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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 Type Pathologic Subtype Related PDQ Treatment Summary
    CNS = central nervous system; WHO = World Health Organization.
    Astrocytomas and Other Tumors of Glial Origin
    -Low-Grade Astrocytomas Diffuse fibrillary astrocytoma Childhood Astrocytomas Treatment
    Gemistocytic astrocytoma
    Pilocytic astrocytoma
    Pilomyxoid astrocytoma
    Pleomorphic xanthoastrocytoma
    Protoplasmic astrocytoma
    Subependymal giant cell astrocytoma
    -High-Grade Astrocytomas Anaplastic astrocytoma Childhood Astrocytomas Treatment
    Anaplastic oligoastrocytoma
    Anaplastic oligodendroglioma
    Giant cell glioblastoma
    Gliomatosis cerebri
    Brain Stem Glioma
    Diffuse intrinsic pontine glioma Childhood Brain Stem Glioma Treatment
    Focal or low-grade brain stem glioma
    CNS Embryonal Tumors
    -Medulloblastomas Anaplastic Childhood CNS Embryonal Tumors Treatment
    Large cell
    Medulloblastoma with extensive nodularity
    -CNS Primitive Neuroectodermal Tumors (PNETs) CNS ganglioneuroblastoma
    CNS neuroblastoma
    -Pineal Parenchymal Tumors Pineoblastoma
    -CNS Atypical Teratoid/Rhabdoid Tumor Childhood CNS Atypical Teratoid/Rhabdoid Tumor Treatment
    CNS Germ Cell Tumors
    -Germinomas Childhood CNS Germ Cell Tumors Treatment
    -Teratomas Immature teratoma
    Mature teratoma
    Teratoma with malignant transformation
    -Non-Germinomatous Germ Cell Tumors Choriocarcinoma
    Embryonal carcinoma
    Mixed germ cell tumor
    Yolk sac tumor
    Craniopharyngioma Childhood 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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