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Childhood Brain and Spinal Cord Tumors Treatment Overview (PDQ®)
Patient VersionHealth Professional VersionEn españolLast Modified: 08/26/2008



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 (08/26/2008)






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Stage Information

Childhood Brain Stem Glioma
Childhood Central Nervous System (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



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.

Childhood Central Nervous System (CNS) Embryonal Tumors

Childhood CNS embryonal tumors include:

  • Atypical teratoid/rhabdoid tumors.
  • 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

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]

Childhood Cerebellar Astrocytoma

Refer to the PDQ summary on Childhood Cerebellar Astrocytoma Treatment for more information.

Childhood Cerebral Astrocytoma

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

Childhood Craniopharyngioma

These are symptomatic benign tumors arising from remnants of Rathke’s pouch. There is no generally accepted staging system and metastasis is rare.[3-6]

Childhood Ependymoma

Refer to the PDQ summary on Childhood Ependymoma Treatment for more information.

Childhood Ependymoblastoma

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

Childhood Malignant Glioma

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

Childhood Medulloblastoma

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

Childhood Medulloepithelioma

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

Childhood Pineal Parenchymal Tumors

Childhood pineal parenchymal tumors include:

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

Refer to the PDQ summaries on Childhood Supratentorial Primitive Neuroectodermal Tumors and Pineoblastoma Treatment and Childhood Central Nervous System Embryonal Tumors Treatment for more information.

Childhood Spinal Cord Tumors

There is no uniformly accepted staging system for childhood primary spinal cord tumors. These tumors are classified based on their location within the spinal cord and histology. Low-grade spinal cord tumors rarely disseminate elsewhere in the nervous system; however, higher grade tumors may disseminate.[7,8] Despite this, because of the location of the tumor and concerns over causing further neurologic deterioration by CSF attainment, routine lumbar spinal punctures are not indicated in the evaluation of a child with a spinal cord tumor. For high-grade glial spinal cord tumors, and possibly lower grade tumors and ependymomas, (refer to the PDQ summary on Childhood Ependymoma Treatment for more information) neuroimaging of the entire neuroaxis (brain and entire spine) is indicated at the time of diagnosis for determination of extent of disease.

Childhood Supratentorial Primitive Neuroectodermal Tumors

Childhood supratentorial primitive neuroectodermal tumors include:

  • Primitive neuroectodermal tumors.
  • Cerebral neuroblastomas.

Refer to the PDQ summaries on Childhood Supratentorial Primitive Neuroectodermal Tumors and Pineoblastoma Treatment and Childhood Central Nervous System Embryonal Tumors Treatment for more information.

Childhood Visual Pathway and Hypothalamic Glioma

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

References

  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. Lapras C, Patet JD, Mottolese C, et al.: Craniopharyngiomas in childhood: analysis of 42 cases. Prog Exp Tumor Res 30: 350-8, 1987.  [PUBMED Abstract]

  4. Habrand JL, Ganry O, Couanet D, et al.: The role of radiation therapy in the management of craniopharyngioma: a 25-year experience and review of the literature. Int J Radiat Oncol Biol Phys 44 (2): 255-63, 1999.  [PUBMED Abstract]

  5. Yaşargil MG, Curcic M, Kis M, et al.: Total removal of craniopharyngiomas. Approaches and long-term results in 144 patients. J Neurosurg 73 (1): 3-11, 1990.  [PUBMED Abstract]

  6. Long-term outcomes for surgically resected craniopharyngiomas. Neurosurgery 46 (2): 291-302; discussion 302-5, 2000.  [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]

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