Stage Information and Treatment of Newly Diagnosed and Recurrent Childhood Brain Tumors
There is no uniformly accepted staging system for childhood brain tumors. These tumors are classified and treated based on their histology and location within the brain (Table 1).
Table 1. The Staging and Treatment of Newly Diagnosed or Recurrent Tumors According to Type of Tumor or Pathologic Subtype
|Tumor Type||Pathologic Subtype||Staging and Treatment of Newly Diagnosed and Recurrent Disease|
|CNS = central nervous system.|
|Astrocytomas and Other Tumors of Glial Origin|| || |
|–Low-Grade Astrocytomas||Diffuse fibrillary astrocytoma||Childhood Astrocytomas Treatment|
|Subependymal giant cell astrocytoma|
|–High-Grade Astrocytomas||Anaplastic astrocytoma||Childhood Astrocytomas Treatment|
|Giant cell glioblastoma|
|Brain Stem Glioma|| || |
| ||Diffuse intrinsic pontine gliomas||Childhood Brain Stem Glioma Treatment|
|Focal or low-grade brain stem gliomas|
|CNS Embryonal Tumors|| || |
|–Medulloblastoma||Anaplastic||Childhood CNS Embryonal Tumors Treatment|
|Medulloblastoma with extensive nodularity|
|–CNS Primitive Neuroectodermal Tumors (PNETs)||CNS ganglioneuroblastoma|
|–Tumors of the Pineal Region||Pineal parenchymal tumor of intermediate differentiation|
|Papillary tumor of the pineal region|
|–CNS Atypical Teratoid/Rhabdoid Tumor|| ||Childhood CNS Atypical Teratoid/Rhabdoid Tumor Treatment|
|CNS Germ Cell Tumors|| || |
|–Germinomas|| ||Childhood CNS Germ Cell Tumors Treatment|
|Teratomas with malignant transformation|
|–Non-Germinomatous Germ Cell Tumors||Choriocarcinoma|
|Mixed germ cell tumors|
|Yolk sac tumor|
|Craniopharyngioma|| ||Childhood Craniopharyngioma Treatment|
|Ependymoma|| ||Childhood Ependymoma Treatment|
|Tumors of the Choroid Plexus|| || |
Recurrence is not uncommon in both low-grade and malignant childhood brain tumors and may occur many years after initial treatment. 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.
Early-phase therapeutic trials may be available for selected patients via Children's Oncology Group phase I institutions, the Pediatric Brain Tumor Consortium, or other entities.
- 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.