Intracranial Ependymomas in Children

Coulon, R.; Till, Kenneth

doi:10.1159/000119663

Cited by 22 publications

(32 citation statements)

References 2 publications

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“…17] than in adults. The 5-year survival rate (operative mortality excluded) varies from 16% [6] to 58% [5], The rate of 51% in our pediatric series is in agreement with the rate of 47% in the most recent pediatric report by Bloom [5] in 1979. Thus, it appears that, at least, 1 child out of 2 will die from recurrence or metastasis in the 5 years following radiotherapy.…”

Section: Discussionsupporting

confidence: 87%

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Intracranial Ependymomas in Childhood Survival and Functional Results of 47 Cases

Pierre-Kahn¹,

Hirsch²,

Fx³

et al. 1983

Pediatr Neurosurg

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A pediatric series of 47 intracranial ependymomas (15 supra- and 32 infratentorial) is presented. All children were operated upon between 1969 and 1979. Among these children, 35 completed the treatment with radiotherapy. The irradiation was usually limited to the intracranial content but in 7 patients, it was extended to the spinal axis as well. Malignant ependymomas represented 69% of the whole series (86% of the supratentorial and 53% of the infratentorial ependymomas). The operative mortality rate was 17%. The 5-year survival rate was 39% in the whole series and 51% when excluding postoperative mortality. Recurrences developed in 41% of cases and metastases in 20%. The study of this series and of the literature points out that: (1) metastases from supratentorial ependymomas are not frequent, almost always supratentorial and secondary to a malignant ependymoma, and (2) metastases from infratentorial ependymomas are almost always intraspinal and occur in one third of these tumors. The rate of seeding is especially high in the case of malignant infratentorial ependymomas (50%) but is also 15% in the case of benign tumors. These data lead the authors to propose a craniospinal irradiation in the case of infratentorial ependymomas whether benign or malignant, an irradiation of only the brain in the case of malignant supratentorial ependymoma, an irradiation of only the tumoral bed when ependymomas are benign and supratentorial.

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Section: Discussionsupporting

confidence: 87%

“…In the past few years, the postoperative mortality rate has significantly decreased from an average of 30% before 1975 [25] to an average of 19% since 1975 (average of the postoperative mortalities in the series) [6,7,12,18,19,26]. The rate of 17% in our series illustrates this general trend.…”

Section: Discussionsupporting

confidence: 61%

Intracranial Ependymomas in Childhood Survival and Functional Results of 47 Cases

Pierre-Kahn¹,

Hirsch²,

Fx³

et al. 1983

Pediatr Neurosurg

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“…Other series confirmed a poor outcome for posterior fossa ependymomas [12,21,26,39], suggesting that tumor location is important in limiting surgical removal and, consequently, affecting the outcome. In particular, considering fourth-ventricle ependymomas, which in our study accounted for 60% of the tumors, in some series most of the tumors seem to arise in the vestibular area or the lateral recess [8,43]. We confirm these data, with 81% of posterior fossa ependymomas arising from the lateral recess of the fourth ventricle.…”

Section: The Role Of Tumor Locationsupporting

confidence: 85%

Prognostic Factors in Childhood Intracranial Ependymomas: The Role of Age and Tumor Location

Sala

Talacchi²,

Mazza³

et al. 1998

Pediatr Neurosurg

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Despite several clinical reports on intracranial ependymomas in children, the factors which affect prognosis, and the possibility that certain combinations of factors might limit survival, are still a matter of debate. Between 1976 and 1996 we operated on 35 children with intracranial ependymomas. Postoperative irradiation was given to 27 patients, with associated chemotherapy in 6 cases. Mean follow-up was 62 months. In 12 patients a 5-year follow-up was possible. In October 1996, 18 patients (51.4%) were still alive, the longest disease-free follow-up being 20 years, and the shortest 8 months. We analyzed the prognostic relevance of eight factors. For each factor, different subgroups were distinguished and compared as follows: age at diagnosis (<4 vs. ≥4 years), sex, tumor location (supratentorial vs. infratentorial), tumor size (<4 vs. 4–7 vs. >7 cm), surgical removal (total vs. subtotal), histology (low-grade vs. anaplastic), morphology (solid vs. cystic), adjuvant therapies (treatment vs. no treatment). Two-way contingency tables were made to identify associations between variables. The only significant association was between age and tumor location (p = 0.022): in children under 4, tumors were almost invariably located in the posterior fossa (9 out of 10 cases) with a clear preference for the lateral recess (8 cases). Other correlations were not significant. Kaplan-Meier survival curves were compared to assess the prognostic relevance of each factor. Survival was significantly lower for children under 4, for those with posterior fossa tumors, and for patients with residual tumor (p < 0.05). A multivariate analysis compared variables which significantly affected survival, revealing that age is the most important factor affecting prognosis (p < 0.05), while tumor location and surgical removal do not add any significance to the effect of age on survival. We conclude that age has the strongest prognostic relevance in childhood intracranial ependymomas, while the effect of tumor location on survival may be related to the high incidence of lateral recess ependymomas in younger children.

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“…About fifty percent (50%) occur before five years of age. Two-thirds of ependymomas are located infratentorially 2,3,4,5,6 . The optimal management of fourth ventricular ependymomas remains controversial 7 .…”

Section: Dicussionmentioning

confidence: 99%

Anaplastic ependymoma of the fourth ventricle causing obstrictive hydrocephalus

Iddrissu

Dakurah²,

Wepeba³

2006

Ghana Medical Journal

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SUMMARYEpendymomas are relatively uncommon and present with a spectrum of biological and clinical characteristics that make specific recommendations regarding their treatment difficult and assignment of prognostic factors controversial. The case of fourth ventricular anaplastic epednymoma in a four-year-old child is reported in which the initial presentation was deterioration of the level of consciousness secondary to acute obstructive hydrocephalus. An initial insertion of a ventriculoperitoneal shunt (V-P) to deal with the acute intracranial hypertension was done. Subsequently, sub-occipital craniectomy and sub-total resection of the tumour were performed successfully. Postoperative radiotherapy was also undertaken on the patient. The pertinent literature is reviewed. At one year follow up our patient had clinically improved with no signs of recurrence. The most important prognostic factors are tumour grade and the presence of residual tumour on post operative imaging studies. A median survival of 31 months is noted in children with infratentorial ependymomas and one year survival is quoted as 81%.

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Intracranial Ependymomas in Children

Cited by 22 publications

References 2 publications

Intracranial Ependymomas in Childhood Survival and Functional Results of 47 Cases

Intracranial Ependymomas in Childhood Survival and Functional Results of 47 Cases

Prognostic Factors in Childhood Intracranial Ependymomas: The Role of Age and Tumor Location

Anaplastic ependymoma of the fourth ventricle causing obstrictive hydrocephalus

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