Abstract
Intracranial ependymomas represent one of the most frequent brain tumors in childhood. Their preferred midline localization and their often controversially discussed classification prompted the present study of 67 intracranial ependymomas in children less than 15 years of age who were operated on from 1951 to 1990. Clinical data and follow-up of all children were retrospectively analyzed by calculation and statistical comparison of progression-free survival (PFS). According to the WHO classification as revised in 1993, 1 grade I subependymoma, 38 grade II ependymomas, and 28 grade III anaplastic (malignant) ependymomas were differentiated. Grade II ependymomas were predominantly located in the IV ventricle and in the supratentorial midline, which often made complete tumor resection impossible. In contrast, the majority of grade III tumors, most often situated in the cerebral hemispheres, could be totally removed. Operative mortality was higher in grade II than in grade III tumors. After recovery from operation, PFS was mainly determined by the histological grading. Median postoperative PFS was 120 months in grade II, but only 18 months in grade III ependymomas (P = 0.1417). Thus, despite varying therapeutic concepts, analysis of this 40-year collective study confirms the prognostic relevance of localization and WHO grading in the case of intracranial ependymoma in children.
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Afra D, Müller W, Slowik F, Wilcke O, Budka H, Turoczy L (1983) Supratentorial lobar ependymomas: reports on the grading and survival periods in 80 cases, including 46 recurrences. Acta Neurochir (Wien) 69:243–251
Bailey P (1927) Further remarks concerning tumors of the glioma group. Bull Johns Hopkins Hosp 40:354–389
Bailey P, Cushing H (1926) A classification of the tumors of the glioma group on a histogenetic basis with a correlated study of prognosis. Lippincott, Philadelphia
Bloom HJG, Walsh LS (1975) Tumours of the central nervous system. In: Bloom HJG, Lemerle J, Neidhardt MK, Voûte PA (eds) Cancer in children — clinical management, chap 10. Springer, Berlin Heidelberg New York, pp 93–119
Breslow N (1970) A generalized Kruskal-Wallis test for comparing k samples subject to unequal patterns of censorship. Biometrika 57:579–594
Chin HW, Maruyama Y, Markesbery W, Young AB (1982) Intracranial ependymoma: results of radiotherapy at the University of Kentucky. Cancer 49:2276–2280
Chiu JK, Woo SY, Ater J, Connelly J, Bruner JM, Maor MH, Eys J van, Oswald MJ, Shallenberger R (1992) Intracranial ependymoma in children: analysis of prognostic factors. J Neurooncol 13:283–290
Coulon RA, Till K (1977) Intracranial ependymomas in children. A review of 43 cases. Child's Brain 3:154–168
Daumas-Duport C (1992) Histological grading of gliomas. Curr Opin Neurol Neurosurg 5:924–931
Dohrmann GJ, Farwell JR, Flannery JT (1976) Ependymomas and ependymoblastomas in children. J Neurosurg 45:273–283
Edwards MS, Levin VA, Wilson CB (1980) Chemotherapy of pediatric posterior fossa tumors. Child's Brain 7:252–260
Ernestus R-I (1996) Intracranial ependymomas. In: Palmer J (ed) Neurosurgery 96 — manual of neurosurgery. Churchill Livingstone, New York, pp 242–247
Ernestus R-I, Wilcke O, Schröder R (1991) Supratentorial ependymomas in childhood: clinicopathological findings and prognosis. Acta Neurochir (Wien) 111:96–102
Ernestus R-I, Schröder R, Klug N (1992) Relationship between the recurrence of intracranial ependymomas and the grade of malignancy. In: Piscol K, Klinger M, Brock M (eds) Advances in neurosurgery, vol 20. Springer, Berlin Heidelberg New York, pp 321–326
Ernestus R-I, Schröder R, Klug N (1992) Spontaneous intracerebral hemorrhage from an unsuspected ependymoma in early infancy. Child's Nerv Syst 8:357–360
Flores LE, Williams DL, Bell BA, O'Brien M, Ragab AH (1986) Delay in the diagnosis of pediatric brain tumors. Am J Dis Child 140:684–686
Fokes EC, Earle KM (1969) Ependymomas. Clinical and pathological aspects. J Neurosurg 30:585–594
Goldwein JW, Corn BW, Finlay JL, Packer JP, Rorke LB, Schut L (1991) Is craniospinal irradiation required to cure children with malignant (anaplastic) intracranial ependymomas? Cancer 67:2766–2771
Goutelle A, Fischer G (1977) Les épendymomes intracrâniens et intrarachidiens. 27e Congrès de la Société de Neuro-chirurgie de langue française, Nice, 24–25 Mai 1977. (Monographies des périodiques Masson no 11) Masson, Paris New York Barcelona
Healey EA, Barnes PD, Kupsky WJ, Scott RM, Sallan SE, Black PMcl, Tarbell NJ (1991) The prognostic significance of postoperative residual tumor in ependymoma. Neurosurgery 28:666–672
Hooper R (1975) Intracranial tumours in childhood. Child's Brain 1:136–140
Ilgren EB, Stiller CA, Hughes JT, Silberman D, Steckel N, Kaye A (1984) Ependymomas: a clinical and pathologic study. I. Biological features. Clin Neuropathol 3:113–121
Kaplan EL, Meier P (1958) Nonparametric estimation from incomplete observations. J Am Statist Assoc 53:457–481
Kernohan JW, Fletcher-Kernohan EM (1935) Ependymomas. A study of 109 cases. Proc Assoc Res Nerv Ment Dis (Baltimore) 16:182–209
Kernohan JW, Mabon RF, Svien HJ, Adson AW (1949) A simplified classification of the gliomas. Proc Staff Meet Mayo Clin 24:71–75
Kleihues P, Burger PC, Scheithauer BW (1993) Histological typing of tumours of the central nervous system, 2nd edn. Springer, Berlin Heidelberg New York
Liu HM, Boggs J, Kidd J (1976) Ependymomas of childhood. I. Histological survey and clinicopathological correlation. Child's Brain 2:92–110
Lyons MK, Kelly PJ (1991) Posterior fossa ependymomas: report of 30 cases and review of the literature. Neurosurgery 28:659–665
Mabon RF, Svien HJ, Kernohan JW, McK Craig W (1949) Ependymomas. Proc Staff Meet Mayo Clin 24:65–71
Marks JE, Adler SJ (1982) Study of ependymomas by site of origin. Int J Radiat Oncol Biol Phys 8:37–43
Mørk SJ, Løken AC (1977) Ependymoma: a follow-up study of 101 cases. Cancer 40:907–915
Pierre-Kahn A, Hirsch JF, Roux FX, Renier D, Sainte-Rose C (1983) Intracranial ependymomas in childhood. Survival and functional results in 46 cases. Child's Brain 10:145–156
Ringertz N (1950) “Grading” of gliomas. Acta Pathol Microbiol Scand 27:51–64
Ringertz N, Reymond A (1949) Ependymomas and choroid plexus papillomas. J Neuropathol Exp Neurol 8:355–380
Rousseau P, Habrand J-L, Sarrazin D, Kalifa C, Terrier-Lacombe M-T, Rekacewicz C, Rey A (1993) Treatment of intracranial ependymomas of children: review of a 15-year experience. Int J Radiat Oncol Biol Phys 28:381–386
Roussy G, Oberling C (1932) Histologic classification of tumors of the central nervous system. Arch Neurol Psychiatry 27:1281–1289
Salazar OM, Castro-Vita H, VanHoutte P, Rubin P, Aygun C (1983) Improved survival in cases of intracranial ependymoma after radiation therapy. Late report and recommendations. J Neurosurg 59:652–659
Schiffer D, Chiò A, Giordana MT, Migheli A, Palma L, Pollo B, Soffietti R, Tribolo A (1991) Histologic prognostic factors in ependymoma. Child's Nerv Syst 7:177–182
Sheline GE (1975) Radiation therapy of tumors of the central nervous system. Cancer 35:957–964
Sutton LN, Goldwein J, Perilongo G, Lang B, Schut L, Rorke L, Packer R (1990–91) Prognostic factors in childhood ependymomas. Pediatr Neurosurg 16:57–65
Svien HJ, Mabon RF, Kernohan JW, McK Craig W (1953) Ependymoma of the brain: pathologic aspects. Neurology 3:1–15
Undijan S, Marinov M (1990) Intracranial ependymomas in children. Child's Nerv Syst 6:131–134
Vanuytsel L, Brada M (1991) The role of prophylactic spinal irradiation in localized intracranial ependymoma. Int J Radiat Oncol Biol Phys 21:825–830
West CR, Bruce DA, Duffner PK (1985) Ependymomas. Factors in clinical and diagnostic staging. Cancer 56:1812–1816
Zülch KJ (1981) Historical development of the classification of brain tumours and the new proposal of the World Health Organization (WHO). Neurosurg Rev 4:123–127
Zülch KJ (1986) Brain tumors. Their biology and pathology, 3rd edn. Springer, Berlin Heidelberg New York Tokyo
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Ernestus, RI., Schröder, R., Stützer, H. et al. Prognostic relevance of localization and grading in intracranial ependymomas of childhood. Child's Nerv Syst 12, 522–526 (1996). https://doi.org/10.1007/BF00261604
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DOI: https://doi.org/10.1007/BF00261604