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Illness and Conditions - Nci
Brain cancer, childhood (general information): Treatment - Health Professional Information [NCI PDQ]
Childhood Brain and Spinal Cord Tumors Treatment (PDQ�(r))Purpose of This PDQ SummaryThis PDQ cancer information summary for health professionals provides peer-reviewed, evidence-based information about the treatment of select childhood brain and spinal cord tumors. It also provides links to the other PDQ childhood brain and spinal cord tumor summaries. 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 this summary, treatments are described as 'standard' or 'conventional' and 'under clinical evaluation.' These designations should not be used as a basis for reimbursement determinations. 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. General InformationNote: Separate PDQ summaries on Childhood Brain Stem Glioma Treatment,Childhood Central Nervous System Embryonal Tumors, Treatment, Childhood Cerebellar Astrocytoma Treatment,Childhood Cerebral Astrocytoma/Malignant Glioma Treatment ,Childhood Ependymoma Treatment, Childhood Medulloblastoma Treatment ,Childhood Supratentorial Primitive Neuroectodermal Tumors and Pineoblastoma Treatment and Childhood Visual Pathway and Hypothalamic Glioma Treatment are also available. The National Cancer Institute provides the PDQ pediatric cancer information treatment summaries as a public service to increase the availability of evidence-based cancer information to health professionals, patients, and the public. Information about ongoing clinical trials is available from the NCI Web site. Current Clinical Trials Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with childhood brain tumor. 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. Classification of Brain TumorsPrimary brain tumors are a diverse group of diseases that together constitute the most common solid tumor in childhood. Between 2,500 and 3,500 children are diagnosed in the United States each year. Immunohistochemical analysis, cytogenetic and molecular genetic findings, and measures of mitotic activity are increasingly used in tumor diagnosis and classification, and will likely alter classification and nomenclature in the future. The classification of childhood brain tumors is based on histology and location.[1] Tumors are classically categorized as infratentorial, supratentorial, sellar, or suprasellar. Common infratentorial (posterior fossa) tumors include:
Supratentorial tumors include:
Sellar or suprasellar tumors include:
References: Classification of Spinal Cord TumorsPrimary central nervous system spinal cord tumors comprise approximately 1% to 2% of all childhood nervous system tumors.[1,2,3] As is the case for primary brain tumors, such lesions are histologically heterogeneous. Approximately 70% of all intramedullary spinal cord tumors will be low-grade astrocytomas and/or gangliogliomas. Other tumor types that occur include ependymomas (refer to the Childhood Ependymoma Treatment for more information), higher-grade glial tumors, and (rarely) primitive neuroectodermal tumors (refer to the PDQ summary on Childhood Medulloblastoma Treatment for more information). (Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.) Myxopapillary ependymomas have a tendency to develop in the conus and cauda equina regions. Symptoms and signs of spinal cord tumors are highly dependent on the location of the tumor and its extent; some low-grade spinal cord tumors are associated with large cysts that extend rostrally and caudally. At times it is impossible to distinguish a tumor that arises in the medulla from a tumor that arises in the upper cervical cord. The classification of spinal cord tumors is based on histopathologic characteristics of the tumor and does not differ from that of primary brain tumors.[1,2,3] References:
General Approach to Care for Children with Brain and Spinal Cord TumorsImportant concepts that should be understood by those treating and caring for a child who has a brain or spinal cord tumor include the following:
References:
Stage InformationChildhood Brain Stem Glioma Childhood brain stem gliomas include:
Refer to the PDQ summary on Childhood Brain Stem Glioma Treatment for more information. Childhood Central Nervous System (CNS) Embryonal Tumors Childhood CNS embryonal tumors include:
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information. Childhood CNS Germ Cell Tumors Childhood CNS germ cell tumors include:
Germ cell brain tumors usually arise in the pineal or suprasellar regions. Histologic subtypes include teratoma (both mature and immature), germinoma, choriocarcinomas, and nongerminomatous germ cell tumors (i.e., embryonal cell carcinoma, yolk cell or endodermal sinus tumor, and mixed germ cell tumors). These tumors have a propensity for subarachnoid spread. Every patient with a germinoma or malignant germ cell tumor should be evaluated with diagnostic imaging of the spinal cord and whole brain. The best method for evaluating spinal cord subarachnoid metastasis is magnetic resonance imaging with gadolinium enhancement. Cerebrospinal fluid CSF) should be examined cytologically and levels of alpha-fetoprotein (AFP) and human chorionic gonadotropin (HCG) determined. AFP and/or HCG may be elevated in the serum of such patients. Prognosis is related to histology; patients with pure germinoma have a more favorable outcome than those with nongerminomatous germ cell tumors (nongerminomas).[1,2] Childhood Cerebellar Astrocytoma Refer to the PDQ summary on Childhood Cerebellar Astrocytoma Treatment for more information. Childhood Cerebral Astrocytoma Refer to the PDQ summary on Childhood Cerebral Astrocytoma/Malignant Glioma Treatmentfor more information. Childhood Craniopharyngioma These are symptomatic benign tumors arising from remnants of Rathke's pouch. There is no generally accepted staging system and metastasis is rare.[3,4,5,6] Childhood Ependymoma Refer to the PDQ summary on Childhood Ependymoma Treatment for more information. Childhood Ependymoblastoma Refer to the PDQ on Childhood Central Nervous System Embryonal Tumors Treatment for more information. Childhood Malignant Glioma Refer to the PDQ summary on Childhood Cerebral Astrocytoma/Malignant Glioma Treatment for more information. Childhood Medulloblastoma Refer to the PDQ summaries on Childhood Medulloblastoma Treatment and Childhood Central Nervous System Embryonal Tumors Treatment for more information. Childhood Medulloepithelioma Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information. Childhood Pineal Parenchymal Tumors Childhood pineal parenchymal tumors include:
Refer to the PDQ summaries on Childhood Supratentorial Primitive Neuroectodermal Tumors and Pineoblastoma Treatment and Childhood Central Nervous System Embryonal Tumors Treatment for more information. Childhood Spinal Cord Tumors There is no uniformly accepted staging system for childhood primary spinal cord tumors. These tumors are classified based on their location within the spinal cord and histology. Low-grade spinal cord tumors rarely disseminate elsewhere in the nervous system; however, higher grade tumors may disseminate.[7,8] Despite this, because of the location of the tumor and concerns over causing further neurologic deterioration by CSF attainment, routine lumbar spinal punctures are not indicated in the evaluation of a child with a spinal cord tumor. For high-grade glial spinal cord tumors, and possibly lower grade tumors and ependymomas, (refer to the PDQ summary on Childhood Ependymoma Treatment for more information) neuroimaging of the entire neuroaxis (brain and entire spine) is indicated at the time of diagnosis for determination of extent of disease. Childhood Supratentorial Primitive Neuroectodermal Tumors Childhood supratentorial primitive neuroectodermal tumors include:
Refer to the PDQ summaries on Childhood Supratentorial Primitive Neuroectodermal Tumors and Pineoblastoma Treatment and Childhood Central Nervous System Embryonal Tumors Treatment for more information. Childhood Visual Pathway and Hypothalamic Glioma Refer to the PDQ summary on Childhood Visual Pathway and Hypothalamic Glioma Treatment for more information. Current Clinical Trials Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with childhood brain tumor. 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. References:
Treatment Option OverviewMany of the improvements in survival in childhood cancer have been made as a result of clinical trials that have attempted to improve on the best accepted therapy available. Clinical trials in pediatrics are designed to compare new therapy with therapy that is currently accepted as standard. This comparison may be done in a randomized study of two treatment arms or by evaluating a single new treatment and comparing the results with those previously obtained with existing therapy. Because of the relative rarity of cancer in children, all patients with brain and spinal cord tumors should be considered for entry into a clinical trial. To determine and implement optimum treatment, treatment planning by a multidisciplinary team of cancer specialists who have experience treating childhood brain tumors is required. Radiation therapy of pediatric brain tumors is technically very demanding and should be carried out in centers that have experience in this area in order to ensure optimal results. Debilitating effects on growth and neurologic development have frequently been observed following radiation therapy, especially in younger children.[1,2,3] Secondary tumors have increasingly been diagnosed in long-term survivors.[4] For this reason, the role of chemotherapy in allowing a delay or reduction in the administration of radiation therapy is under study, and preliminary results suggest that chemotherapy can be used to delay, limit, and sometimes obviate, the need for radiation therapy in children with benign and malignant lesions.[5,6,7] Long-term management of these patients is complex and requires a multidisciplinary approach. The designations in PDQ that treatments are 'standard' or 'under clinical evaluation' are not to be used as a basis for reimbursement determinations. Current Clinical Trials Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with childhood brain tumor. 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. References:
Treatment of Newly Diagnosed Childhood Brain and Spinal Cord TumorsChildhood Brain Stem Glioma Childhood brain stem gliomas include:
Refer to the PDQ summary on Childhood Brain Stem Glioma Treatment for more information Childhood Central Nervous System (CNS) Embryonal Tumors Childhood CNS embryonal tumors include:
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information. Childhood CNS Germ Cell Tumors Childhood CNS germ cell tumors include:
Surgery, other than biopsy, to establish the diagnosis rarely plays a role in the treatment of CNS germinomas. The role of surgical resection for nongerminomatous germ cell tumors and teratomas remains to be defined.[1] For germinomas, irradiation with doses of 45 Gy to 54 Gy to the tumor and 21 Gy to 36 Gy to the whole brain and spine is usually curative. In selected cases, germinoma can be effectively treated with ventricular field radiation therapy and at lower dose levels (30-36 Gy) following response to chemotherapy.[1] Although experience with pre-irradiation chemotherapy has shown that most of these tumors respond to cyclophosphamide and platinum-containing drugs, the definitive role of chemotherapy has yet to be determined.[1] Disseminated germinomas are treated with craniospinal irradiation,[2] [3] alone or in combination with chemotherapy. The usual dose to the tumor is 45 Gy to 54Gy with 27 Gy to 36 Gy to the whole brain and spine. Although nongerminomatous germ cell tumors, such as embryonal carcinomas, yolk cell tumors, and mixed germ cell tumors, may respond to chemotherapeutic agents such as cisplatin or carboplatin, etoposide, cyclophosphamide, and vinblastine, as do such histologies outside of the CNS, optimal combination of agents, and the timing of chemotherapy in relation to radiation therapy remains to be determined.[4,5,6] Information about ongoing clinical trials is available from the NCI Web site. Childhood Cerebellar Astrocytoma Refer to the PDQ summary on Childhood Cerebellar Astrocytoma Treatment for more information. Childhood Cerebral Astrocytoma Refer to the PDQ summary on Childhood Cerebral Astrocytoma/Malignant Glioma Treatment for more information. Childhood Craniopharyngioma Therapies for craniopharyngioma include surgery and conventional external radiation therapy, and in selected cases, stereotactic radiosurgery or intracavitary irradiation. In general, each of these modalities, either alone or in combination, can give a high rate of long-term disease control in the majority of patients. Debate centers on the relative morbidity of the different approaches.[7,8,9,10,11,12,13] Treatment of cystic tumors with intracavitary chemotherapy has also been reported.[14] Childhood Ependymoma Refer to the PDQ summary on Childhood Ependymoma Treatment for more information. Childhood Ependymoblastoma Refer to the PDQ on Childhood Central Nervous System Embryonal Tumors Treatment for more information. Childhood Malignant Glioma Refer to the PDQ summary on Childhood Cerebral Astrocytoma/Malignant Glioma Treatment for more information. Childhood Medulloblastoma Refer to the PDQ summaries on Childhood Medulloblastoma Treatment and Childhood Central Nervous System Embryonal Tumors Treatment for more information. Childhood Medulloepithelioma Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information. Childhood Pineal Parenchymal Tumors Childhood pineal parenchymal tumors include:
Refer to the PDQ summaries on Childhood Supratentorial Primitive Neuroectodermal Tumors and Pineoblastoma Treatment and Childhood Central Nervous System Embryonal Tumors Treatment for more information. Childhood Spinal Cord Tumors The optimal treatment for intrinsic/intramedullary glial spinal cord tumors has not been determined by prospective randomized trials. Therapeutic options include surgery alone, surgery plus local radiation therapy, and possibly adjuvant chemotherapy in selected cases.[15,16] Extensive surgical resections are technically possible for many patients with intramedullary spinal cord tumors, but may result in worsening neurologic status in at least 10% of cases.[15,16] Surgery is usually indicated at least to determine the type of tumor present; for low-grade glial tumors this may be the only treatment required.[15] In one recent series of 164 children and young adults with intramedullary low-grade glial tumors or ganglioglial spinal cord tumors, 70% were controlled for 5 years after extensive surgical resections.[15] Radiation therapy has been demonstrated to control disease in some patients with low-grade glial tumors after subtotal resections.[16,17,18] The role of chemotherapy for spinal cord tumors is poorly characterized, but some very young children with low-grade glial tumors have been successfully treated with a carboplatin and vincristine drug regimen.[19] Outcomes for patients with high-grade glial tumors have been extremely poor; most develop progressive disease within 3 years of treatment with surgery, radiation, and/or chemotherapy.[15,17,18] The optimal treatment for children with spinal ependymomas has not been well characterized (refer to the PDQ summary on Childhood Ependymoma Treatment for more information). As is the case for glial tumors, treatment options predominantly consist of either surgery alone or surgery followed by local radiation therapy.[15,16] Management of primitive neuroectodermal tumors of the spinal cord is also not well delineated, and most patients are treated on treatment protocols designed for children with high-risk medulloblastoma. (Refer to the PDQ summary on Childhood Medulloblastoma Treatment for more information.) Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information. Childhood Supratentorial Primitive Neuroectodermal Tumors Childhood supratentorial primitive neuroectodermal tumors include:
Refer to the PDQ summaries on Childhood Supratentorial Primitive Neuroectodermal Tumors and Pineoblastoma Treatment and Childhood Central Nervous System Embryonal Tumors Treatment for more information. Childhood Visual Pathway and Hypothalamic Glioma Refer to the PDQ summary on Childhood Visual Pathway and Hypothalamic Glioma Treatment for more information. References:
Treatment of Recurrent Childhood Brain and Spinal Cord TumorsRecurrence is not uncommon in both low-grade and malignant childhood brain tumors and may occur many years after initial treatment.[1] Disease may occur at the primary tumor site or, especially in malignant tumors, at noncontiguous central nervous system (CNS) sites. Systemic relapse is rare but may occur. At time of recurrence, a complete evaluation for extent of relapse is indicated for all malignant tumors and, at times, for lower-grade lesions. Biopsy or surgical re-resection may be necessary for confirmation of relapse; 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 based on the initial tumor type, the length of time between initial treatment and the reappearance of the lesion, and the clinical picture. Childhood Brain Stem Glioma Childhood brain stem gliomas include:
Refer to the PDQ summary on Childhood Brain Stem Glioma Treatment for more information. CNS Tumors in Children Younger Than 3 Years Studies have addressed the treatment of infants who have progressive disease in spite of chemotherapy. Approaches that have been used include further surgery, chemotherapy, local and/or craniospinal radiation therapy, high-dose chemotherapy supported by autologous stem cell rescue, or combinations of chemotherapy and radiation therapy. Overall salvage rates have been less than optimal, but a subgroup of children, primarily those with localized disease at the time of relapse, may experience prolonged disease control and possible 'cure' with treatment after recurrence.[2] [3,4,5,6,7] For children younger than 3 years, the use of high-dose craniospinal irradiation has been associated with poor neurocognitive outcome. Treatment for young children with multiple recurrent and/or disseminated brain tumors is even more problematic and entry into phase I and phase II trials is indicated to identify more effective and less toxic agents. Childhood CNS Embryonal Tumors ChildhoodCNS embryonal tumors include:
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors for more information. Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information. Childhood Central Nervous System CNS Germ Cell Tumors Childhood central nervous system CNS germ cell tumors include:
Germ cell tumors may be chemoresponsive. Patients may benefit from the types of agents that are used into treat germ cell tumors in other locations;these agents include: cisplatin, etoposide, and cyclophosphamide. Patients with recurrent germ cell tumors for whom the standard chemotherapy options have failed may be entered into phase I and phase II studies that are designed to determine the activity and toxic effects of agents new to the treatment of this tumor. Childhood Cerebellar Astrocytoma Refer to the PDQ summary on Childhood Cerebellar Astrocytoma Treatment for more information. Childhood Cerebral Astrocytoma/Malignant Glioma Refer to the PDQ summary on Childhood Cerebral Astrocytoma/Malignant Glioma Treatment for more information. Childhood Ependymoma Refer to the PDQ summary on Childhood Ependymoma Treatment for more information. Childhood Ependymoblastoma Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information. Childhood Low-Grade Glial Tumors Surgical resection, radiation therapy (especially if not previously given), and chemotherapy may result in prolonged disease stabilization for children with recurrent low-grade tumors. Resection is an option for those patients with a surgically accessible lesion and has the advantage of documenting the histology of the recurrent tumor. Radiation therapy, if not previously given, may result in tumor shrinkage and long-term disease control. Chemotherapy with drugs such as carboplatin and vincristine has recently been shown to result in tumor shrinkage and disease control for children with low-grade glial neoplasms.[8] Similar results have been demonstrated for hypothalamic and chiasmatic tumors treated with etoposide.[9] Entry into phase I and phase II trials is indicated to identify more effective and less toxic agents. Childhood Medulloblastoma Refer to the PDQ summary on Childhood Medulloblastoma Treatment and Childhood Central Nervous System Embryonal Tumors Treatment for more information. Childhood Medulloepithelioma Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information. Childhood Pineal Parenchymal Tumors Childhood pineal parenchymal tumors include:
Refer to the PDQ summaries on Childhood Supratentorial Primitive Neuroectodermal Tumors and Pineoblastoma Treatment and Childhood Central Nervous System Embryonal Tumors Treatment for more information. Childhood Spinal Cord Tumors At the time of recurrence, low-grade spinal cord glial tumors can be treated with re-resection with or without the use of radiation therapy. Recurrent low-grade and high-grade tumors which cannot be re-resected can be treated on protocols designed for histologically similar brain tumors. For more information, refer to the PDQ summaries on Childhood Ependymoma Treatment, Childhood Medulloblastoma Treatment, and Childhood Central Nervous System Embryonal Tumors Treatment. Childhood Supratentorial Primitive Neuroectodermal Tumors Childhood suprantentorial primitive neuroectodermal tumors include:
Refer to the PDQ summaries on Childhood Supratentorial Primitive Neuroectodermal Tumors and Pineoblastoma Treatment and Childhood Central Nervous System Embryonal Tumors Treatment for more information. Childhood Visual Pathway and Hypothalamic Glioma Refer to the PDQ summary on Childhood Visual Pathway and Hypothalamic Glioma Treatment for more information. Current Clinical Trials Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent childhood brain tumor. 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. References:
Get More Information From NCICALL 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. Deaf and hard-of-hearing callers with TTY equipment may call 1-800-332-8615. The call is free and a trained Cancer Information Specialist is available to answer your questions. CHAT ONLINE The NCI's LiveHelp�(r) 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 our 'Best Bets' search box in the upper right hand corner of each Web page. The results that are most closely related to your search term will be listed as Best Bets at the top of the list of search results. 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), TTY at 1-800-332-8615. Changes to This Summary (04/03/2008)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. This summary was extensively revised. More InformationABOUT PDQ
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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). Date Last Modified: 2008-04-03
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