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Childhood Brain and Spinal Cord Tumors Treatment Overview (PDQ®)
Patient Version   Health Professional Version   En español   Last Modified: 01/02/2009



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






Treatment Option Overview






Treatment of Newly Diagnosed Childhood Brain and Spinal Cord Tumors






Treatment of Recurrent Childhood Brain and Spinal Cord Tumors






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Changes to This Summary (01/02/2009)






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Past Highlights
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



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 Glioma

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

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 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

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.

Current Clinical Trials

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

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.

Current Clinical Trials

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.

Childhood Cerebellar Astrocytoma

Refer to the PDQ summary on Childhood Cerebellar Astrocytoma 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 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.

Childhood Cerebral Astrocytoma

Refer to the PDQ summary on Childhood Cerebral Astrocytoma/Malignant 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 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.

Childhood Craniopharyngioma

Refer to the PDQ summary on Childhood Craniopharyngioma 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 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.

Childhood Ependymoma

Refer to the PDQ summary on Childhood Ependymoma 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 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.

Childhood Ependymoblastoma

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

Childhood Malignant Glioma

Refer to the PDQ summary on Childhood Cerebral Astrocytoma/Malignant 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 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.

Childhood Medulloblastoma

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

Childhood Medulloepithelioma

Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors 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 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

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.

Current Clinical Trials

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.

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.[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.

Current Clinical Trials

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

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.

Current Clinical Trials

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.

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 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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