with giant cells. This 59-year-old woman presented with a 6-month history of numbness and burning sensation affecting the left lower limb and hemi-trunk. A cervico-thoracic MRI revealed a solid intra-medullary tumour at the level of T1–T3, slightly T1-hypointense, T2-hyperintense and contrast enhancing. A complete surgical resection was carried out through a C7 to T4 laminectomy. Recovery was complete with no sign of recurrence at 18-month follow-up. The initial histological diagnosis of glioblastoma was challenged on the basis of the imaging and operative findings of a well-circumscribed tumour. The case was sent to us for second opinion and we diagnosed a GCE, WHO grade II, with a biphasic pattern including a predominant giant cell component (>90%), with genetic evidence of polyploidy, and a very limited classic component, showing a characteristic loss of chromosome 22. Our report adds to the clinical, imaging, pathological and genetic characterisation of GCE and brings the first genetic evidence that these rare tumours are at least bi-clonal. It also suggests that GCE have a good prognosis after complete surgical resection.
Similar content being viewed by others
References
McLendon RE, Wiestler OD, Kros JM, Korshunov A, Ng H-K (2007) Ependymoma. In: Louis D, Ohgaki H, Wiestler OD, Cavenee WK (eds) WHO Classification of tumors of the Central Nervous System. IARC, Lyon, pp 74–80
Burger PC, Scheithauer BW (2007) Tumours of the central nervous system. Armed Force Institute of Pathology (AFIP), fourth series, fascicle 7. The American Registry of Pathology, ARP Press, Washington, pp 145–165
Adamek D, Dec M, Sobol G, Urbanowicz B, Jaworski M (2008) Giant cell ependymoma: a case report. Clin Neurol Neurosurg 110:176–181
Barbagallo GM, Caltabiano R, Parisi G, Albanese V, Lanzafame S (2009) Giant cell ependymoma of the cervical spinal cord: case report and review of the literature. Eur Spine J 18(Suppl 2):186–190
Brown DF, Chason DP, Schwartz LF, Coimbra CP, Rushing EJ (1998) Supratentorial giant cell ependymoma: a case report. Mod Pathol 11:398–403
Cooper PB et al (2006) Rare giant cell ependymoma in an octogenarian. Case report and review of the literature. J Neurosurg 105:908–911
Fourney DR, Siadati A, Bruner JM, Gokaslan ZL, Rhines LD (2004) Giant cell ependymoma of the spinal cord. Case report and review of the literature. J Neurosurg 100:75–79
Karabagli PDH, Sav A (2011) Giant cell ependymoma of the lumbar spine: case report and review of the literature. J Neurol Sci 28(1):109–115
Jeon YK, Jung HW, Park SH (2004) Infratentorial giant cell ependymoma: a rare variant of ependymoma. Pathol Res Pract 200:717–725
Pal P, Fernandes H, Ellison DW (2005) Woman aged 24 years with fourth ventricular mass. Brain Pathol 15(367–368):373
Pimentel J et al (2001) Supratentorial giant cell ependymoma. Clin Neuropathol 20:31–37
Sangoi AR, Lim M, Dulai M, Vogel H, Chang S (2008) Suprasellar giant cell ependymoma: a rare neoplasm in a unique location. Hum Pathol 39:1396–1401
Szpak GM et al (2008) Giant cell ependymoma of the spinal cord and fourth ventricle coexisting with syringomyelia. Folia Neuropathol 46:220–231
Zec N, De Girolami U, Schofield DE, Scott RM, Anthony DC (1996) Giant cell ependymoma of the filum terminale. A report of two cases. Am J Surg Pathol 20:1091–1101
Shamji MF, Benoit BG, Perry A, Jansen GH (2009) Giant cell ependymoma of the thoracic spine: pathology case report. Neurosurgery 64:E566–E567; discussion E567
Shintaku M, Sakamoto T (2009) Tanycytic ependymoma of the filum terminale with pleomorphic giant cells. Brain Tumor Pathol 26:79–82
Shintaku M, Nagata N, Itoh H (2009) Tanycytic ependymoma of the spinal cord with anaplastic cytological features. Brain Tumor Pathol 26:7–10
Nakamura M, Chiba K, Matsumoto M, Ikeda E, Toyama Y (2006) Pleomorphic xanthoastrocytoma of the spinal cord. Case report. J Neurosurg Spine 5:72–75
Lopes MBS, Wiestler OD, Stemmer-Rachaminov AQ, Sharma MC (2007) Tuberous sclerosis coplex and subependymal giant cell astrocytoma. In: Louis D, Ohgaki H, Wiestler OD, Cavenee WK (eds) WHO Classification of tumors of the Central Nervous System. IARC, Lyon, pp 218–221
Acknowledgments
Dr. Elettra Bianchi is a research assistant for the Belgian FNRS (grant No 7.4631.10 Télévie FNRS).
Conflict of interest
None.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Bianchi, E., Lejeune, JP., Sartenaer, D. et al. Giant cell ependymoma of the thoracic spinal cord. Acta Neurol Belg 112, 71–75 (2012). https://doi.org/10.1007/s13760-012-0038-9
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s13760-012-0038-9