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Adult Brain Tumors Treatment (PDQ®)

Health Professional Version
Last Modified: 02/28/2014

Classification of Adult Brain Tumors

This classification is based on the World Health Organization (WHO) classification of central nervous system (CNS) tumors.[1] The WHO approach incorporates and interrelates morphology, cytogenetics, molecular genetics, and immunologic markers in an attempt to construct a cellular classification that is universally applicable and prognostically valid. Earlier attempts to develop a TNM-based classification were dropped: tumor size (T) is less relevant than tumor histology and location, nodal status (N) does not apply because the brain and spinal cord have no lymphatics, and metastatic spread (M) rarely applies because most patients with CNS neoplasms do not live long enough to develop metastatic disease.[2]

The WHO grading of CNS tumors establishes a malignancy scale based on histologic features of the tumor.[3] The histologic grades are as follows:

WHO grade I includes lesions with low proliferative potential, a frequently discrete nature, and the possibility of cure following surgical resection alone.

WHO grade II includes lesions that are generally infiltrating and low in mitotic activity but recur more frequently than grade I malignant tumors after local therapy. Some tumor types tend to progress to higher grades of malignancy.

WHO grade III includes lesions with histologic evidence of malignancy, including nuclear atypia and increased mitotic activity. These lesions have anaplastic histology and infiltrative capacity. They are usually treated with aggressive adjuvant therapy.

WHO grade IV includes lesions that are mitotically active, necrosis-prone, and generally associated with a rapid preoperative and postoperative progression and fatal outcomes. The lesions are usually treated with aggressive adjuvant therapy.

The following table is from the WHO Classification of Tumours of the Central Nervous System and lists the tumor types and grades.[4] Tumors limited to the peripheral nervous system are not included. Detailed descriptions of histopathology, grading methods, incidence, and what is known about etiology specific to each tumor type can be found in the WHO classification book.[4]

WHO Grades of CNS Tumorsa
 I II III IV 
a Reprinted with permission from Louis, DN, Ohgaki H, Wiestler, OD, Cavenee, WK. World Health Organization Classification of Tumours of the Nervous System. IARC, Lyon, 2007.
Astrocytic tumors
Subependymal giant cell astrocytomaX
Pilocytic astrocytomaX
Pilomyxoid astrocytomaX
Diffuse astrocytomaX
Pleomorphic xanthoastrocytomaX
Anaplastic astrocytomaX
GlioblastomaX
Giant cell glioblastomaX
GliosarcomaX
Oligondendroglial tumors
OligodendrogliomaX
Anaplastic oligodendrogliomaX
Oligoastrocytic tumors
OligoastrocytomaX
Anaplastic oligoastrocytomaX
Ependymal tumors
SubependymomaX
Myxopapillary ependymomaX
EpendymomaX
Anaplastic ependymomaX
Choroid plexus tumors
Choroid plexus papillomaX
Atypical choroid plexus papillomaX
Choroid plexus carcinomaX
Other neuroepithelial tumors
Angiocentric gliomaX
Chordoid glioma of the third ventricleX
Neuronal and mixed neuronal-glial tumors
GangliocytomaX
GangliogliomaX
Anaplastic gangliomaX
Desmoplastic infantile astrocytoma and gangliogliomaX
Dysembryoplastic neuroepithelial tumorX
Central neurocytomaX
Extraventricular neurocytomaX
Cerebellar liponeurocytomaX
Paraganglioma of the spinal cordX
Papillary glioneuronal tumorX
Rosette-forming glioneural tumor of the fourth ventricleX
Pineal tumors
PineocytomaX
Pineal parenchymal tumor of intermediate differentiationXX
PineoblastomaX
Papillary tumor of the pineal regionXX
Embryonal tumors
MedulloblastomaX
CNS primitive neuroectodermal tumor (PNET)X
Atypical teratoid/rhabdoid tumorX
Tumors of the cranial and paraspinal nerves
SchwannomaX
NeurofibromaX
PerineuriomaXXX
Malignant peripheral nerve sheath tumor (MPNST)XXX
Meningeal tumors
MeningiomaX
Atypical meningiomaX
Anaplastic/malignant meningiomaX
HemangiopericytomaX
Anaplastic hemangiopericytomaX
HemangioblastomaX
Tumors of the sellar region
CraniopharyngiomaX
Granular cell tumor of the neurohypophysisX
PituicytomaX
Spindle cell oncocytoma of the adenohypophysisX

References
  1. Kleihues P, Cavenee WK, eds.: Pathology and Genetics of Tumours of the Nervous System. Lyon, France: International Agency for Research on Cancer, 2000. 

  2. Brain and spinal cord. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 593-7. 

  3. Kleihues P, Burger PC, Scheithauer BW: The new WHO classification of brain tumours. Brain Pathol 3 (3): 255-68, 1993.  [PUBMED Abstract]

  4. Louis DN, Ohgaki H, Wiestler OD, et al.: The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol 114 (2): 97-109, 2007.  [PUBMED Abstract]