J Korean Neurosurg Soc.  2013 Dec;54(6):521-524. 10.3340/jkns.2013.54.6.521.

Multiple Spinal Cord Recurrences of an Intracranial Ependymoma after 14 Years

• Hong S
• Choe WJ
• Moon CT

• KMID: 2190951
• DOI: http://doi.org/10.3340/jkns.2013.54.6.521

Abstract

Ependymoma can spread via cerebrospinal fluid, but late spinal recurrences of intracranial tumor are very rare. We describe a case of a 33-year-old male who presented with multiple, delayed, recurrent lesions in the spinal cord from an intracranial ependymoma. The patient underwent gross total resection and postoperative radiation therapy 14 years prior to visit for a low grade ependymoma in the 4th ventricle. The large thoraco-lumbar intradural-extramedullary spinal cord tumor was surgically removed and the pathologic diagnosis was an anaplastic ependymoma. An adjuvant whole-spine radiation therapy for residual spine lesions was performed. After completion of radiation therapy, a MRI showed a near complete response and the disease was stable for three years.

Keyword

Fourth ventricle ependymoma; Delayed spinal recurrence; Anaplastic transformation

MeSH Terms

Adult
Cerebrospinal Fluid
Diagnosis
Ependymoma*
Humans
Magnetic Resonance Imaging
Male
Recurrence*
Spinal Cord Neoplasms
Spinal Cord*
Spine

Figure

• 

  Fig. 1 Pre-operative outside thoracolumbar MR study showing a well demarcated and heterogeneously enhancing intradural extramedullary mass at the T12-L1 level. The spinal cord is considerably compressed by the tumor.

• 

  Fig. 2 Pathological study (A and B : H&E ×400, C and D : electron microscope) shows a solid tumor with neoplastic cells with round or elongated nuclei. Periluminal ciliary basal bodies and even clusters of blepharoplasts are noted, which is consistent with an anaplastic ependymoma.

• 

  Fig. 3 The postoperative whole spine MR image demonstrats that the main thoracolumbar tumor has been removed but multiple intradural extramedullary enhancing lesions (arrows) from C6 to L5 are found (A, B, and C). There is no evidence of local recurrence of the tumor in the posterior fossa (D and E).

• 

  Fig. 4 After completion of radiation therapy, a near complete response is observed. The disease was stable and well controlled for 3 years. The surveillance for the recurrence of the tumor was performed by an annual whole spine MR study despite the patient's stable neurologic status.

Reference

1. Bademci G, Tun K, Erden E, Evliyaoglu C, Unlu A. Late dissemination of ependymoma : case report. Neurocirugia (Astur). 2007; 18:333–336. PMID: 17882342.

2. Bortolotto S, Chiadò-Piat L, Cavalla P, Bosone I, Mauro A, Schiffer D. CDKN2A/p16 in ependymomas. J Neurooncol. 2001; 54:9–13. PMID: 11763427.

3. Celli P, Cervoni L, Salvati M, Cantore G. Recurrence from filum terminale ependymoma 42 years after 'total' removal and radiotherapy. J Neurooncol. 1997; 34:153–156. PMID: 9210062.

4. Chao ST, Kobayashi T, Benzel E, Reddy CA, Stevens GH, Prayson RA, et al. The role of adjuvant radiation therapy in the treatment of spinal myxopapillary ependymomas. J Neurosurg Spine. 2011; 14:59–64. PMID: 21142463.

5. Ernestus RI, Schröder R, Stützer H, Klug N. The clinical and prognostic relevance of grading in intracranial ependymomas. Br J Neurosurg. 1997; 11:421–428. PMID: 9474274.

6. Good CD, Wade AM, Hayward RD, Phipps KP, Michalski AJ, Harkness WF, et al. Surveillance neuroimaging in childhood intracranial ependymoma : how effective, how often, and for how long? J Neurosurg. 2001; 94:27–32. PMID: 11147894.

7. Halvorsen CM, Kolstad F, Hald J, Johannesen TB, Krossnes BK, Langmoen IA, et al. Long-term outcome after resection of intraspinal ependymomas : report of 86 consecutive cases. Neurosurgery. 2010; 67:1622–1631. discussion 1631. PMID: 21107192.

8. Iunes EA, Stávale JN, de Cássia Caldas Pessoa R, Ansai R, Onishi FJ, de Paiva Neto MA, et al. Multifocal intradural extramedullary ependymoma. Case report. J Neurosurg Spine. 2011; 14:65–70. PMID: 21142461.

9. Kim DG, Cho BK, Yang HJ, Chi JG, Jung HW, Kim HJ, et al. Intracranial Ependymoma : Clinicopathologic Features and Prognostic Factors. J Korean Neurosurg Soc. 1991; 20:893–899.

10. Kleihues P, Burger PC, Scheithauer BW, Zülch KJ. Histological typing of tumours of the central nervous system, ed 2. Berlin: Springer-Verlag;1993.

11. Lyons MK, Kelly PJ. Posterior fossa ependymomas : report of 30 cases and review of the literature. Neurosurgery. 1991; 28:659–664. discussion 664-665. PMID: 1876243.

12. McLaughlin MP, Marcus RB Jr, Buatti JM, McCollough WM, Mickle JP, Kedar A, et al. Ependymoma: results, prognostic factors and treatment recommendations. Int J Radiat Oncol Biol Phys. 1998; 40:845–850. PMID: 9531369.

13. Metellus P, Guyotat J, Chinot O, Durand A, Barrie M, Giorgi R, et al. Adult intracranial WHO grade II ependymomas : long-term outcome and prognostic factor analysis in a series of 114 patients. Neuro Oncol. 2010; 12:976–984. PMID: 20484442.

14. Nakasu S, Ohashi M, Suzuki F, Matsuda M. Late dissemination of fourth ventricle ependymoma : a case report. J Neurooncol. 2001; 55:117–120. PMID: 11817702.

15. Ochiai H, Yamakawa Y, Kawano H, Shimao Y, Hayashi T. Late spinal cord metastasis of fourth ventricle ependymoma appeared nineteen years after the initial treatment. J Neurooncol. 2010; 96:295–299. PMID: 19629395.

16. Rehman S, Brock C, Newlands ES. A case report of a recurrent intracranial ependymoma treated with temozolomide in remission 10 years after completing chemotherapy. Am J Clin Oncol. 2006; 29:106–107. PMID: 16462515.

17. Rezai AR, Woo HH, Lee M, Cohen H, Zagzag D, Epstein FJ. Disseminated ependymomas of the central nervous system. J Neurosurg. 1996; 85:618–624. PMID: 8814165.

18. Salazar OM, Castro-Vita H, VanHoutte P, Rubin P, Aygun C. Improved survival in cases of intracranial ependymoma after radiation therapy. Late report and recommendations. J Neurosurg. 1983; 59:652–659. PMID: 6886786.

19. Vanuytsel L, Brada M. The role of prophylactic spinal irradiation in localized intracranial ependymoma. Int J Radiat Oncol Biol Phys. 1991; 21:825–830. PMID: 1831193.

20. Whitaker SJ, Bessell EM, Ashley SE, Bloom HJ, Bell BA, Brada M. Postoperative radiotherapy in the management of spinal cord ependymoma. J Neurosurg. 1991; 74:720–728. PMID: 2013772.