This cancer treatment information summary provides an overview of the diagnosis, classification, treatment, and prognosis of childhood supratentorial primitive neuroectodermal tumors (SPNET) of the cortex and pineal (pineoblastoma) tumors.

The National Cancer Institute provides the PDQ pediatric cancer treatment information summaries as a public service to increase the availability of evidence-based cancer information to health professionals, patients, and the public. These summaries are updated regularly according to the latest published research findings by an Editorial Board of pediatric oncology specialists.

Primary brain tumors are a diverse group of diseases that together constitute the most common solid tumor of childhood. Brain tumors are classified according to histology, but tumor location and extent of spread are important factors that affect treatment and prognosis. Immunohistochemical analysis, cytogenetic and molecular genetic findings, and measures of mitotic activity are increasingly used in tumor diagnosis and classification.

Approximately 50% of brain tumors in children are infratentorial, with three fourths of these located in the cerebellum or fourth ventricle. Common infratentorial (posterior fossa) tumors include the following:

1. Cerebellar astrocytoma (usually pilocytic but also fibrillary and, less frequently, high-grade).
2. Medulloblastoma (primitive neuroectodermal tumor [PNET] ).
3. Ependymoma (cellular, papillary, clear cell, tanycytic or anaplastic).
4. Brain stem glioma (often diagnosed neuroradiographically without biopsy; more frequently high-grade and may be low-grade).
5. Atypical teratoid/rhabdoid tumors.

Supratentorial tumors include those tumors that occur in the sellar or suprasellar region and/or other areas of the cerebrum. Sellar/suprasellar tumors comprise approximately 20% of childhood brain tumors and include the following:

1. Craniopharyngioma.
2. Diencephalic (central tumors involving the chiasm, hypothalamic, and/or thalamic) gliomas generally of low grade (including astrocytoma, grade 1 or 2).
3. Germ cell tumors (germinoma and nongerminomatous).

Other tumors that occur supratentorially include the following:

1. Low-grade cerebral hemispheric astrocytoma or glioma (grade 1 or grade 2).
2. High-grade or malignant astrocytoma (anaplastic astrocytoma, glioblastoma multiforme [grade 3 or grade 4]).
3. Mixed glioma (low-grade or high-grade).
4. Oligodendroglioma (low-grade or high-grade).
5. Primitive neuroectodermal tumor (including cerebral neuroblastoma, pineoblastoma, ependymoblastoma).
6. Ependymoma (cellular or anaplastic).
7. Meningioma.
8. Choroid plexus tumors (papilloma and carcinoma).
9. Pineal parenchymal tumors (pineocytoma, or mixed pineal parenchymal tumor).
10. Neuronal and mixed neuronal glial tumor (ganglioglioma, desmoplastic infantile ganglioglioma, dysembryoplastic neuroepithelial tumor).
11. Metastasis (rare) from extra-neural malignancies.

Important general concepts that should be understood by those caring for a child with a brain tumor include the following:

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



2. Children with primary brain tumors represent a major therapy challenge that, for optimal results, requires the coordinated efforts of pediatric specialists in fields such as neurosurgery, neurology, rehabilitation, neuropathology, radiation oncology, pediatric oncology, neuro-oncology, neuroradiology, endocrinology, and psychology, who have special expertise in the care of patients with these diseases.[1-3]



3. More than one half of children diagnosed with brain tumors will survive 5 years from diagnosis. In some subgroups of patients, an even higher rate of survival and cure is possible. Each child’s treatment should be approached with curative intent, and the possible long-term sequelae of the disease and its treatment should be considered before therapy is begun.



4. For most childhood brain tumors, the optimal treatment regimen has not been determined. Children who have brain tumors should be considered for enrollment in clinical trials when an appropriate study is available. Such clinical trials are being carried out by institutions and cooperative groups.



5. 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.[4]



6. Most childhood brain tumors remains unknown.[5,6]

This summary discusses the treatment of childhood supratentorial primitive neuroectodermal and pineal (pineoblastoma) tumors.

Information about ongoing clinical trials is available from the NCI Cancer.gov Web site.

References

1. 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. 
   
   
2. Pollack IF: Brain tumors in children. N Engl J Med 331 (22): 1500-7, 1994.  [PUBMED Abstract]
   
   
3. Cohen ME, Duffner PK, eds.: Brain Tumors in Children: Principles of Diagnosis and Treatment. 2nd ed. New York: Raven Press, 1994. 
   
   
4. 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]
   
   
5. Kuijten RR, Bunin GR: Risk factors for childhood brain tumors. Cancer Epidemiol Biomarkers Prev 2 (3): 277-88, 1993 May-Jun.  [PUBMED Abstract]
   
   
6. 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]
   
   

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