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Tipos de Cancer / Cánceres Pediátricos / Tumores del Cerebro (Infancia) / Recursos de NCI
National Cancer Institute
Ultima Vez Modificado: 9 de abril del 2008
Purpose of This PDQ® Summary
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This PDQ® cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of childhood cerebral astrocytoma/malignant glioma. This summary is reviewed regularly and updated as necessary by the PDQ® Pediatric Treatment Editorial Board.
Information about the following is included in this summary:
This summary is intended as a resource to inform and assist clinicians and other health professionals who care for pediatric cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.
In the summary, treatments are described as standard or conventional and under clinical evaluation. These designations should not be used as a basis for reimbursement determinations.
This summary is also available in a patient version, which is written in less-technical language, and in Spanish. [Note: The PDQ® childhood brain tumor treatment summaries are in the process of being substantially revised. This revision process was prompted by changes in the nomenclature and classification for pediatric central nervous system tumors. New PDQ® childhood brain tumor treatment summaries will be added and some existing summaries will be replaced or their content combined with other PDQ® childhood brain tumor treatment summaries in the near future.]
The National Cancer Institute provides the PDQ® pediatric cancer treatment information summaries as a public service to increase the availability of evidence-based cancer information to health professionals, patients, and the public.
In recent decades, dramatic improvements in survival have been achieved for children and adolescents with cancer. Childhood and adolescent cancer survivors require close follow-up because cancer therapy side effects may persist or develop months or years after treatment. (Refer to the PDQ® Late Effects of Treatment for Childhood Cancer summary for specific information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors.)
Primary brain tumors are a diverse group of diseases that together constitute the most common solid tumor of childhood. Brain tumors are classified according to histology, but tumor location and extent of spread are important factors that affect treatment and prognosis. Immunohistochemical analysis, cytogenetic and molecular genetic findings, and measures of mitotic activity are increasingly used in tumor diagnosis and classification.
Refer to the PDQ® Childhood Brain and Spinal Cord Tumors Treatment Overview summary for information about the general classification of childhood brain and spinal cord tumors.
Cellular Classification
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Various classification schemata have been used to separate glial tumors into prognostic subsets. 1 According to the most recent classification of the World Health Organization, glial tumors are divided on the basis of histologic criteria into the following subsets: pilocytic astrocytomas, low-grade nonpilocytic astrocytomas, anaplastic gliomas, and glioblastoma multiforme. 2 Various types of nonpilocytic astrocytomas, such as fibrillary protoplasmic and gemistocytic, have been identified. Both malignant and benign varieties of oligodendrogliomas may occur. In young children (especially those younger than 1 year at diagnosis), new variants such as dysembryoplastic neuroepithelial tumor and desmoplastic infantile gangliogliomas are increasingly recognized. Mixed gliomas are classified separately, as are gangliogliomas and other primary neuronal tumors. In general, the grade of the tumor is predictive of outcome; patients with higher-grade tumors have a poorer prognosis. High-grade gliomas with p53 expression and mutation are also associated with a poorer prognosis. 3 MIB-1 labeling index, a marker of cellular proliferative activity, is predictive of outcome in childhood malignant brain tumors. Both histologic classification and proliferative activity evaluation have been shown to be independently associated with survival. 4
Low-Grade Cerebral Astrocytoma
Low-grade cerebral astrocytomas (grade I [pilocytic] and grade II) have a relatively favorable prognosis, particularly if complete excision is possible. 1 2 In the World Health Organization brain tumor classification schema, all grade 1 tumors are pilocytic astrocytomas. The pilomyxoid variant of pilocytic astrocytoma may be a more aggressive variant and may be more likely to disseminate. 3 There is no generally recognized staging system. Tumor spread, when it occurs, is usually by contiguous extension; dissemination to other central nervous system sites may rarely occur. Although metastasis is unlikely, tumors may be of multifocal origin, especially when associated with neurofibromatosis type 1 (NF-1). Low-grade astrocytomas have a predilection for certain anatomic locations in children, including the cerebellum, diencephalon (i.e., chiasm, hypothalamus, and thalamus), and brainstem.
Patients with NF-1 constitute a special group of patients with low-grade gliomas. In general, treatment is not required for incidental tumors found with surveillance scans. Symptomatic lesions or those that have radiographically progressed may require treatment. 4
High-Grade Cerebral Astrocytoma
High-grade or malignant astrocytoma (anaplastic astrocytoma [grade III] and glioblastoma multiforme [grade IV]) may occur anywhere above the tentorium. Malignant astrocytoma is often locally invasive and extensive. 1 2 Spread via the subarachnoid space may occur. Metastasis outside of the central nervous system has been reported but is extremely infrequent until after multiple local relapses. There is no generally recognized staging system. Biologic markers, such as p53 overexpression and mutation status, may be useful predictors of outcome in patients with malignant gliomas. 5 Although malignant astrocytoma carries a generally poor prognosis in younger patients, those with anaplastic astrocytoma and those in whom a gross total resection is possible may fare better. 6
Treatment of Low-Grade Childhood Cerebral Astrocytoma
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To determine and implement optimum treatment, treatment planned by a multidisciplinary team of cancer specialists who have experience treating childhood brain tumors is required.
The usual treatment for low-grade supratentorial astrocytoma is determined by location. Hemispheric tumors are often amenable to complete surgical resection. 1 2 Low-grade diencephalic tumors can also be aggressively resected, with resultant long-term disease control; 3 4 however, such resection may result in significant neurologic sequelae, especially in children younger than 2 years at diagnosis. 3 Because of the infiltrative nature of some deep-seeded lesions, extensive surgical resection may not be appropriate and biopsy followed by either radiation or chemotherapy should be considered. Treatment options for patients with incompletely resected tumor must be individualized and may include observation, re-resection, chemotherapy, and radiation.
Radiation therapy is often reserved until progressive disease is documented, 1 5 and its use may be further delayed through the use of chemotherapy, a strategy that is commonly employed in young children. 6 7 Debilitating effects on growth and neurologic development have frequently been observed following radiation therapy, especially in younger children with extensive lesions. For those children with low-grade glioma for whom radiation therapy is indicated, conformal radiotherapeutic approaches appear effective and offer the potential for reducing the acute and long-term toxicities associated with this modality. 8 9
Evaluation with detailed electroencephalographic mapping and surgery designed to remove the tumor and adjacent epileptic foci has been recommended for those patients with low-grade tumor and seizures. 2 Excellent results in tumor and seizure control, however, have been reported with magnetic resonance-based total tumor resection. 10 Chemotherapy can be used to delay, and sometimes obviate, the need for radiation therapy in children with benign lesions. 6 The most widely used regimen to treat progressive or symptomatic nonresectable, low-grade gliomas is a combination of carboplatin and vincristine. 6 7 The 3-year progressive-free survival for children younger than 5 years is 74%, but most patients ultimately require further treatment. Other chemotherapeutic regimens have also been shown to be effective. 11 12 13
Radiation and alkylating agents are used as a last resort for patients with NF-1, given the increased risk of inducing neurotoxicity and second malignancy in this population. 14 15
Treatment Options Under Clinical Evaluation
The following is an example of a national and/or institutional clinical trial that is currently being conducted. Information about ongoing clinical trials is available from the NCI Web site.
The designations in PDQ® that treatments are standard or under clinical evaluation are not to be used as a basis for reimbursement determinations.
Check for U.S. clinical trials from NCI's PDQ® Cancer Clinical Trials Registry that are now accepting patients with childhood low-grade cerebral astrocytoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Treatment of High-Grade Childhood Cerebral Astrocytoma
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The therapy for both children and adults with supratentorial high-grade astrocytoma includes surgery, radiation therapy, and chemotherapy. Outcome in high-grade gliomas occurring in childhood may be more favorable than that in adults, but it is not clear if this difference is caused by biologic variations in tumor characteristics, therapies used, tumor resectability, or other factors that are not presently understood. 1 The ability to obtain a complete resection is associated with a better prognosis. 2 Radiation therapy is administered to a field that widely encompasses the entire tumor. Alternatively, it can be administered to the entire brain with a cone down to the tumor volume. 3 The radiation therapy dose to the tumor bed is usually at least 54 Gy. Despite such therapy, overall survival rates remain poor. Among patients treated with surgery, radiation therapy and nitrosourea (lomustine)-based chemotherapy, 5-year progression-free survival was 19% 3%; survival was 40% in those who had total resections. 4 In one trial, children with glioblastoma multiforme who were treated on a prospective randomized trial with adjuvant lomustine, vincristine, and prednisone fared better than those children treated with radiation therapy alone. 5 In adults, the addition of temozolomide (Temodal) during and after radiation therapy resulted in improved 2-year event-free survival as compared to treatment with radiation therapy alone. Adult patients with glioblastoma multiforme with a methylated O6-methylguanine-DNA-methyltransferase (MGMT) promoter benefited from temozolomide, whereas those who did not have a methylated MGMT promoter did not. 6 7 The role of temozolomide given concurrently with radiation therapy for children with supratentorial high-grade gliomas has not yet been demonstrated but is under clinical evaluation. Children younger than 3 years may benefit from chemotherapy to delay, modify, or, in selected cases, obviate the need for radiation therapy. 8 9 Clinical trials that evaluate chemotherapy with or without radiation therapy are ongoing. Information about ongoing clinical trials is available from the NCI Web site.
Treatment Options Under Clinical Evaluation
The following is an example of a national and/or institutional clinical trial that is currently being conducted. Information about ongoing clinical trials is available from the NCI Web site.
Check for U.S. clinical trials from NCI's PDQ® Cancer Clinical Trials Registry that are now accepting patients with childhood high-grade cerebral astrocytoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Recurrent Childhood Cerebral Astrocytoma
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Recurrent Low-Grade Cerebral Astrocytoma
Low-grade glioma may recur many years after initial treatment. 1 Disease typically recurs at the primary tumor site. Multifocal or disseminated disease is rare but can occur. 2 Most children whose low-grade fibrillary astrocytomas recur will harbor low-grade lesions; however, malignant transformation is possible. 3 Biopsy or surgical resection may be necessary for confirmation of relapse because other entities, such as secondary tumor and radiation-related brain necrosis, may be clinically indistinguishable from tumor recurrence. The need for further surgical intervention must be individualized on the basis of the initial tumor type, the length of time between initial treatment and the reappearance of the mass lesion, and the clinical status of the child.
An individual plan needs to be tailored on the basis of patient age, tumor location, and prior treatment. Drug combinations, such as carboplatin and vincristine, may be useful at the time of recurrence for children with low-grade gliomas. 4 5 For those children with recurrent low-grade glioma for whom radiation therapy is indicated, conformal radiotherapeutic approaches appear effective and offer the potential for reducing the acute and long-term toxicities associated with this modality. 6 7
Recurrent High-Grade Cerebral Astrocytoma
Most patients with high-grade gliomas will eventually have tumor recurrence, usually within 3 years of original diagnosis but perhaps many years after initial treatment. Disease may recur at the primary tumor site, at the margin of the resection/radiation bed, or at noncontiguous central nervous system sites. Systemic relapse is rare but may occur. At the time of recurrence, a complete evaluation for extent of relapse is indicated for all malignant tumors. Biopsy or surgical resection may be necessary for confirmation of relapse because other entities, such as secondary tumor and treatment-related brain necrosis, may be clinically indistinguishable from tumor recurrence. The need for surgical intervention must be individualized on the basis of the initial tumor type, the length of time between initial treatment and the reappearance of the mass lesion, and the clinical picture.
Patients for whom initial treatment fails may benefit from additional treatment. 8 High-dose chemotherapy with hematopoietic stem cell transplant may be effective in a subset of patients with minimal residual disease at time of treatment. 9 10 Such patients should also be considered for entry into trials of novel therapeutic approaches. Information about ongoing clinical trials is available from the NCI Web site.
Check for U.S. clinical trials from NCI's PDQ® Cancer Clinical Trials Registry that are now accepting patients with recurrent childhood cerebral astrocytoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Get More Information From NCI
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Call 1-800-4-CANCER
For more information, U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237) Monday through Friday from 9:00 a.m. to 4:30 p.m. A trained Cancer Information Specialist is available to answer your questions.
Chat online
The NCI's LiveHelp online chat service provides Internet users with the ability to chat online with an Information Specialist. The service is available from 9:00 a.m. to 11:00 p.m. Eastern time, Monday through Friday. Information Specialists can help Internet users find information on NCI Web sites and answer questions about cancer.
Write to us
For more information from the NCI, please write to this address:
Search the NCI Web site
The NCI Web site provides online access to information on cancer, clinical trials, and other Web sites and organizations that offer support and resources for cancer patients and their families. For a quick search, use the search box in the upper right corner of each Web page. The results for a wide range of search terms will include a list of "Best Bets," editorially chosen Web pages that are most closely related to the search term entered.
There are also many other places to get materials and information about cancer treatment and services. Hospitals in your area may have information about local and regional agencies that have information on finances, getting to and from treatment, receiving care at home, and dealing with problems related to cancer treatment.
Find Publications
The NCI has booklets and other materials for patients, health professionals, and the public. These publications discuss types of cancer, methods of cancer treatment, coping with cancer, and clinical trials. Some publications provide information on tests for cancer, cancer causes and prevention, cancer statistics, and NCI research activities. NCI materials on these and other topics may be ordered online or printed directly from the NCI Publications Locator. These materials can also be ordered by telephone from the Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237).
Changes to This Summary (04/09/2008)
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The PDQ® cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Editorial changes were made to this summary.
Important:
This information is intended mainly for use by doctors and other health care professionals. If you have questions about this topic, you can ask your doctor, or call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).
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Calcium Leucovorin, Citrovorum Factor, Folinic Acid
Cladribine (2-CDA, Leustatin®)
Cyclophosphamide (Cytoxan®, Neosar®, Endoxan®)
Cyclosporine (Neoral®, Sandimmune®, Restasis®, Gengraf®)
Cytarabine (Cytosar-U®, Ara-C)
Irinotecan (Camptosar®, CPT-11)
Leucovorin (Calcium Leucovorin, Citrovorum Factor, Folinic Acid)
Calcium Leucovorin, Citrovorum Factor, Folinic Acid
Leucovorin (Calcium Leucovorin, Citrovorum Factor, Folinic Acid)
Leuprolide Acetate (Lupron®, Lupron Depot®, Eligard®, Prostap®, Viadur®) - For Men
Leuprolide Acetate (Lupron®, Lupron Depot®, Eligard®, Prostap®, Viadur®) - For Women
Lupron®, Lupron Depot®, Eligard®, Prostap®, Viadur®
Lupron®, Lupron Depot®, Eligard®, Prostap®, Viadur®
Busulfan (Myleran®, Busulfex®)
Intravesicular Mitomycin (Mutamycin®, Mitomycin-C, given into the bladder)
Mechlorethamine (Mustargen®, Nitrogen Mustard)
mechlorethamine, mustine, Mustargen®
Megestrol (Megace®, Megace-ES®)
Mercaptopurine (Purinethol®, 6-MP)
Methotrexate (Mexate®, Folex®, Rheumatrex®, Amethopterin, MTX)
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