The Role of Chemotherapy inNewly Diagnosed Ependymoma of Childhood

Souweidane, Mark M.; Bouffet, Éric; Finlay, Jonathan L.

doi:10.1159/000028664

Cited by 34 publications

(13 citation statements)

References 33 publications

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“…Chemotherapy has not yielded a significant improvement in survival for patients with ependymal tumors. 46,55 High-dose chemotherapy followed by autologous bone marrow transplantation has been tried experimentally. 40 At this point, the data are not sufficient to justify the use of chemotherapy in patients with ependymomas, except in younger children (to substitute for or delay radiation therapy 56 ) or patients in whom surgery and radiation therapy have failed to control tumor growth.…”

Section: Ependymomamentioning

confidence: 99%

Initial Management of Childhood Brain Tumors: Neurosurgical Considerations

2008

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Intracranial tumors are the most common solid tumors in children. The infratentorial compartment will be the primary site for 60% to 70% of these tumors, including astrocytomas, medulloblastomas, and ependymomas. Several technological advancements have increased our knowledge of the cell biology of pediatric brain tumors, facilitated earlier diagnosis, and improved neurosurgical resections while minimizing neurological deficits. These in turn have not only improved the survival of children with brain tumors but also their quality of life. Current management strategies in most cases rely on surgery coupled with adjuvant therapies, including radiation therapy and chemotherapy. The vulnerability of the immature brain to adjuvant therapies creates many challenges for the treating physician. We review current diagnostic and therapeutic approaches and outcome for children harboring the most common pediatric brain tumors: astrocytomas (low- and high-grade glioma), ependymoma, medulloblastoma, and craniopharyngioma. The emphasis will be on the neurosurgical management of children with these tumors.

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Section: Ependymomamentioning

confidence: 99%

Initial Management of Childhood Brain Tumors: Neurosurgical Considerations

2008

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“…5,7,23 The following clinical variables seem to favor long-term survival: 1) adult age; 2) tumors located within the cerebral hemisphere; 3) more benign histological appearance; and 4) total resection. 29 In another retrospective study of 31 children presenting with intracranial ependymomas between 1976 and 1993, it was found that failure at the primary site was the major obstacle to improved cure rates. 27 In a review of 30 patients harboring histologically confirmed posterior fossa ependymomas who underwent surgery at the Mayo Clinic (age range 1-69 years), the following factors were found: 1) younger (Յ 5 years) age was associated with a poorer prognosis; 2) there was a trend toward a better 5-year survival rate with a gross-total resection; 3) tumors recur at the primary intracranial site; and 4) symptomatic spinal seeding does not occur frequently.…”

Section: Prognosis and Survivalmentioning

confidence: 99%

“…Radiation ranging from 3500 to 7200 cGy doses have been given over 5 to 6 weeks. 29 For chemotherapy of ependymomas, the platinum compounds, cisplatin and carboplatin, may be the most effective agents discovered to date. 6 For tumors in the posterior fossa, the recommended dose is 5400 cGy.…”

Section: Surgery Radiation Therapy Andmentioning

confidence: 99%

“…Craniospinal-axis fields are used only when spinal seeding is radiologically or pathologically evident. 29 Tomita, et al, 31 concluded that, according to their experience, recurrent tumors are resistant to chemotherapy. Because of the severity of this disease, however, even young children with intracranial ependymomas usually require chemo-and radiation therapy.…”

Section: Surgery Radiation Therapy Andmentioning

confidence: 99%

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Intracranial ependymoma

Maksoud¹,

Hahn²,

Engelhard³

2002

FOC

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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 ependy-momas 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 radio-surgery 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.

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“…Standard treatment of MEPN is aggressive surgery. However, tumor recurrence can occur with incomplete resection and the role of adjuvant chemotherapy (25, 36, 37) or radiotherapy (30, 33, 41) is unresolved for the subset of patients with recurrence or in patients in whom gross total resection cannot be achieved.…”

Section: Introductionmentioning

confidence: 99%

Unique Molecular Characteristics of Pediatric Myxopapillary Ependymoma

Barton

Donson

Kleinschmidt‐DeMasters

et al. 2010

Brain Pathology

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Myxopapillary ependymoma (MEPN) generally can be cured by gross total surgical resection and usually manifest a favorable prognosis. However, surgery is less curative in tumors that are large, multifocal or extend outside the thecal sac. Late recurrences may occur, particularly in pediatric patients. The role of adjuvant therapy is unclear in the clinical management of recurrent tumors. Clinical trial design requires a better understanding of tumor biology. Unique molecular features of MEPN were investigated by using microarray technology to compare the gene expression of five pediatric MEPN to 24 pediatric intracranial ependymoma (EPN). The upregulation of three genes of interest, homeobox B13 (HOXB13), neurofilament, light polypeptide (NEFL) and PDGFRα, was further studied by immunohistochemistry in a larger cohort that included adult MEPN and EPN specimens. Protein expression in MEPN was compared to subependymoma, spinal EPN, intracranial EPN and normal fetal and adult ependyma. Immunoreactivity for HOXB13, NEFL and PDGFRα was strongest in MEPN and virtually absent in subependymoma. Spinal and intracranial EPN generally expressed weak or focal staining. MEPN manifests unique gene and protein expression patterns compared to other EPNs. Aberrant expression of HOXB13 suggests possible recapitulation of developmental pathways in MEPN tumorigenesis. PDGFRα may be a potential therapeutic target in recurrent MEPN.

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The Role of Chemotherapy inNewly Diagnosed Ependymoma of Childhood

Cited by 34 publications

References 33 publications

Initial Management of Childhood Brain Tumors: Neurosurgical Considerations

Initial Management of Childhood Brain Tumors: Neurosurgical Considerations

Intracranial ependymoma

Unique Molecular Characteristics of Pediatric Myxopapillary Ependymoma

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