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Table of Contents Purpose of This PDQ Summary
General Information
Cellular Classification
Stage Information
Treatment Option Overview
Untreated Childhood Cerebellar Astrocytoma
Recurrent Childhood Cerebellar Astrocytoma
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Changes to This Summary (07/22/2008)
More Information
Purpose of This PDQ Summary
This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of childhood cerebellar astrocytoma. This summary is reviewed regularly and updated as necessary by the PDQ Pediatric Treatment Editorial Board 1.
Information about the following is included in this summary:
- Cellular classification.
- Stage information.
- Treatment options.
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 the 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 2, which is written in less-technical language, and in Spanish 3. [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.] General Information
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.
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.
Refer to the PDQ Childhood Brain and Spinal Cord Tumors Treatment Overview 4 summary for information about the general classification of childhood brain and spinal cord tumors. Cellular Classification
The classification of brain tumors is based on both histopathologic
characteristics and location in the brain. More than 80% of all childhood
cerebellar gliomas will be pilocytic astrocytomas, which are also considered to
be grade 1 astrocytomas. Most of the remainder will be diffuse or
fibrillary astrocytomas. Malignant gliomas are rare.[1] The pathologic
classification of pediatric brain tumors is a specialized area that is
undergoing evolution; review of the diagnostic tissue by a neuropathologist who
has particular expertise in this area is strongly recommended.
These generally low-grade, often cystic astrocytic tumors are localized to the
cerebellum. Except for malignant gliomas, contiguous spread or metastasis
outside that region is extremely rare. The presence of certain histologic
features has been used retrospectively to stratify cerebellar astrocytomas into
two distinct groups: pilocytic or Gilles type A tumors and diffuse or Gilles
type B tumors; the latter tumors have a poor prognosis.[2] Expert neuropathologic
review is important.
References
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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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Gilles FH, Sobel EL, Tavaré CJ, et al.: Age-related changes in diagnoses, histological features, and survival in children with brain tumors: 1930-1979. The Childhood Brain Tumor Consortium. Neurosurgery 37 (6): 1056-68, 1995.
[PUBMED Abstract]
Stage Information
In general, tumors are separated into either cerebellar hemisphere or more diffuse, midline lesions.[1]
References
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Hayostek CJ, Shaw EG, Scheithauer B, et al.: Astrocytomas of the cerebellum. A comparative clinicopathologic study of pilocytic and diffuse astrocytomas. Cancer 72 (3): 856-69, 1993.
[PUBMED Abstract]
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 available,
accepted therapy. 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 that were previously
obtained with existing therapy.
Because of the relative rarity of cancer in children, all patients with brain
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 technically very
demanding and should be carried out in centers that have experience in that
area in order to ensure optimal results.
The designations in PDQ that treatments are “standard” or “under clinical
evaluation” are not to be used as a basis for reimbursement determinations.
Untreated Childhood Cerebellar Astrocytoma
Surgical resection is the primary treatment for childhood cerebellar
astrocytoma.[1-3] Complete or near complete removal can be obtained in 90% to
95% of patients with juvenile pilocytic tumors. Diffuse cerebellar
astrocytomas may be less amenable to total resection, and this may account for
the poorer outcome. The extent of resection necessary for cure is unknown
because patients with microscopic and even gross residual tumor after surgery
may experience long-term progression-free survival without postoperative
therapy.[3,4] Following resection, a postoperative MRI is obtained. Surveillance
scans are then obtained periodically for totally resected tumors,
although the value of this is uncertain.[5] The optimal use of radiation
therapy is the subject of controversy. Treatment is often withheld in patients with residual disease until tumor progression has been documented.[6] Chemotherapy may be useful for
delaying radiation therapy in the very young child with unresectable,
progressive cerebellar astrocytoma.[7]
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 cerebellar astrocytoma 5. 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 6.
References
-
Campbell JW, Pollack IF: Cerebellar astrocytomas in children. J Neurooncol 28 (2-3): 223-31, 1996 May-Jun.
[PUBMED Abstract]
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Schneider JH Jr, Raffel C, McComb JG: Benign cerebellar astrocytomas of childhood. Neurosurgery 30 (1): 58-62; discussion 62-3, 1992.
[PUBMED Abstract]
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Due-Tønnessen BJ, Helseth E, Scheie D, et al.: Long-term outcome after resection of benign cerebellar astrocytomas in children and young adults (0-19 years): report of 110 consecutive cases. Pediatr Neurosurg 37 (2): 71-80, 2002.
[PUBMED Abstract]
-
Hayostek CJ, Shaw EG, Scheithauer B, et al.: Astrocytomas of the cerebellum. A comparative clinicopathologic study of pilocytic and diffuse astrocytomas. Cancer 72 (3): 856-69, 1993.
[PUBMED Abstract]
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Sutton LN, Cnaan A, Klatt L, et al.: Postoperative surveillance imaging in children with cerebellar astrocytomas. J Neurosurg 84 (5): 721-5, 1996.
[PUBMED Abstract]
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Garcia DM, Marks JE, Latifi HR, et al.: Childhood cerebellar astrocytomas: is there a role for postoperative irradiation? Int J Radiat Oncol Biol Phys 18 (4): 815-8, 1990.
[PUBMED Abstract]
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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]
Recurrent Childhood Cerebellar Astrocytoma
Recurrence may take place in childhood cerebellar gliomas and may develop many
years after initial treatment. Disease can be at the primary tumor site or,
especially in malignant tumors, at noncontiguous central nervous system sites.
Systemic relapse is rare, but may occur. At the time of recurrence, a complete
evaluation to determine the extent of relapse is indicated for all patients.
Biopsy or surgical resection may be necessary for confirmation of relapse
because 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 on the basis of the initial
tumor type, the length of time between initial treatment and the reappearance
of the mass lesion, and the clinical picture.
Patients with cerebellar astrocytoma (pilocytic or diffuse) who relapse after
being treated with surgery alone should be considered for another surgical
resection.[1] If this is not feasible, local radiation therapy is the usual
treatment.[2] If there is recurrence in an unresectable site after irradiation,
chemotherapy should be considered.[2] There is little information regarding
the activity of chemotherapy in this disease. Studies of novel therapeutic
approaches that are designed to test the activity and toxicity of chemotherapy
in recurrent brain tumor patients should be considered.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent childhood cerebellar astrocytoma 7. 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 6.
References
-
Austin EJ, Alvord EC Jr: Recurrences of cerebellar astrocytomas: a violation of Collins' law. J Neurosurg 68 (1): 41-7, 1988.
[PUBMED Abstract]
-
Garcia DM, Marks JE, Latifi HR, et al.: Childhood cerebellar astrocytomas: is there a role for postoperative irradiation? Int J Radiat Oncol Biol Phys 18 (4): 815-8, 1990.
[PUBMED Abstract]
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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.
Editorial changes were made to this summary. More Information
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