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

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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