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Childhood Brain and Spinal Cord Tumors Treatment Overview (PDQ®)

Health Professional Version
Last Modified: 05/10/2012

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 AstrocytomasDiffuse fibrillary astrocytomaChildhood Astrocytomas Treatment
Gemistocytic astrocytoma
Oligoastrocytoma
Oligodendroglioma
Pilocytic astrocytoma
Pilomyxoid astrocytoma
Pleomorphic xanthoastrocytoma
Protoplasmic astrocytoma
Subependymal giant cell astrocytoma
High-Grade AstrocytomasAnaplastic astrocytomaChildhood Astrocytomas Treatment
Anaplastic oligoastrocytoma
Anaplastic oligodendroglioma
Giant cell glioblastoma
Glioblastoma
Gliomatosis cerebri
Gliosarcoma
Brain Stem Glioma
Diffuse intrinsic pontine gliomasChildhood Brain Stem Glioma Treatment
Focal or low-grade brain stem gliomas
CNS Embryonal Tumors
MedulloblastomaAnaplasticChildhood CNS Embryonal Tumors Treatment
Classic
Desmoplastic/nodular
Large cell
Medulloblastoma with extensive nodularity
CNS Primitive Neuroectodermal Tumors (PNETs)CNS ganglioneuroblastoma
CNS neuroblastoma
Ependymoblastoma
Medulloepithelioma
Tumors of the Pineal RegionPineal parenchymal tumor of intermediate differentiation
Pineoblastoma
Pineocytoma
Papillary tumor of the pineal region
CNS Atypical Teratoid/Rhabdoid TumorChildhood CNS Atypical Teratoid/Rhabdoid Tumor Treatment
CNS Germ Cell Tumors
GerminomasChildhood CNS Germ Cell Tumors Treatment
TeratomasImmature teratomas
Mature teratomas
Teratomas with malignant transformation
Non-Germinomatous Germ Cell TumorsChoriocarcinoma
Embryonal carcinoma
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.[1] 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.

References
  1. 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.  [PUBMED Abstract]