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INTRODUCTION

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Epidemiology

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Epidemiology
Incidence:

23,380 (male: 12,820; female: 10,560. Estimated new cases for 2014 in the United States)

7.7 per 100,000 men 5.4 per 100,000 women

Deaths: Estimated 14,320 in 2014 (male: 8,090; female: 6,230)
Median age at diagnosis: 57 years
Male to female ratio: Slight male predominance in the incidence of malignant brain tumors

Siegel R et al. CA Cancer J Clin 2014;64:9–29

Surveillance, Epidemiology and End Results (SEER) Program, available from http://seer.cancer.gov (accessed in 2013)

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Work-up

  1. Neuroimaging study (MRI of the brain preferred over CT)

    No other staging work-up is required except:

  2. Primary CNS lymphoma: MRI of spine, lumbar puncture, and ophthalmologic examination

  3. PNET/medulloblastoma: MRI of spine and lumbar puncture when safe to do so

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Pathology

  1. Neuroepithelial Tumors

    1. Glial tumors

      1. Astrocytic tumors (45–50%)

        • Glioblastoma multiforme (GBM grade IV): 50%

        • Anaplastic astrocytoma (AA grade III): 30%

        • Diffuse astrocytoma (grade II): 20%

        • Gliosarcoma (grade IV): <5% of GBM

        • Pilocytic astrocytoma (grade I): <1%

      2. Oligodendrogliomas/oligoastrocytomas (5–10%)

        • Oligodendroglioma (O grade II)

        • Anaplastic oligodendroglioma (AO grade III)

        • Oligoastrocytoma (OA grade II)

        • Anaplastic oligoastrocytoma (AOA grade III)

      3. Ependymal tumors (5%)

        • Myxopapillary ependymoma (grade I)

        • Ependymoma (grade II)

        • Anaplastic ependymoma (grade III)

        • Subependymoma

    2. Neuronal and mixed glial-neuronal tumors (<1%)

      • Central neurocytoma

      • Dysembryoplastic neuroepithelial tumor

      • Desmoplastic infantile astrocytoma/ganglioma

      • Ganglioma

      • Paraganglioma

    3. Nonglial tumors

      1. Embryonal tumors (<5%)

        • Primitive neuroectodermal tumors (PNET)

        • Medulloblastoma

        • Ependymoblastoma

      2. Choroid plexus tumors (<1%)

        • Choroid plexus papilloma

      3. Pineal tumors (<1%)

        • Pineoblastoma

        • Pineocytoma

  2. Meningeal Tumors (15–28%)

    • Benign meningioma (grade I): >90%

    • Atypical meningioma (grade II): 6%

    • Anaplastic meningioma (grade III): 2%

    • Hemangiopericytoma

  3. Germ Cell Tumors (<1%)

  4. Nerve Sheet Tumors (4–8%)

    • Schwannoma

  5. Primary CNS Lymphoma (PCNSL) (1–5%)

  6. Metastatic Tumors

 

Adesina AM et al. Histopathology of primary tumors of the central nervous system. In: Prados M, ed. Brain Cancer: American Cancer Society Atlas of Clinical Oncology. Hamilton, Ontario, Canada: BC Decker Inc.; 2002:16–47

Greenberg H et al. Brain Tumors. Oxford University Press, 1999:1–26

WHO Classification of Tumors. IARC Press, 2000:6–7

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Staging

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Staging

(TNM staging does not apply to most brain tumors because they rarely metastasize. Ependymal tumors such as medulloblastoma are exceptions to this rule)

WHO Designation WHO Grade St. Anne–Mayo Grade
Grade Nuclear Atypia Mitosis Endothelial Proliferation Necrosis
Pilocytic astrocytoma I Astrocytoma grade 1 (no criteria present)
Diffuse astrocytoma II + Usually − Astrocytoma grade 2 (1 criterion present)
Anaplastic astrocytoma III + + ± Astrocytoma grade 3 (2 criteria present)
Glioblastoma IV + +, Active Usually + Usually + Astrocytoma grade 4 (3 or 4 criteria present)

The current 2000 WHO grading system stratifies previous low-grade astrocytomas (WHO grades I and II) into different subtypes. WHO grade I pilocytic astrocytoma is characteristically well circumscribed, cystic, and localized. WHO grade II diffuse astrocytoma, like WHO grades III and IV, is characteristically infiltrative and progressive

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