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PubMed1997

The clinical and prognostic relevance of grading in intracranial ependymomas.

The clinical relevance of grading in ependymomas is almost always regarded as controversial. According to the classification of brain tumours revised by the World Health Organization (WHO) in 1993, brain tumours of ependymal origin are differentiated as subependymomas Grade I, ependymomas Grade II, and anaplastic (malignant) ependymomas Grade III. The purpose of the present retrospective study of 126 patients with intracranial ependymomas was to assess the clinical and prognostic significance of the topical classification and grading system by a uni- and multivariate statistical analysis. 87 Grade II ependymomas were predominantly located in the midline and in the fourth ventricle, whereas 39 anaplastic ependymomas Grade III were most often found in the cerebral hemispheres. Excluding the localization-linked operative mortality, progression-free survival (PFS) was significantly dependent on the histological grading. Median PFS time was 7.5 years in Grade II, but only 1.5 years in Grade III ependymomas. Stratifying for the two time intervals 1951-1970 and 1971-1990, and excluding the operative mortality, a multivariate Cox' model analysis of the covariates age, localization, grading, extent of surgery, and radiation therapy revealed that only the histological grading and radiation therapy had a significant impact on PFS. Thus, the WHO grading system has a statistically significant relevance for the long-term prognosis of intracranial ependymomas. However, the therapeutic management including radical tumour resection and additional local irradiation should be independent of the grading.

Adolescent↗
PubMed1997

Expression of telomerase RNA component correlates with the MIB-1 proliferation index in ependymomas.

Although there is general agreement that certain morphologic subtypes of ependymoma are benign, the biologic behavior of other ependymal neoplasms is poorly understood and not clearly related to conventional histopathologic criteria. The absence of universally accepted standards has prompted the search for more objective biologic markers. Telomerase is an RNA-containing enzyme associated with immortality in proliferating stem cells and many tumors. We investigated the proliferative activity of 26 ependymomas as determined by MIB-1 immunolabeling and compared the results with the in situ expression of human telomerase RNA (hTR) and WHO tumor grade. The study included 9 WHO grade I ependymomas (6 subependymomas and 3 myxopapillary ependymomas), 13 WHO grade II ependymomas, and 4 anaplastic (WHO grade III) ependymomas. The proliferation index (PI) and telomerase RNA expression were significantly increased in grade III ependymomas (p < 0.0001 for PI and p = 0.0015 for hTR). In these tumors, the PI and hTR expression were highly correlated (p = 0.0001). Of note, a single case designated grade II showed both increased proliferative activity and the highest hTR expression detected in this series of ependymal neoplasms. Our results suggest that the PI and hTR expression may be important biologic markers, independent of other histopathologic criteria of tumor grade. Future studies examining the correlation of MIB-1 cell kinetics and hTR expression with clinical parameters in selected ependymoma subtypes are needed to determine the prognostic relevance of these markers.

Adolescent↗
PubMed2001

CD99 immunoreactivity in ependymoma.

The expression of CD99 in normal ependymal cells and ependymoma has been reported. However, only limited numbers of tumors have been studied, and the pattern of CD99 expression has not been described. The authors' purpose was to investigate CD99 immunoreactivity in ependymoma and its use for differential diagnosis. Twenty-five ependymomas were immunostained with antibody directed at CD99. The result of immunostaining of ependymomas was compared with 63 nonependymal tumors that histologically resemble ependymal neoplasms. The nonependymal tumors included 19 astrocytic tumors, 6 oligodendroglial tumors, 8 choroid plexus neoplasms, 2 central neurocytomas, 5 medulloblastomas, 10 primitive neuroectodermal tumors (PNET), and 13 pituitary adenomas. All ependymomas showed strong expression of CD99 in membranous pattern with intracytoplasmic or intercellular dots (ICDs). The expression pattern of CD99 was not correlated with histologic type or grade of ependymomas. Among 63 nonependymal tumors, 11 (17.5%) showed incomplete membrane staining for CD99; diffuse in 4 PNETs and focal in 5 choroid plexus neoplasms (3 papillomas and 2 carcinomas) and one each of pituitary adenoma and oligodendroglioma. The ICD was not found in nonependymal tumors except a case of choroid plexus papilloma. However, membrane staining or ICD for CD99 was not distinctive in nonependymal tumors. In conclusion, the characteristic pattern of anti-CD99 antibody, i.e., diffuse strong membranous immunostaining with ICDs, is useful in distinguishing ependymomas from the central nervous system tumors that histologically mimic ependymoma.

12E7 Antigen↗
PubMed2002

Intracranial ependymoma.

OBJECT: An intracranial ependymoma is a relatively rare but very interesting variety of glioma. In this paper, the authors compiled a review of the pathological features, imaging characteristics, and treatment strategies related to this brain tumor. METHODS: A Medline search was conducted using the term "ependymoma." The bibliographies of papers obtained were also checked for articles and chapters that could provide additional understanding of this tumor. Malignant ependymomas and ependymomas of the spinal cord (including myxopapillary ependymomas) were excluded from this review. CONCLUSIONS: The posterior fossa is the most frequent site for an intracranial ependymoma. Children are frequently affected. Most authors recommend resecting as much of the tumor as is safely possible. Microscopically, ependymal tumors show both epithelial and glial features. Glial fibrillary acidic protein immunohistochemistry, therefore, helps in identifying ependymomas. Because ependymomas often recur despite surgical intervention, radiotherapy and/or radiosurgery may also play an important role in their treatment. The use of chemotherapy in the treatment of these tumors, especially in the very young, is still being studied.

Brain Neoplasms↗
PubMed1998

Survival and prognostic factors following radiation therapy and chemotherapy for ependymomas in children: a report of the Children's Cancer Group.

OBJECT: Ependymomas in children continue to generate controversy regarding their histological diagnosis and grading. optimal management, and possible prognostic factors. To increase our knowledge of these tumors the authors addressed these issues in a cohort of children with prospectively staged ependymomas treated with radiotherapy and chemotherapy. METHODS: Children between the ages of 2 and 17.3 years harboring an intracranial ependymoma confirmed by a central review of the tumor's pathological characteristics were treated according to Children's Cancer Group Protocol 921 from 1986 to 1992. Treatment following surgery and postoperative tumor staging (including brain computerized tomography or magnetic resonance [MR] imaging, spinal MR imaging or myelography, and cerebrospinal fluid cytological investigation) included craniospinal irradiation with a local boost to the primary tumor and patient randomization to receive adjuvant chemotherapy with either 1) CCNU, vincristine, and prednisone, or 2) the eight-drugs-in-1-day regimen. Centralized review of the tumor pathological characteristics revealed 20 ependymomas and 12 anaplastic ependymomas in the 32 children included in the study. Diagnoses made at the individual institutions included anaplastic (malignant) ependymoma (15 patients), ependymoma (four patients), ependymoblastoma (nine patients), ependymoastrocytoma (one patient), and primitive neuroectodermal tumor (three patients), which were discordant with the centralized review diagnosis in 22 of 32 cases. Only three of the 32 patients had metastatic disease (two with M and one with M3 stages). At surgery, 47% of tumors were estimated to be totally resected. Among the 14 of 17 patients who suffered a relapse and were evaluated for site of relapse, 10 (71%) had an isolated local relapse, three (21%) had concurrent local and metastatic relapse, and only one (7%) had an isolated metastatic relapse. Kaplan-Meier estimates of 5-year progression-free survival (PFS) and overall survival rates were 50 +/- 10% and 64 +/- 9%, respectively. CONCLUSIONS: Predictors of PFS duration included an estimate of the extent of resection made at surgery (total compared with less than total, p = 0.0001) and the amount of residual tumor on postoperative imaging as verified by centralized radiological review (< or = 1.5 cm2 compared with > 1.5 cm2, p < 0.0001). No other factors, including centrally reviewed tumor histopathological type, location, metastasis and tumor (M and T) stages, patient age, race, gender, or chemotherapy treatment regimen significantly correlated with PFS duration. The pattern of predominantly local relapse and the important influence of residual tumor or the extent of resection on PFS duration confirms a prevailing impression that local disease control is the major factor in the prediction of outcome of ependymoma. Survival rates were comparable with those reported by other investigators who have treated patients with similar doses of radiation and no chemotherapy.

Adolescent↗
PubMedDate not supplied

DNA ploidy and nuclear morphometry in adult intracranial ependymomas.

The identification of prognostic parameters in ependymomas remains an important but controversial issue in particular to the significance of histopathological features. Intracranial location is a rare presentation for adult ependymomas. DNA ploidy status and nuclear morphometric features of ependymomas are not well-established. We retrospectively studied the clinicopathologic features of 12 adult intracranial ependymomas and performed image cytometric DNA analysis with nuclear morphometry. The overall survival rate was 75%. Two cases were anaplastic ependymomas. Statistically, histology failed to demonstrate a correlation with clinical outcome or overall survival. DNA ploidy (p = 0.033), DNA index (p = 0.016) and 5c exceeding rate (p = 0.017) were statistically correlated with clinical outcome, but not correlated with overall survival. Nuclear morphometric features measured using 3 different data selection methods were not correlated with clinical outcome or overall survival. It is difficult to identify prognostic factors in a series of cases with limited number. The problem is complicated by the fact that the common criteria used for recognizing malignancy in gliomas are not completely reliable in ependymomas. Nuclear morphometric findings support the general impression that ependymomas are morphologically highly variable tumors. In spite of the small sample size of our series and other factors influencing survival such as the extent of resection and malignant histology, aneuploidy can be suggested as a promising factor, which may reflect potential aggressiveness of the tumor in adult intracranial ependymomas.

Adolescent↗
PubMed1999

Diagnosis of spinal cord ependymoma and astrocytic tumours with magnetic resonance imaging.

Ependymoma and astrocytic tumours, both originating from the glial tissue, constitute the majority of intramedullary spinal cord tumours. The purpose of this study is to evaluate the diagnostic ability of magnetic resonance imaging (MRI) in these two types of intramedullary spinal cord tumours. MRI findings of 17 ependymomas and 11 astrocytic tumours of the spinal cord were reviewed. Pre-contrast T(1)-weighted images revealed abnormal enlargement of the spinal cord in all cases. Peritumoural cyst formation was noted in 14 ependymomas (82%) and four astrocytic tumours (36%). Evidence of haemorrhage was identified in five (29%) ependymomas and one (9%) astrocytic tumour. Gadolinium-MRI (Gd-MRI) revealed marked enhancement in 11 ependymomas (65%), while six ependymomas showed irregular, partial or no enhancement. Five of these six ependymomas had evidence of prior haemorrhage. Eight astrocytic tumours (73%) were enhanced on Gd-MRI and the pattern of enhancement was more irregular or partial than that of ependymomas. Two pilocytic astrocytomas showed marked ring-like enhancement. In conclusion, detection of haemorrhage and Gd-MRI will help to differentiate these two types of tumours. Further study will be needed to elucidate the relationship between the histological subtypes of the tumours and MRI findings. Copyright 1999 Harcourt Publishers Ltd.

Journal Article↗
PubMed1988

Histologic characterization of 41 ependymomas with the help of a personal computer.

Fourty-one ependymomas were histologically analyzed in relation to patient age and sex and tumor location. A discriminant analysis model using Bayes' formula and a personal computer were employed. Ependymomas situated in the posterior fossa had a higher incidence in children. Ependymoblastomas were all situated above the tentorium and occurred only in young children. We identified three tumor groups on the basis of common histologic characteristics: benign ependymomas, anaplastic ependymomas, and ependymoblastomas. The main features useful for the grouping were the degree of differentiation and anaplasia. Ependymomas from the cauda equina showed histologic characteristics that allowed them to be differentiated from other benign ependymomas. In each group the particular histologic characteristics, age, and location were significant in terms of diagnosis and prognosis. This method of analysis may help to more precisely define ependymomas and may provide pathologists and clinicians with a quantifiable diagnostic tool that may be of help in establishing the appropriate treatment.

Adolescent↗
PubMed2004

The histologic grade is a main prognostic factor for patients with intracranial ependymomas treated in the microneurosurgical era: an analysis of 258 patients.

BACKGROUND: Ependymomas account for 3-5% of all intracranial malignancies and occur most often in children and young adults. These neoplasms continue to generate considerable controversy with regard to their rational clinical management. It has been shown that the histologic classification of ependymoma is a significant predictor of clinical outcome in patients with ependymoma. METHODS: Ependymomas from 258 patients who underwent microsurgery at a single institution were evaluated histologically to elucidate the prognostic utility of a recently proposed grading scheme. Pathologic and clinical data then were compared using univariate and multivariate analyses. RESULTS: Increasing grade of ependymoma malignancy was found to be associated strongly and independently with worse clinical outcomes in terms of both event-free survival and overall survival. The effect of radiotherapy also was found to be related to histologic grade and was more beneficial for patients who had anaplastic ependymomas and had undergone complete tumor removal. CONCLUSIONS: The application of a uniform diagnostic criteria for grading ependymomas highlighted the key role of tumor histology in clinical outcome in a cohort of patients who were treated in the microsurgical era. The recently proposed grading scheme is likely to be practically useful, reproducible, and clinically applicable.

Adolescent↗
PubMed2006

p53 Pathway dysfunction in primary childhood ependymomas.

BACKGROUND: Childhood ependymoma remains a major therapeutic challenge despite surgery, chemotherapy, and irradiation. We hypothesized that p53 function might be abrogated in ependymomas and implicated in their resistance to anti-cancer therapy. PROCEDURE: Primary ependymomas at diagnosis or relapse from 24 children were analyzed for p53 pathway, using a functional assay in yeast, RT-PCR, Western blot analysis, and/or immunohistochemistry for TP53 mutation, p14(ARF) deletion and promoter hypermethylation, MDM2 and PAX5 expression, respectively. p53-mediated response to radiation-induced DNA damage was evaluated using Western blot and flow cytometry analysis in two ependymoma xenograft models, IGREP37 and IGREP83, derived from primary anaplastic childhood ependymomas. RESULTS: No TP53, MDM2, p14(ARF), PAX5 gene abnormalities were detected in the primary ependymomas tumors and xenografts tested. Interestingly, despite the lack of these abnormalities, p53 induced p21-mediated G(1) growth arrest in response to irradiation was altered in the IGREP37 xenograft tumors. Although irradiation induced necrosis and apoptotic cell death, IGREP37 tumors were moderately sensitive to radiation therapy in vivo. In contrast, irradiation yielded significant tumor growth delays and tumor regressions in the p53 functional IGREP83 xenografts. CONCLUSION: Alterations in p53-mediated growth arrest in ependymomas might be implicated in the radio-resistance of these tumors and demand further evaluation.

Animals↗
PubMed1996

Prognostic relevance of localization and grading in intracranial ependymomas of childhood.

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.

Adolescent↗
PubMed1992

Predicting the recurrence of ependymomas from the bromodeoxyuridine labeling index.

The usefulness of histopathological grading in predicting the prognosis of patients with ependymomas is controversial. To clarify the discrepancy between the histological malignancy and the prognosis of these tumors, we estimated the proliferative potential of 32 intracranial and intraspinal ependymomas and correlated the findings with the clinical behavior. Each patient received an intraoperative infusion of bromodeoxyuridine (BUdR, 200 mg/m2 i.v.) before tumor removal; the BUdR labeling index (LI), or percentage of BUdR-labeled cells, was determined immunohistochemically in excised specimens. The mean BUdR LI (+/- SD) of intracranial malignant ependymomas was 4.1 +/- 2.8%. Nonmalignant intracranial and intraspinal ependymomas and subependymomas had mean LIs of 1.5 +/- 0.9%, 1.1 +/- 0.3%, and less than 1%, respectively. Overall, 44% of the tumors recurred. There were no statistically significant differences in the recurrence rates of intracranial and intraspinal ependymomas, including subependymomas (43% and 44%, respectively), or of intracranial ependymomas with LIs greater than 1.0% and less than 1.0% (67% and 44%, respectively). However, the early recurrence rate (within 24 months after treatment) of tumors with LIs greater than 1.0% was higher than that of tumors with LIs of less than 1.0% (100% vs. 25%, P less than 0.05). The BUdR LI also showed a statistically significant inverse correlation with the time to recurrence. These findings indicate that BUdR LI reflects the proliferative potential of individual ependymomas and can be used to help predict the recurrence and estimate the prognosis of these tumors.

Adolescent↗
PubMed2004

Light microscopic demonstration of the microlumen of ependymoma: a study of the usefulness of antigen retrieval for epithelial membrane antigen (EMA) immunostaining.

To determine the origin of dotlike epithelial membrane antigen (EMA) immunoreactivity of ependymoma, which is consistent with the eosinophilic globular body in hematoxylin and eosin (H&E) stain, an immunoelectron microscopic study was undertaken. The usefulness of antigen retrieval pretreatment in detecting the dotlike EMA immunoreactivity in ependymomas was also studied. The materials were 29 ependymomas, 7 autopsy brains as a normal control, and 50 brain tumors of various types. The study confirmed that most of the brown dots in EMA immunostain in ependymoma represented microlumina of tumor cells. In ependymomas, plain EMA immunostaining showed dotlike positivity in only six cases (21%), and antigen retrieval pretreatment increased the number of positives up to 26 cases (90%). Antigen retrieved CD99 detected 23 positive cases (80%) in ependymomas. On the basis of the results, although some false positive findings were raised by antigen retrieval pretreatment, the authors positively recommend adoption of the technique, especially when ependymoma remains as one of the differential diagnoses of the tumor.

12E7 Antigen↗
PubMed2000

Eosinophilic inclusions in ependymoma represent microlumina: a light and electron microscopic study.

A study was undertaken to determine the pathological significance of previously unrecognized intracytoplasmic eosinophilic inclusions (IEIs) in ependymoma. The study group consisted of 58 ependymomas, all of which were pathologically characterized and graded according to the 1993 WHO classification. Electron microscopic studies were performed in 16 cases. The study showed that 33 (57%) ependymomas had IEIs and that in 8 cases these were abundant. Round and eosinophilic, their sizes varied from 10 microns to a tiny dot. Similar eosinophilic bodies were also observed between tumor cells. The inclusions were weakly PAS positive. On immunostains, IEIs were frequently positive for glial fibrillary acidic protein, less often for S-100 protein, and for epithelial membrane protein and CAM 5.2. They were negative for AE1/AE3, carcinoembryonic antigen and Ber-EP4. Ultrastructurally, IEIs represented intracytoplasmic lumens containing microvilli and cilia. These microlumina also frequently contained granulo-tubular materials. With reference to tumor subtypes, IEIs occurred most frequently in ordinary and clear cell ependymomas. IEIs were also present in 4 of 6 anaplastic ependymomas studied. In conclusion, IEIs represent microlumina and occur in more than a half of ependymomas including malignant examples. Their finding is a helpful diagnostic feature of ependymoma.

Adolescent↗
PubMed2002

Loss of heterozygosity on chromosome 22 in human ependymomas.

Ependymomas are glial tumors of the brain and spinal cord. The most frequent genetic change in sporadic ependymomas is monosomy 22, suggesting the presence of an ependymoma tumor suppressor gene on chromosome 22. Thirty-three pairs of matched normal and tumor specimens from ependymoma patients were genotyped using 12 polymorphic microsatellite markers spanning the long arm of chromosome 22. Allelic deletion was found in 12 of 33 tumors (36.4%). Eight tumors showed partial deletions and 4 tumors exhibited loss of the entire arm of 22q. We identified two common regions of deletion: one at 22q11.21-12.2 flanked by markers D22S420 and D22S300, and a second candidate region at 22q13.1-13.3 between D22S274 and D22S1149. The size of each region was 21.1 and 2.4 cM, respectively. Thus, our results suggest that one or more tumor suppressor genes associated with ependymoma may be present on chromosome 22. Comparison of these results with clinicopathological data indicate that allelic losses on 22q tend to occur more frequently in intracranial anaplastic ependymomas in children and intraspinal ependymomas in adults.

Adult↗
PubMed2006

Three-dimensional (3D) reconstruction and quantitative analysis of the microvasculature in medulloblastoma and ependymoma subtypes.

In the World Health Organisation (WHO) classification of tumours of the nervous system, four main histopathological subtypes of medulloblastomas (classic medulloblastoma, desmoplastic medulloblastoma, medulloblastoma with extensive nodularity and advanced neuronal differentiation and large cell/anaplastic medulloblastoma) as well as of ependymal tumours (low-grade ependymoma, anaplastic ependymoma, myxopapillary ependymoma and subependymoma) are recognised. Under the hypothesis that the microvascular architecture of tumours is a reflection of the histopathological subtype, we performed three-dimensional reconstructions of the microvasculature in these subtypes of medulloblastomas and ependymal tumours using computerised image analysis. In addition, we quantitatively assessed three microvascular parameters (number, area, perimeter) in these neoplasms. Three-dimensional reconstructions showed a dense pattern of irregular vessels in classic and large cell medulloblastoma. In desmoplastic medulloblastoma and medulloblastoma with extensive nodularity, the vessels were more unevenly distributed and organised around the nodular areas. Classic medulloblastoma and large cell medulloblastoma had on average the largest vessel area and perimeter. The highest number of vessels was seen in classic medulloblastoma and medulloblastoma with extensive nodularity. Three-dimensional analysis of ependymal tumours showed that low-grade ependymoma had larger but fewer vessels compared to anaplastic ependymoma, while myxopapillary ependymoma had a complex, heterogeneous pattern of vessels and subependymoma few but regular vessels. In ependymal tumours, the highest values for vessel number, vessel area and vessel perimeter were found in anaplastic ependymoma and the lowest values in subependymoma. We conclude that our three-dimensional reconstructions shed unprecedented light on the tumour vasculature in medulloblastomas and ependymal tumours and expect that such reconstructions are helpful tools for further studies on tumour angiogenesis.

Adult↗
PubMed2006

[Metastasis of a lumbosacral ependymoma with very long disease-free survival: a case report and review of the literature].

Ependymoma is rare glial tumour of the central nervous system and is considered to be low-grade. The lumbosacral location of spinal ependymoma is the most common. Prognosis of ependymomas is dependent on tumour location, histological subtype and differentiation, extent of the tumour and of the completeness of the surgical resection. One of the characteristics of this kind of tumour is to present the possibility of a seeding of the entire cerebrospinal axis, by the way of cerebrospinal liquid. We describe the case of a young male patient operated by incomplete resection of a lumbar ependymoma. Six months later, the patient's symptoms reappeared and an external radiotherapy at curative doses and chemotherapy were delivered. Evolution of the remaining tumour was diagnosed 6 years after at the primary site and operated by large incomplete resection. A second session of radiotherapy was therefore administered. Twenty-four years after this episode, cervical pain and gait troubles appear. Complete imaging study concluded to a cervical extramedullary intradural tumour and to the persistence of the primary lumbosacral tumour. Macroscopical complete resection of the cervical tumour was performed and pathological findings concluded to a metastasis of his lumbar ependymoma. External radiotherapy was delivered on this site with a total dose of 50 Gy. Eight years after this episode, the patient is alive without evidence of distant disease. The primary lumbosacral ependymoma is stable. Ependymomas are often recurrent at the primary site, but can seed on the entire cerebrospinal axis. Awareness of such aberrant tumoral behaviour, even after such a long disease free interval, may warrant more careful follow-up of patients with this diagnosis.

Adolescent↗
PubMed2001

The local field in infratentorial ependymoma: does the entire posterior fossa need to be treated?

INTRODUCTION: In the past decade, there have been multiple reports indicating that the predominant problem in the curative treatment of intracranial ependymoma is local failure. As a result, many have recommended local field radiotherapy. For infratentorial ependymoma, there is controversy regarding what constitutes the local field. Some radiation oncologists advocate coverage of the entire posterior fossa, whereas others recommend radiotherapy to the tumor bed and a safety margin. METHODS AND MATERIALS: From 1984 to 1998, 28 patients with posterior fossa ependymoma were diagnosed at our institution. There were 18 males and 10 females with a median age of 12 years (range, 2-81 years). Four patients (14%) had high-grade ependymoma and 3 (11%) had M+ disease at initial diagnosis. Gross total resection was achieved in 17 (61%) and postoperative radiotherapy (RT) was given to 22 (77%). Radiotherapy fields were craniospinal in 10, whole brain in 1, posterior fossa in 2, and tumor bed with a 2-cm. margin in 9. Median dose to the primary site was 54 Gy (range, 45-55 Gy). All 4 patients with high-grade ependymoma received craniospinal RT. Six patients did not receive RT after surgery. Magnetic resonance imaging (MRI) or computed tomography (CT) scans of the brain at initial diagnosis were compared to MRI or CT scans of patients at relapse to determine if the local relapse was in the tumor bed or nontumor bed posterior fossa. Median follow-up was 127 months (range, 14-188 months). RESULTS: Six patients have relapsed. For the 11 patients who had craniospinal or whole brain radiotherapy (RT), 3 recurred (tumor bed 1, spine 1, nontumor bed posterior fossa + spine 1). Both patients who failed in the spine had high-grade tumors. Neither of the 2 treated with posterior fossa fields relapsed. For the 9 patients who had tumor bed RT alone and the 6 who did not receive RT, there were 3 relapses; all were in the tumor bed. There were no relapses in the nontumor bed posterior fossa. CONCLUSION: For nondisseminated, low-grade infratentorial ependymoma, the radiotherapy volume does not need to include the entire posterior fossa. This information can be used to minimize late effects of RT in the era of three-dimensional (conformal) radiotherapy. No conclusion can be reached regarding the appropriate local field for high-grade infratentorial ependymoma because of the small number of patients.

Adolescent↗