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Intramedullary Ependymomas: Clinical Presentation, Surgical Treatment Strategies and Prognosis

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Abstract

Intramedullary ependymomas are rare tumors but comprise the majority of intramedullary glial neoplasms in the adult. These tumors are benign, slow-growing lesions which are optimally treated with gross-total surgical resection without adjuvant therapy. This objective can be attained safely in a majority, of patients. Post-operative functional outcome is related to pre-operative functional status. Hence, early diagnosis, prior to symptomatic progression, is critical to the successful treatment of these tumors. Adjuvant therapy is indicated for the rare malignant or disseminated tumor or following sub-total resection.

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References

  1. Helseth A, Mørk SJ: Primary intraspinal neoplasms in Norway, 1955-1986. A population-based survey of 467 patients. J Neurosurg 71: 842-845, 1989

    Google Scholar 

  2. Mørk SJ, Løken AC: Ependymoma. A follow-up study of 101 cases. Cancer 40: 907-915, 1977

    Google Scholar 

  3. Sonneland PRL, Scheithauer BW, Onofrio BM: Myxopapillary ependymoma. A clinicopathologic and immunocytochemical study of 77 cases. Cancer 56: 883-893, 1985

    Google Scholar 

  4. McCormick PC, Torres R, Post KD, Stein BM: Intramedullary ependymoma of the spinal cord. J Neurosurg 72: 523-533, 1990

    Google Scholar 

  5. Samii M, Klekamp J: Surgical results of 100 intramedullary tumors in relation to accompanying syringomyelia. Neurosurgery 35: 865-873, 1994

    Google Scholar 

  6. McCormick PC, Stein BM: Intramedullary tumors in adults. Neurosurg Clin North Am 1: 609-630, 1990

    Google Scholar 

  7. Cooper PR: Outcome after operative treatment of intramedullary spinal cord tumors in adults: intermediate and long-term results in 51 patients. Neurosurgery 25: 855-859, 1989

    Google Scholar 

  8. Epstein FJ, Farmer J-P, Freed D: Adult intramedullary spinal cord ependymomas: the result of surgery in 38 patients. J Neurosurg 79: 204-209, 1993

    Google Scholar 

  9. Russell DS, Rubenstein LJ: Pathology of Tumors of the Nervous System. Williams & Wilkins, Baltimore, 1989

    Google Scholar 

  10. Cristante L, Herrmann H-D: Surgical management of intramedullary spinal cord tumors: functional outcome and sources of morbidity. Neurosurgery 35: 69-76, 1994

    Google Scholar 

  11. McCormick PC, Post KD, Stein BM: Intradural extramedullary tumors in adults. Neurosurg Clin North Am 1: 591-608, 1990

    Google Scholar 

  12. Hoshimaru M, Koyama T, Hashimmoto N, Kikuchi H: Results of microsurgical treatment for intramedullary spinal cord ependymomas: analysis of 36 cases. Neurosurgery 44: 264-269, 1999

    Google Scholar 

  13. Sandler HM, Papadopoulos SM, Thuntan AF, Ross DA: Spinal cord astrocytoma: results of therapy. Neurosurgery 30: 490-493, 1999

    Google Scholar 

  14. Sloof JL, Kernohan JW, McCarthy CS: Primary Intramedullary Tumors of the Spinal Cord and Filum Terminale. W.B. Saunders, Philadelphia, 1964

    Google Scholar 

  15. Neumann HPH, Eggert HR, Weigel K: Hemangioblastomas of the central nervous system: a 10-year study with special reference to Von Hippel-Lindau syndrome. J Neurosurg 70: 24-30, 1989

    Google Scholar 

  16. Lee M, Epstein FJ, Rezzai AR, Zagzag D: Nonneoplastic intramedullary spinal cord lesions mimicking tumors. Neurosurgery 43: 788-795, 1998

    Google Scholar 

  17. Mulvehill JJ, Parry DM, Sherman JL, Pikus A, Kaiser-Kupfer MI, Eldridge R: NIH confeence: neurofibromatosis 1 (Recklinghausen disease) and neurofibromatosis 2 (bilateral acoustic neurofibromatosis)-an update. Ann Intern Med 113: 39-52, 1990

    Google Scholar 

  18. Evans DGR, Huson SM, Donnai D, Neary W, Blair V, Teare D, Newton V, Strachan T, Ramsan R, Hariis R: A genetic study of type 2 neurofibromatosis in the United Kingdom: I-Prevalence, mutation rate, fitness, and confirmation of maternal transmission effect on severity. J Med Genet 29: 841-846, 1992

    Google Scholar 

  19. Trofatter JA, MacCollin MM, Rutter JL, Murrell JR, Duyao MP, Parry DM, Eldridge R, Kley N, Menon AG, Pulsaki K, Haase VH, Ambrose CM, Munroe D, Bove, Haines JL, Martuza RL, MacDonald ME, Seizinger BR, Short MP, Buckler AJ, Gussella JF: A novel moesin-, ezrin-, radixin-like gene is a candidate for the neurofibromatosis 2 tumor suppressor. Cell 72: 791-800, 1993

    Google Scholar 

  20. Rouleau GA, Wertelecki W, Haines JL, Hobbs WJ, Troffatter JA, Seizinger BR, Martuza RL, Superneau DW, Conneally PM, Gusella JF: Genetic linkage of bilateral acoustic neurofibromatosis to a DNA marker on chromosome 22. Nature 329: 246-228, 1987

    Google Scholar 

  21. Rouleau GA, Merel P, Lutchman M, Sanson M, Zucman J, Marineau C, Hoang-Xuan K, Demczuk S, Desmaze C. Plougastel B, Pulst SM, Lenoir G, Biijlsma V, Fashold R, Dumanski J, de Jong P, Parry D, Eldridge R, Aurias A, Delattre O, Thomas G: Alteration in a new, gene encoding a putative membrane organizing protein causes neurofibromatosis type 2. Nature 363: 515-521, 1993

    Google Scholar 

  22. Martuza RL, Eldridge R: Neurofibromatosis 2: bilateral acoustic neurofibromatosis. N EngI J Med 318: 684-688, 1988

    Google Scholar 

  23. Rodriguez HA, Berthrong M: Muliple primary tumors in von Recklinghausen's neurofibromatosis. Arch Neurol 14: 467-475, 1966

    Google Scholar 

  24. Mautner V-F, Tatagiba M, Guthoff R, Samii M, Pulst S-M: Neurofibromatosis 2 in the pediatric age group. Neurosurgery 33: 92-96, 1993

    Google Scholar 

  25. Lee M, Rezai AR, Freed D, Epstein FJ: Intramedullary spinal cord tumors in neurofibromatosis. Neurosurgery 38: 32-37, 1996

    Google Scholar 

  26. Birch BD, Johnson JP, Parsa A, Dasai RD, Yoon JT, Lycette CA, Li YM, Bruce JN: Frequent type 2 neurofibromatosis gene transcript mutations in sporadic intramedullary spinal cord ependymomas. Neurosurgery 39: 135-140, 1996

    Google Scholar 

  27. Cohen AR, Wisoff JH, Allen JC, Epstein FJ: Malignant astrocytomas of the spinal cord. J Neurosurg 70: 50-54, 1989

    Google Scholar 

  28. Kopelson G, Linggood RM: Intramedullary spinal cord astrocytoma versus glioblastoma. The prognostic importance of histologic grade. Cancer 50: 732-735, 1982

    Google Scholar 

  29. Adams DC, Emerson RG. Heyer EJ: Intraoperative evoked potential monitoring with controlled neuromuscular blockade. Anesth Anal 77: 913-918, 1993

    Google Scholar 

  30. Morota N, Deletis V, Constantini S, Kofler M, Cohen H, Epstein FJ: The role of motor evoked potentials during surgery for intramedullary spinal cord tumors. Neurosurgery 41: 1327-1336, 1997

    Google Scholar 

  31. Epstein FJ, Farmer J-P, Schneider SJ: Intraoperative ultrasonography: an important surgical adjunct for intramedullary tumors. J Neurosurg 74: 729-733, 1991

    Google Scholar 

  32. Cooper PR, Epstein F: Radical resection of intramedullary spinal cord tumors in adults. Recent experience in 29 patients. J Neurosurg 63: 492-499, 1985

    Google Scholar 

  33. Epstein FJ, Farmer J-P, Freed D: Adult intramedullary astrocytomas of the spinal cord. J Neurosurg 77: 355-359, 1992

    Google Scholar 

  34. Brotchi J, Dewitte O, Leviver M, Balérianx D, vandesteene A, Raftopolous C, Flament-Durand J, Noterman J: A survey of 65 tumors within the spinal cord: surgical results and the importance of preoperative magnetic resonance imaging. Neurosurgery 29: 651-657, 1991

    Google Scholar 

  35. Fisher G, Mansuy L: Total removal of intramedullary ependymomas: follow-up study of 16 cases. Surg Neurol l4: 243-249, 1980

    Google Scholar 

  36. Rawlings CE, Giangaspero F, Burger PC, Bullard DE: Ependymomas: a clinicopathologic study. Surg Neurol 29: 271-281. 1988

    Google Scholar 

  37. Guidetti B, Mercuri S, Vaonozzi R: Long-term results of the surgical treatment of 129 intramedullary spinal gliomas. J Neurosurg 54: 323-330, 1981

    Google Scholar 

  38. Greenwood JJ: Intramedullary tumors of the spinal cord. A follow-up study after total surgical removal. J Neurosurg 20: 665-668, 1963

    Google Scholar 

  39. Malis LI: Intramedullary spinal cord tumors. Clin Neurosurg 25: 512-539, 1978

    Google Scholar 

  40. Garrido E, Stein BM: Microsurgical removal of intramedullary spinal cord tumors. Surg Neurol 7: 215-219, 1977

    Google Scholar 

  41. Linstadt DE, Wara WM, Leibel SA, Gutin PH, Wilson CB, Sheline GE: Postoperative radiotherapy of primary spinal cord tumors. Int J Radiat Oncol Biol Phys 16: 1397-1403, 1989

    Google Scholar 

  42. McLaughlin MP, Buatti JM, Marcus RB, Maria BL, Mickle PJ, Kedar A: Outcome after radiotherapy of primary spinal cord glial tumors. Rad Oncol Invest 6: 276-280, 1998

    Google Scholar 

  43. Wen BC, Hussey DH, Hitchon PW, Schelper RL, Vigliotti AP, Doornbos JF, Van Filder JC: The role of radiation therapy in the management of ependymomas of the spinal cord. Int J Radiat Oncol Biol Phys 20: 781-786, 1991

    Google Scholar 

  44. Shaw EG, Evans RG, Scheithauer BW, Ilstrup DM, Earle JD: Radiotherapeutic management of adult intraspinal ependymomas. In J Radiat Oncol Bio Phys 12: 323-327, 1986

    Google Scholar 

  45. Garcia DM: Primary spinal cord tumors treated with surgery and postoperative irradiation. In J Radiat Oncol Biol Phys 11: 1933-1939, 1985

    Google Scholar 

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Authors and Affiliations

  1. Department of Neurological Surgery, The Neurological Institute of New York, New York Presbyterian Hospital, New York, NY, USA

    Theodore H. Schwartz & Paul C. McCormick

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  1. Theodore H. Schwartz
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  2. Paul C. McCormick
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Schwartz, T.H., McCormick, P.C. Intramedullary Ependymomas: Clinical Presentation, Surgical Treatment Strategies and Prognosis. J Neurooncol 47, 211–218 (2000). https://doi.org/10.1023/A:1006414405305

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