Purpose of This PDQ Summary
General Information
Classification of Brain Tumors
Classification of Spinal Cord Tumors
General Approach to Care for Children with Brain and Spinal Cord Tumors
Stage Information

Childhood Central Nervous System (CNS) Atypical Teratoid/Rhabdoid Tumor Childhood Brain Stem Glioma Childhood CNS Embryonal Tumors Childhood CNS Germ Cell Tumors Childhood Cerebellar Astrocytoma Childhood Cerebral Astrocytoma Childhood Craniopharyngioma Childhood Ependymoma Childhood Ependymoblastoma Childhood Malignant Glioma Childhood Medulloblastoma Childhood Medulloepithelioma Childhood Pineal Parenchymal Tumors Childhood Spinal Cord Tumors Childhood Supratentorial Primitive Neuroectodermal Tumors Childhood Visual Pathway and Hypothalamic Glioma

Treatment Option Overview
Treatment of Newly Diagnosed Childhood Brain and Spinal Cord Tumors

Childhood Central Nervous System (CNS) Atypical Teratoid/Rhabdoid Tumor Childhood Brain Stem Glioma         Current Clinical Trials Childhood CNS Embryonal Tumors         Current Clinical Trials Childhood CNS Germ Cell Tumors         Current Clinical Trials Childhood Cerebellar Astrocytoma         Current Clinical Trials Childhood Cerebral Astrocytoma         Current Clinical Trials Childhood Craniopharyngioma         Current Clinical Trials Childhood Ependymoma         Current Clinical Trials Childhood Ependymoblastoma         Current Clinical Trials Childhood Malignant Glioma         Current Clinical Trials Childhood Medulloblastoma         Current Clinical Trials Childhood Medulloepithelioma         Current Clinical Trials Childhood Pineal Parenchymal Tumors         Current Clinical Trials Childhood Spinal Cord Tumors         Current Clinical Trials Childhood Supratentorial Primitive Neuroectodermal Tumors         Current Clinical Trials Childhood Visual Pathway and Hypothalamic Glioma         Current Clinical Trials

Treatment of Recurrent Childhood Brain and Spinal Cord Tumors

Childhood Central Nervous System (CNS) Atypical Teratoid/Rhabdoid Tumor Childhood Brain Stem Glioma         Current Clinical Trials CNS Tumors in Children Aged 3 Years and Younger Childhood CNS Embryonal Tumors Childhood CNS Germ Cell Tumors         Current Clinical Trials Childhood Cerebellar Astrocytoma         Current Clinical Trials Childhood Cerebral Astrocytoma/Malignant Glioma Childhood Craniopharyngioma Childhood Ependymoma         Current Clinical Trials Childhood Ependymoblastoma Childhood Low-Grade Glial Tumors Childhood Medulloblastoma         Current Clinical Trials Childhood Medulloepithelioma         Current Clinical Trials Childhood Pineal Parenchymal Tumors         Current Clinical Trials Childhood Spinal Cord Tumors         Current Clinical Trials Childhood Supratentorial Primitive Neuroectodermal Tumors         Current Clinical Trials Childhood Visual Pathway and Hypothalamic Glioma         Current Clinical Trials

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Changes to This Summary (01/02/2009)
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Purpose of This PDQ Summary

This 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:

• Classification of childhood brain and spinal cord tumors.
• General approach to care for childhood brain tumor patients.
• Stage information about select childhood brain and spinal cord tumors, and links to the other PDQ childhood brain and spinal cord tumor summaries.
• Treatment options about select childhood brain and spinal cord tumors, and links to the other PDQ childhood brain and spinal cord tumor summaries.

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: 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.]

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General Information

Note: Separate PDQ summaries on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor 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 Craniopharyngioma Treatment, and Childhood Visual Pathway and Hypothalamic Glioma 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.

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Classification of Brain Tumors

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:

1. Cerebellar astrocytomas (usually pilocytic but also fibrillary and, less frequently, high-grade).
2. Medulloblastomas (primitive neuroectodermal tumors [PNETs]).
3. Ependymomas (cellular, papillary, clear cell, tanycytic, or anaplastic).
4. Brain stem gliomas are typically diffuse intrinsic pontine gliomas or diffuse intrinsic high-grade tumors that are diagnosed neuroradiographically without biopsy. Focal, tectal, and exophytic cervicomedullary tumors are generally low-grade tumors.
5. Atypical teratoid/rhabdoid tumors.

Supratentorial tumors include:

1. Low-grade cerebral hemispheric astrocytomas (grade 1 [pilocytic] or grade 2).
2. High-grade or malignant astrocytomas (anaplastic astrocytomas, glioblastomas multiforme [grade 3 or grade 4]).
3. Mixed gliomas (low-grade or high-grade).
4. Oligodendrogliomas (low-grade or high-grade).
5. PNETs (including cerebral neuroblastomas, pineoblastomas, and ependymoblastomas).
6. Atypical teratoid/rhabdoid tumors.
7. Ependymomas (cellular or anaplastic).
8. Meningiomas.
9. Choroid plexus tumors (papillomas and carcinomas).
10. Pineal parenchymal tumors (pineocytomas, mixed pineal parenchymal tumors, and pineal parenchymal tumors of intermediate differentiation).
11. Neuronal and mixed neuronal glial tumors (gangliogliomas, desmoplastic infantile gangliogliomas, and dysembryoplastic neuroepithelial tumors).
12. Metastasis (rare) from extraneural malignancies.

Sellar or suprasellar tumors include:

1. Craniopharyngiomas.
2. Diencephalic astrocytomas (central tumors involving the chiasm, hypothalamus, and/or thalamus) that are generally low-grade (including astrocytomas, grade 1 [pilocytic] or grade 2).
3. Germ cell tumors (germinomas or nongerminomatous).

References

1. Kleihues P, Cavenee WK, eds.: Pathology and Genetics of Tumours of the Nervous System. Lyon, France: International Agency for Research on Cancer, 2000. 
   
   

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Classification of Spinal Cord Tumors

Primary central nervous system spinal cord tumors comprise approximately 1% to 2% of all childhood nervous system tumors.[1-3] 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.[1-3]

References

1. Constantini S, Miller DC, Allen JC, et al.: Radical excision of intramedullary spinal cord tumors: surgical morbidity and long-term follow-up evaluation in 164 children and young adults. J Neurosurg 93 (2 Suppl): 183-93, 2000.  [PUBMED Abstract]
   
   
2. Bouffet E, Pierre-Kahn A, Marchal JC, et al.: Prognostic factors in pediatric spinal cord astrocytoma. Cancer 83 (11): 2391-9, 1998.  [PUBMED Abstract]
   
   
3. Hardison HH, Packer RJ, Rorke LB, et al.: Outcome of children with primary intramedullary spinal cord tumors. Childs Nerv Syst 3 (2): 89-92, 1987.  [PUBMED Abstract]
   
   

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General Approach to Care for Children with Brain and Spinal Cord Tumors

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:

1. The cause of most childhood brain tumors remains unknown.[1-3]



2. Selection of an appropriate therapy can only occur if the correct diagnosis is made and the stage of the disease is accurately determined.



3. Children with primary brain or spinal cord tumors represent a major therapy challenge that, for optimal results, requires the coordinated efforts of pediatric specialists in fields such as neurosurgery, neuropathology, radiation oncology, pediatric oncology, neuro-oncology, neurology, rehabilitation, neuroradiology, endocrinology, and psychology, who have special expertise in the care of patients with these diseases.[4-6] For example, radiation therapy of pediatric brain tumors is very technically demanding and should be carried out in centers that have experience in this area.



4. For most childhood brain and spinal cord tumors, the optimal treatment regimen has not been determined. Children who have brain and spinal cord tumors should be considered for enrollment in a clinical trial when an appropriate study is available. Such clinical trials are being carried out by institutions and cooperative groups. 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. Information about ongoing clinical trials is available from the NCI Web site.



5. While more than 50% of children diagnosed with brain tumors will survive more than 5 years from diagnosis, survival rates are wide-ranging depending on tumor type and stage. Long-term sequelae related to the initial presence of the tumor and subsequent treatment are common.[7-9] For more information about possible long-term or late effects, refer to the PDQ summary on Late Effects of Treatment for Childhood Cancer.



6. Guidelines for pediatric cancer centers and their role in the treatment of pediatric patients with cancer have been outlined by the American Academy of Pediatrics.[10]

References

1. Kuijten RR, Bunin GR: Risk factors for childhood brain tumors. Cancer Epidemiol Biomarkers Prev 2 (3): 277-88, 1993 May-Jun.  [PUBMED Abstract]
   
   
2. Kuijten RR, Strom SS, Rorke LB, et al.: Family history of cancer and seizures in young children with brain tumors: a report from the Childrens Cancer Group (United States and Canada). Cancer Causes Control 4 (5): 455-64, 1993.  [PUBMED Abstract]
   
   
3. Fisher JL, Schwartzbaum JA, Wrensch M, et al.: Epidemiology of brain tumors. Neurol Clin 25 (4): 867-90, vii, 2007.  [PUBMED Abstract]
   
   
4. Strother DR, Poplack IF, Fisher PG, et al.: Tumors of the central nervous system. In: Pizzo PA, Poplack DG, eds.: Principles and Practice of Pediatric Oncology. 4th ed. Philadelphia, Pa: Lippincott, Williams and Wilkins, 2002, pp 751-824. 
   
   
5. Pollack IF: Brain tumors in children. N Engl J Med 331 (22): 1500-7, 1994.  [PUBMED Abstract]
   
   
6. Cohen ME, Duffner PK, eds.: Brain Tumors in Children: Principles of Diagnosis and Treatment. 2nd ed. New York: Raven Press, 1994. 
   
   
7. Ris MD, Packer R, Goldwein J, et al.: Intellectual outcome after reduced-dose radiation therapy plus adjuvant chemotherapy for medulloblastoma: a Children's Cancer Group study. J Clin Oncol 19 (15): 3470-6, 2001.  [PUBMED Abstract]
   
   
8. Johnson DL, McCabe MA, Nicholson HS, et al.: Quality of long-term survival in young children with medulloblastoma. J Neurosurg 80 (6): 1004-10, 1994.  [PUBMED Abstract]
   
   
9. Packer RJ, Sutton LN, Goldwein JW, et al.: Improved survival with the use of adjuvant chemotherapy in the treatment of medulloblastoma. J Neurosurg 74 (3): 433-40, 1991.  [PUBMED Abstract]
   
   
10. Guidelines for the pediatric cancer center and role of such centers in diagnosis and treatment. American Academy of Pediatrics Section Statement Section on Hematology/Oncology. Pediatrics 99 (1): 139-41, 1997.  [PUBMED Abstract]
    
    

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Stage Information

Childhood Central Nervous System (CNS) Atypical Teratoid/Rhabdoid Tumor

Refer to the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor for more information.

Childhood brain stem gliomas include:

• Diffuse intrinsic pontine gliomas.
• Focal or low grade brain stem gliomas.

Refer to the PDQ summary on Childhood Brain Stem Glioma Treatment for more information.

Childhood CNS embryonal tumors include:

• CNS atypical teratoid/rhabdoid tumors. (Refer to the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor for more information.)
• Ependymoblastomas.
• Medulloblastomas.
• Medulloepitheliomas.
• Pineal parenchymal tumors of intermediate differentiation.
• Pineoblastomas.
• Supratentorial primitive neuroectodermal tumors.

Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.

Childhood CNS germ cell tumors include:

• Germinomas.
• Embryonal yolk sac tumors.
• Choriocarcinomas.
• Immature teratomas.
• Mature teratomas.
• Teratomas with malignant transformation.
• Mixed germ cell tumors.
• Nongerminomatous germ cell tumors.

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 Cerebellar Astrocytoma Treatment for more information.

Refer to the PDQ summary on Childhood Cerebral Astrocytoma/Malignant Glioma Treatment for more information.

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 Cerebral Astrocytoma/Malignant Glioma 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:

• Pineoblastomas.
• Pineocytomas.
• Pineal parenchymal tumors of intermediate differentiation.

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:

• Primitive neuroectodermal tumors.
• Cerebral neuroblastomas.

Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.

Refer to the PDQ summary on Childhood Visual Pathway and Hypothalamic Glioma Treatment for more information.

References

1. Matsutani M, Sano K, Takakura K, et al.: Primary intracranial germ cell tumors: a clinical analysis of 153 histologically verified cases. J Neurosurg 86 (3): 446-55, 1997.  [PUBMED Abstract]
   
   
2. Balmaceda C, Modak S, Finlay J: Central nervous system germ cell tumors. Semin Oncol 25 (2): 243-50, 1998.  [PUBMED Abstract]
   
   
3. Constantini S, Miller DC, Allen JC, et al.: Radical excision of intramedullary spinal cord tumors: surgical morbidity and long-term follow-up evaluation in 164 children and young adults. J Neurosurg 93 (2 Suppl): 183-93, 2000.  [PUBMED Abstract]
   
   
4. Bouffet E, Pierre-Kahn A, Marchal JC, et al.: Prognostic factors in pediatric spinal cord astrocytoma. Cancer 83 (11): 2391-9, 1998.  [PUBMED Abstract]
   
   

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Treatment Option Overview

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.[1-3] 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.[5-7] Long-term management of these patients is complex and requires a multidisciplinary approach.

References

1. Ris MD, Packer R, Goldwein J, et al.: Intellectual outcome after reduced-dose radiation therapy plus adjuvant chemotherapy for medulloblastoma: a Children's Cancer Group study. J Clin Oncol 19 (15): 3470-6, 2001.  [PUBMED Abstract]
   
   
2. Johnson DL, McCabe MA, Nicholson HS, et al.: Quality of long-term survival in young children with medulloblastoma. J Neurosurg 80 (6): 1004-10, 1994.  [PUBMED Abstract]
   
   
3. Packer RJ, Sutton LN, Goldwein JW, et al.: Improved survival with the use of adjuvant chemotherapy in the treatment of medulloblastoma. J Neurosurg 74 (3): 433-40, 1991.  [PUBMED Abstract]
   
   
4. Jenkin D: Long-term survival of children with brain tumors. Oncology (Huntingt) 10 (5): 715-9; discussion 720, 722, 728, 1996.  [PUBMED Abstract]
   
   
5. Duffner PK, Horowitz ME, Krischer JP, et al.: Postoperative chemotherapy and delayed radiation in children less than three years of age with malignant brain tumors. N Engl J Med 328 (24): 1725-31, 1993.  [PUBMED Abstract]
   
   
6. Packer RJ, Lange B, Ater J, et al.: Carboplatin and vincristine for recurrent and newly diagnosed low-grade gliomas of childhood. J Clin Oncol 11 (5): 850-6, 1993.  [PUBMED Abstract]
   
   
7. Mason WP, Grovas A, Halpern S, et al.: Intensive chemotherapy and bone marrow rescue for young children with newly diagnosed malignant brain tumors. J Clin Oncol 16 (1): 210-21, 1998.  [PUBMED Abstract]
   
   

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Treatment of Newly Diagnosed Childhood Brain and Spinal Cord Tumors

Childhood Central Nervous System (CNS) Atypical Teratoid/Rhabdoid Tumor

Refer to the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor for more information.

Childhood brain stem gliomas include:

• Diffuse intrinsic pontine gliomas.
• Focal or low-grade brain stem gliomas.

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:

• CNS atypical teratoid/rhabdoid tumors. (Refer to the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor for more information.)
• Ependymoblastomas.
• Medulloblastomas.
• Medulloepitheliomas.
• Pineal parenchymal tumors of intermediate differentiation.
• Pineoblastomas.
• Supratentorial primitive neuroectodermal tumors.

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:

• Germinomas.
• Embryonal yolk sac tumors.
• Choriocarcinomas.
• Immature teratomas.
• Mature teratomas.
• Teratomas with malignant transformation.
• Mixed germ cell tumors.
• Nongerminomatous germ cell tumors.

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.[4-6] 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 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 Cerebellar Astrocytoma 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 cerebellar astrocytoma, childhood high-grade cerebellar astrocytoma, childhood low-grade cerebellar astrocytoma and untreated childhood cerebellar 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.

Refer to the PDQ summary on Childhood Cerebral Astrocytoma/Malignant 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 low-grade cerebral astrocytoma and 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.

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 Cerebral Astrocytoma/Malignant 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 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:

• Pineoblastomas.
• Pineocytomas.
• Pineal parenchmal tumors of intermediate differentiation.

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.[8-10] 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:

• Primitive neuroectodermal tumors.
• Cerebral Neuroblastomas.

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.

Refer to the PDQ summary on Childhood Visual Pathway and Hypothalamic 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 visual pathway and hypothalamic 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.

References

1. Matsutani M, Sano K, Takakura K, et al.: Primary intracranial germ cell tumors: a clinical analysis of 153 histologically verified cases. J Neurosurg 86 (3): 446-55, 1997.  [PUBMED Abstract]
   
   
2. Dearnaley DP, A'Hern RP, Whittaker S, et al.: Pineal and CNS germ cell tumors: Royal Marsden Hospital experience 1962-1987. Int J Radiat Oncol Biol Phys 18 (4): 773-81, 1990.  [PUBMED Abstract]
   
   
3. Linstadt D, Wara WM, Edwards MS, et al.: Radiotherapy of primary intracranial germinomas: the case against routine craniospinal irradiation. Int J Radiat Oncol Biol Phys 15 (2): 291-7, 1988.  [PUBMED Abstract]
   
   
4. Balmaceda C, Heller G, Rosenblum M, et al.: Chemotherapy without irradiation--a novel approach for newly diagnosed CNS germ cell tumors: results of an international cooperative trial. The First International Central Nervous System Germ Cell Tumor Study. J Clin Oncol 14 (11): 2908-15, 1996.  [PUBMED Abstract]
   
   
5. Bouffet E, Baranzelli MC, Patte C, et al.: Combined treatment modality for intracranial germinomas: results of a multicentre SFOP experience. Société Française d'Oncologie Pédiatrique. Br J Cancer 79 (7-8): 1199-204, 1999.  [PUBMED Abstract]
   
   
6. Robertson PL, DaRosso RC, Allen JC: Improved prognosis of intracranial non-germinoma germ cell tumors with multimodality therapy. J Neurooncol 32 (1): 71-80, 1997.  [PUBMED Abstract]
   
   
7. Constantini S, Miller DC, Allen JC, et al.: Radical excision of intramedullary spinal cord tumors: surgical morbidity and long-term follow-up evaluation in 164 children and young adults. J Neurosurg 93 (2 Suppl): 183-93, 2000.  [PUBMED Abstract]
   
   
8. Bouffet E, Pierre-Kahn A, Marchal JC, et al.: Prognostic factors in pediatric spinal cord astrocytoma. Cancer 83 (11): 2391-9, 1998.  [PUBMED Abstract]
   
   
9. Hardison HH, Packer RJ, Rorke LB, et al.: Outcome of children with primary intramedullary spinal cord tumors. Childs Nerv Syst 3 (2): 89-92, 1987.  [PUBMED Abstract]
   
   
10. O'Sullivan C, Jenkin RD, Doherty MA, et al.: Spinal cord tumors in children: long-term results of combined surgical and radiation treatment. J Neurosurg 81 (4): 507-12, 1994.  [PUBMED Abstract]
    
    
11. Hassall TE, Mitchell AE, Ashley DM: Carboplatin chemotherapy for progressive intramedullary spinal cord low-grade gliomas in children: three case studies and a review of the literature. Neuro-oncol 3 (4): 251-7, 2001.  [PUBMED Abstract]
    
    

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Treatment of Recurrent Childhood Brain and Spinal Cord Tumors

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.

Refer to the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor for more information.

Childhood brain stem gliomas include:

• Diffuse intrinsic pontine gliomas.
• Focal or low grade brain stem gliomas.

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.[2-7] 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:

• CNS atypical teratoid/rhabdoid tumors. (Refer to the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor for more information.)
• Ependymoblastomas.
• Medulloblastomas.
• Medulloepitheliomas.
• Pineal parenchymal tumors of intermediate differentiation.
• Pineoblastomas.
• Supratentorial primitive neuroectodermal tumors.

Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors for more information.

Childhood CNS germ cell tumors include:

• Germinomas.
• Embryonal yolk sac tumors.
• Choriocarcinomas.
• Immature teratomas.
• Mature teratomas.
• Teratomas with malignant transformation.
• Mixed germ cell tumors.
• Nongerminomatous germ cell tumors.

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 Cerebellar Astrocytoma 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 cerebellar 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.

Refer to the PDQ summary on Childhood Cerebral Astrocytoma/Malignant Glioma Treatment for more information.

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:

• Pineoblastomas.
• Pineocytomas.
• Pineal parenchmal tumors of intermediate differentiation.

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, Childhood Medulloblastoma 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:

• Primitive neuroectodermal tumors.
• Cerebral neuroblastomas.

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.

Refer to the PDQ summary on Childhood Visual Pathway and Hypothalamic 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 visual pathway and hypothalamic 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.

References

1. Jenkin D, Greenberg M, Hoffman H, et al.: Brain tumors in children: long-term survival after radiation treatment. Int J Radiat Oncol Biol Phys 31 (3): 445-51, 1995.  [PUBMED Abstract]
   
   
2. Fisher PG, Needle MN, Cnaan A, et al.: Salvage therapy after postoperative chemotherapy for primary brain tumors in infants and very young children. Cancer 83 (3): 566-74, 1998.  [PUBMED Abstract]
   
   
3. Walter AW, Mulhern RK, Gajjar A, et al.: Survival and neurodevelopmental outcome of young children with medulloblastoma at St Jude Children's Research Hospital. J Clin Oncol 17 (12): 3720-8, 1999.  [PUBMED Abstract]
   
   
4. Goldwein JW, Glauser TA, Packer RJ, et al.: Recurrent intracranial ependymomas in children. Survival, patterns of failure, and prognostic factors. Cancer 66 (3): 557-63, 1990.  [PUBMED Abstract]
   
   
5. Dupuis-Girod S, Hartmann O, Benhamou E, et al.: Will high dose chemotherapy followed by autologous bone marrow transplantation supplant cranio-spinal irradiation in young children treated for medulloblastoma? J Neurooncol 27 (1): 87-98, 1996.  [PUBMED Abstract]
   
   
6. Dunkel IJ, Boyett JM, Yates A, et al.: High-dose carboplatin, thiotepa, and etoposide with autologous stem-cell rescue for patients with recurrent medulloblastoma. Children's Cancer Group. J Clin Oncol 16 (1): 222-8, 1998.  [PUBMED Abstract]
   
   
7. Guruangan S, Dunkel IJ, Goldman S, et al.: Myeloablative chemotherapy with autologous bone marrow rescue in young children with recurrent malignant brain tumors. J Clin Oncol 16 (7): 2486-93, 1998.  [PUBMED Abstract]
   
   
8. Packer RJ, Lange B, Ater J, et al.: Carboplatin and vincristine for recurrent and newly diagnosed low-grade gliomas of childhood. J Clin Oncol 11 (5): 850-6, 1993.  [PUBMED Abstract]
   
   
9. Chamberlain MC, Grafe MR: Recurrent chiasmatic-hypothalamic glioma treated with oral etoposide. J Clin Oncol 13 (8): 2072-6, 1995.  [PUBMED Abstract]
   
   

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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.

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Changes to This Summary (01/02/2009)

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.

Stage Information

Added Childhood Central Nervous System (CNS) Atypical Teratoid/Rhabdoid Tumor as a new subsection.

Treatment of Newly Diagnosed Childhood Brain and Spinal Cord Tumors

Added Childhood Central Nervous System (CNS) Atypical Teratoid/Rhabdoid Tumor as a new subsection.

Treatment of Recurrent Childhood Brain and Spinal Cord Tumors

Added Childhood Central Nervous System (CNS) Atypical Teratoid/Rhabdoid Tumor as a new subsection.

Added Childhood Craniopharyngioma as a new subsection.

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More Information

About PDQ

• PDQ® - NCI's Comprehensive Cancer Database.

  Full description of the NCI PDQ database.

Additional PDQ Summaries

• PDQ® Cancer Information Summaries: Adult Treatment

  Treatment options for adult cancers.

• PDQ® Cancer Information Summaries: Pediatric Treatment

  Treatment options for childhood cancers.

• PDQ® Cancer Information Summaries: Supportive and Palliative Care

  Side effects of cancer treatment, management of cancer-related complications and pain, and psychosocial concerns.

• PDQ® Cancer Information Summaries: Screening/Detection (Testing for Cancer)

  Tests or procedures that detect specific types of cancer.

• PDQ® Cancer Information Summaries: Prevention

  Risk factors and methods to increase chances of preventing specific types of cancer.

• PDQ® Cancer Information Summaries: Genetics

  Genetics of specific cancers and inherited cancer syndromes, and ethical, legal, and social concerns.

• PDQ® Cancer Information Summaries: Complementary and Alternative Medicine

  Information about complementary and alternative forms of treatment for patients with cancer.

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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