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Adult Brain Tumors Treatment (PDQ®)

Health Professional Version

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
 IIIIIIIV
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 astrocytoma X  
Diffuse astrocytoma X  
Pleomorphic xanthoastrocytoma X  
Anaplastic astrocytoma  X 
Glioblastoma   X
Giant cell glioblastoma   X
Gliosarcoma   X
Oligondendroglial tumors
Oligodendroglioma X  
Anaplastic oligodendroglioma  X 
Oligoastrocytic tumors
Oligoastrocytoma X  
Anaplastic oligoastrocytoma  X 
Ependymal tumors
SubependymomaX   
Myxopapillary ependymomaX   
Ependymoma X  
Anaplastic ependymoma  X 
Choroid plexus tumors
Choroid plexus papillomaX   
Atypical choroid plexus papilloma X  
Choroid plexus carcinoma  X 
Other neuroepithelial tumors
Angiocentric gliomaX   
Chordoid glioma of the third ventricle X  
Neuronal and mixed neuronal-glial tumors
GangliocytomaX   
GangliogliomaX   
Anaplastic ganglioma  X 
Desmoplastic infantile astrocytoma and gangliogliomaX   
Dysembryoplastic neuroepithelial tumorX   
Central neurocytoma X  
Extraventricular neurocytoma X  
Cerebellar liponeurocytoma X  
Paraganglioma of the spinal cordX   
Papillary glioneuronal tumorX   
Rosette-forming glioneural tumor of the fourth ventricleX   
Pineal tumors     
PineocytomaX   
Pineal parenchymal tumor of intermediate differentiation XX 
Pineoblastoma   X
Papillary tumor of the pineal region XX 
Embryonal tumors
Medulloblastoma   X
CNS primitive neuroectodermal tumor (PNET)   X
Atypical teratoid/rhabdoid tumor   X
Tumors of the cranial and paraspinal nerves
SchwannomaX   
NeurofibromaX   
PerineuriomaXXX 
Malignant peripheral nerve sheath tumor (MPNST) XXX
Meningeal tumors
MeningiomaX   
Atypical meningioma X  
Anaplastic/malignant meningioma  X 
Hemangiopericytoma X  
Anaplastic hemangiopericytoma  X 
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]
  • Updated: February 28, 2014