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Brain Cancer Health Center

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Childhood Brain and Spinal Cord Tumors Treatment Overview (PDQ®): Treatment - Health Professional Information [NCI] - 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 TypePathologic SubtypeRelated 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
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
Gliomatosis cerebri
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
Large cell
Medulloblastoma with extensive nodularity
-CNS Primitive Neuroectodermal Tumors (PNETs)CNS ganglioneuroblastoma
CNS neuroblastoma
-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
CraniopharyngiomaChildhood Craniopharyngioma Treatment
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.

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