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This PDQ cancer information summary for health professionals provides comprehensive, 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.
This summary is also available in a patient version, which is written in less technical language, and in Spanish.
Note: Separate PDQ summaries on Childhood Astrocytomas Treatment, Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment, Childhood Brain Stem Glioma Treatment, Childhood Central Nervous System Embryonal Tumors Treatment, Childhood Ependymoma Treatment, and Childhood Craniopharyngioma Treatment are also available.
The National Cancer Institute (NCI) 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.
Primary 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:
Primary 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 PDQ summary on Childhood Ependymoma Treatment for more information), higher-grade glial tumors, and (rarely) primitive neuroectodermal tumors (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]
Important concepts that should be understood by those treating and caring for a child who has a brain or spinal cord tumor include the following:
Childhood astrocytomas are classified as low-grade or high-grade.
Refer to the PDQ summary on Childhood Astrocytomas Treatment for more information.
Refer to the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment for more information.
Childhood brain stem gliomas include:
Refer to the PDQ summary on Childhood Brain Stem Glioma Treatment for more information.
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 include:
Germ cell brain tumors usually arise in the pineal or suprasellar regions. Histologic subtypes include teratomas (both mature and immature), germinomas, choriocarcinomas, and nongerminomatous germ cell tumors (i.e., embryonal cell carcinoma, yolk cell or endodermal sinus tumors, 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]
Refer to the PDQ summary on Childhood Craniopharyngioma Treatment for more information.
Refer to the PDQ summary on Childhood Ependymoma Treatment for more information.
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Refer to the PDQ summary on Childhood Astrocytomas Treatment for more information.
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Childhood pineal parenchymal tumors include:
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
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. [3] [4] 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 include:
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Many 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 very technically demanding and should be carried out in centers that have experience in this area 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.
Childhood astrocytomas are classified as low-grade or high-grade.
Refer to the PDQ summary on Childhood Astrocytomas Treatment for more information.
Refer to the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment for more information.
Childhood brain stem gliomas include:
Refer to the PDQ summary on Childhood Brain Stem Glioma Treatment for more information
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with childhood brain stem glioma. 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.
Childhood CNS embryonal tumors include:
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with childhood embryonal 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.
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 54 Gy with 27 Gy to 36 Gy to the whole brain and spine. Although nongerminomatous germ cell tumors (e.g., embryonal carcinomas, yolk cell tumors, and mixed germ cell tumors) may respond to chemotherapeutic agents (e.g., 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]
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with childhood central nervous system germ cell 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.
Refer to the PDQ summary on Childhood Craniopharyngioma Treatment for more information.
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with childhood craniopharyngioma. 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.
Refer to the PDQ summary on Childhood Ependymoma Treatment for more information.
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with newly diagnosed childhood ependymoma. 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.
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with childhood ependymoblastoma. 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.
Refer to the PDQ summary on Childhood Astrocytomas Treatment for more information.
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with childhood cerebral astrocytoma/malignant glioma. 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.
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with untreated childhood medulloblastoma. 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.
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with childhood medulloepithelioma. 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.
Childhood pineal parenchymal tumors include:
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with childhood pineal parenchymal 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.
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. [7] [8] 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. [7] [8] 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. [7] 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. [7] Radiation therapy has been demonstrated to control disease in some patients with low-grade glial tumors after subtotal resections. [8] [9] [10] 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. [11] 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. [7] [9] [10]
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. [7] [8] 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 Central Nervous System Embryonal Tumors Treatment for more information.
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with childhood spinal cord neoplasm. 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.
Childhood supratentorial primitive neuroectodermal tumors include:
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with childhood supratentorial primitive neuroectodermal 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.
Recurrence 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 astrocytomas are classified as low-grade or high-grade.
Refer to the PDQ summary on Childhood Astrocytomas Treatment for more information.
Refer to the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment for more information.
Childhood brain stem gliomas include:
Refer to the PDQ summary on Childhood Brain Stem Glioma Treatment for more information.
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent childhood brain stem glioma. 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.
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 aged 2 years and younger, 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 include:
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Childhood CNS germ cell tumors include:
Germ cell tumors may be chemoresponsive. Patients may benefit from the types of agents that are used to 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.
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with childhood central nervous system germ cell 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.
Refer to the PDQ summary on Childhood Craniopharyngioma Treatment for more information.
Refer to the PDQ summary on Childhood Ependymoma Treatment for more information.
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent childhood ependymoma. 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.
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
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.
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent childhood medulloblastoma. 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.
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with childhood medulloepithelioma. 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.
Childhood pineal parenchymal tumors include:
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent childhood pineoblastoma. 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.
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 and Childhood Central Nervous System Embryonal Tumors Treatment.
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent childhood spinal cord neoplasm. 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.
Childhood suprantentorial primitive neuroectodermal tumors include:
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent childhood supratentorial primitive neuroectodermal 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.
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