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

Health Professional Version
Last Modified: 01/28/2014

Stage Information and Treatment of Newly Diagnosed and Recurrent Childhood Brain Tumors

Presently, there is no uniformly accepted staging system for most childhood brain tumors. These tumors are classified and treated based on their histology and location within the brain (see Table below). However, with advances in molecular data, it is conceivable that genomic factors will refine classification approaches for certain groups of tumors, such as medulloblastomas [1,2] and low-grade gliomas.[3,4]

Newly Diagnosed or Recurrent Tumor Type and Its Related PDQ Treatment Summary
Tumor Type Pathologic Subtype Related PDQ Treatment Summary 
CNS = central nervous system; WHO = World Health Organization.
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 gliomaChildhood Brain Stem Glioma Treatment
Focal or low-grade brain stem glioma
CNS Embryonal Tumors
MedulloblastomasAnaplasticChildhood CNS Embryonal Tumors Treatment
Classic
Desmoplastic/nodular
Large cell
Medulloblastoma with extensive nodularity
CNS Primitive Neuroectodermal Tumors (PNETs)CNS ganglioneuroblastoma
CNS neuroblastoma
Ependymoblastoma
Medulloepithelioma
Pineal Parenchymal TumorsPineoblastoma
CNS Atypical Teratoid/Rhabdoid TumorChildhood CNS Atypical Teratoid/Rhabdoid Tumor Treatment
CNS Germ Cell Tumors
GerminomasChildhood CNS Germ Cell Tumors Treatment
TeratomasImmature teratoma
Mature teratoma
Teratoma with malignant transformation
Non-Germinomatous Germ Cell TumorsChoriocarcinoma
Embryonal carcinoma
Mixed germ cell tumor
Yolk sac tumor
Craniopharyngioma Childhood Craniopharyngioma Treatment
Ependymoma
Subependymoma (WHO Grade I)Childhood Ependymoma Treatment
Myxopapillary ependymoma (WHO Grade I)
Ependymoma (WHO Grade II)
Anaplastic ependymoma (WHO Grade III)
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 determination of 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. Northcott PA, Shih DJ, Peacock J, et al.: Subgroup-specific structural variation across 1,000 medulloblastoma genomes. Nature 488 (7409): 49-56, 2012.  [PUBMED Abstract]

  2. Kool M, Korshunov A, Remke M, et al.: Molecular subgroups of medulloblastoma: an international meta-analysis of transcriptome, genetic aberrations, and clinical data of WNT, SHH, Group 3, and Group 4 medulloblastomas. Acta Neuropathol 123 (4): 473-84, 2012.  [PUBMED Abstract]

  3. Jones DT, Kocialkowski S, Liu L, et al.: Tandem duplication producing a novel oncogenic BRAF fusion gene defines the majority of pilocytic astrocytomas. Cancer Res 68 (21): 8673-7, 2008.  [PUBMED Abstract]

  4. Pfister S, Janzarik WG, Remke M, et al.: BRAF gene duplication constitutes a mechanism of MAPK pathway activation in low-grade astrocytomas. J Clin Invest 118 (5): 1739-49, 2008.  [PUBMED Abstract]