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Chapter 5: Brain Cancer

Lyndon Kim; Howard Fine

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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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