Cancer Information

Childhood Cerebellar Astrocytoma Treatment (PDQ®)


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.

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, which is written in less-technical language, and in Spanish. 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.

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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 summary for information about the general classification of childhood brain and spinal cord tumors.

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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.

1Kleihues P, Cavenee WK, eds.: Pathology and Genetics of Tumours of the Nervous System. Lyon, France: International Agency for Research on Cancer, 2000.2Gilles 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.

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Stage Information

In general, tumors are separated into either cerebellar hemisphere or more diffuse, midline lesions.[1]

1Hayostek 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.

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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.

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Untreated Childhood Cerebellar Astrocytoma

Surgical resection is the primary treatment for childhood cerebellar astrocytoma.[1][2][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. 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.

1Campbell JW, Pollack IF: Cerebellar astrocytomas in children. J Neurooncol 28 (2-3): 223-31, 1996 May-Jun.2Schneider JH Jr, Raffel C, McComb JG: Benign cerebellar astrocytomas of childhood. Neurosurgery 30 (1): 58-62; discussion 62-3, 1992.3Due-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, 2002.4Hayostek 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, 1993.5Sutton LN, Cnaan A, Klatt L, et al.: Postoperative surveillance imaging in children with cerebellar astrocytomas. J Neurosurg 84 (5): 721-5, 1996.6Garcia 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, 1990.7Packer 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.

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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. 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.

1Austin EJ, Alvord EC Jr: Recurrences of cerebellar astrocytomas: a violation of Collins' law. J Neurosurg 68 (1): 41-7, 1988.2Garcia 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.

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Changes to This Summary (07/22/2008)

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.

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Important:

This information is intended mainly for use by doctors and other health care professionals. If you have questions about this topic, you can ask your doctor, or call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).

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This information was last updated on 2008-04-09