Radiation Therapy or Combination Chemotherapy in Treating Patients With Clinically or Radiologically Progressive Low-Grade Gliomas (SIOP-LGG-2004)

DISEASE CHARACTERISTICS:

• Histologically confirmed low-grade glioma, of 1 of the following histologic subtypes:

  • Pilocytic astrocytoma I° and variants
  • Subependymal giant cell astrocytoma I°
  • Dysembryoplastic neuroepithelial tumor I°
  • Desmoplastic infantile ganglioglioma I°
  • Ganglioglioma I° and II°
  • Pleomorphic xanthoastrocytoma II°
  • Oligodendroglioma II°
  • Oligoastrocytoma II°

  • Astrocytoma II°

    • Fibrillary astrocytoma II°
    • Protoplasmatic astrocytoma II°
    • Gemistocytic astrocytoma II°
• Children with chiasmatic-hypothalamic tumors may be eligible without histological diagnosis, if neuroradiologic findings meet unequivocal criteria for the presence of a low-grade glioma

• Primary tumor localization: intracranial and/or spinal cord

  • No diffuse intrinsic tumors of the pons, even if histologically an astrocytoma I° or II° is diagnosed

    • Exception: pontine glioma II° in neurofibromatosis type 1 (NF1) disease allowed
• Children presenting with disseminated low-grade glioma will be eligible for the study
• All eligible patients without NF1 disease receiving chemotherapy as their first nonsurgical therapy are eligible for randomization
• Children are eligible for the trial regardless of the presence of associated genetic disease: NF1 disease will be the prominent one, all children with NF1 disease are entered into the study arm III in case of an indication for nonsurgical therapy

• Patients presenting with rare intracranial neoplasms of low-grade malignancy, but nonglial origin, may be followed according to the low-grade glioma strategy, but they are not subject of this therapy trial.

  • Data from these patients may be registered to learn about those therapeutic interventions which may prove useful to these patients and to develop separate strategies in the future
  • Patients with choroid plexus papilloma should be entered into the SIOP-CPT study (Prof. Dr. J. Wolff, M.D. Anderson Cancer Center, Houston, Texas)

PATIENT CHARACTERISTICS:

• No preexisting impairments of health status, making the conduct of the study impossible or ethically unwise
• No evidence of pregnancy or lactation period
• Pregnancy has to be prevented in fertile adolescent girls during chemotherapy by reliable contraception methods (e.g., hormonal contraception)

PRIOR CONCURRENT THERAPY:

• The tumor should not be pretreated with chemotherapy or radiotherapy

  • Children treated with chemotherapy or radiotherapy prior to entering the study will be evaluated separately

    • Previous treatment with steroids is not considered a chemotherapeutic treatment

• Surgery of any type and extent allowed

  • Prior surgical resection of the tumor allowed
• Participation in another clinical study concurrently with this study (e.g., endocrinologic study) allowed provided it is not interfering with the present treatment strategy
• No other concurrent chemotherapy
• Concurrent medication for associated or other conditions (e.g., hormone replacement, anticonvulsants) that does not containing cytostatic drugs allowed