cns tumor classification (full)

7/28/2019 CNS Tumor Classification (Full)

1/21

Classification of Brain Tumors

I. Primary tumors of the brainA. Gliomas

Lowest grade tumors

Pilocytic astrocytoma

Subependymal giant cell astrocytoma

Protoplasmic astrocytoma

Ganglioglioma

Xanthomatous astrocytoma

Subependymoma Lower grade malignancies

Fibrillary (gemistocytic, protoplasmic) astrocytoma

Ependymoma

Oligodendroglioma

Mixed oligo-astrocytoma

Optic nerve glioma

Higher-grade malignancies

Anaplastic astrocytoma

Anaplastic oligodendroglioma

Anaplastic mixed glioma

Highest-grade malignancies

Glioblastoma multiforme

Gliosarcoma

Gliomatosis cerebri

B. Meningioma

Benign

Atypical Malignant

C. Primitive Neuroectodermal Tumors (Pnet)

Medulloblastoma

Ependymoblastoma

Pineoblastoma
http://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Gliomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pilocytic%20astrocytomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Subependymal%20giant%20cell%20astrocytomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Protoplasmic%20astrocytomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Gangliogliomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Xanthomatoushttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Subependymomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Fibrillary%20astrocytomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Ependymomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Oligodendrogliomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Mixed%20oligo-astrocytomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Optic%20nerve%20gliomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Anaplastic%20astrocytomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Anaplastic%20oligodendrogliomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Anaplastic%20mixed%20gliomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Glioblastoma%20multiformehttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Gliosarcomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Gliomatosis%20cerebrihttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Meningiomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Primitive%20neuroectodermal%20tumorhttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Medulloblastomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Ependymoblastomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pineoblastomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pilocytic%20astrocytomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Subependymal%20giant%20cell%20astrocytomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Protoplasmic%20astrocytomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Gangliogliomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Xanthomatoushttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Subependymomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Fibrillary%20astrocytomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Ependymomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Oligodendrogliomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Mixed%20oligo-astrocytomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Optic%20nerve%20gliomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Anaplastic%20astrocytomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Anaplastic%20oligodendrogliomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Anaplastic%20mixed%20gliomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Glioblastoma%20multiformehttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Gliosarcomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Gliomatosis%20cerebrihttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Meningiomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Primitive%20neuroectodermal%20tumorhttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Medulloblastomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Ependymoblastomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pineoblastomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Glioma


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D. Pituitary tumors

Pituitary adenoma

Pituitary carcinoma

Craniopharyngioma

Rathke's cleft cystE. Pineal Tumors

Pineal cyst

Pineocytoma

Pineoblastoma

Germinoma

Mixed germ cell tumor

Pineal gliomas

Pineal teratomaF. Choroid plexus tumors

Choroid plexus papilloma

Choroid plexus carcinoma

G. Other, more benign primary tumors

Neurocytoma

Dysembroplastic neuroepithelial tumor (DNT)

Lipoma

Hemangioblastoma

Hamartoma Teratoma

H. Tumors of nerves and/or nerve sheaths

Neuroma

Schwannoma

Neurofibroma

I. Cysts

Colloid cyst

Arachnoid cysts Dermoid

Epidermoid

Rathke's cleft cyst

Pineal cyst

J. Other primary tumors, including skull base

Chondroma
http://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pituitary%20adenomashttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pituitary%20carcinomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Craniopharyngiomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Rathke's%20cleft%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pineal%20tumorshttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pineal%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pineocytomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pineoblastomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Germinomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Choroid%20plexus%20papillomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Choroid%20plexus%20carcinomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Neurocytomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Dysembryoplastic%20neuroepithelial%20tumorhttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Lipomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Hemangioblastomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Hamartomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Teratomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Neuromahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Neurofibromahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Cystshttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Colloid%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Arachnoid%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Dermoid%20or%20epidermoid%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Dermoid%20or%20epidermoid%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Rathke's%20cleft%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pineal%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Chondromahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pituitary%20adenomashttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pituitary%20carcinomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Craniopharyngiomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Rathke's%20cleft%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pineal%20tumorshttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pineal%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pineocytomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pineoblastomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Germinomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Choroid%20plexus%20papillomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Choroid%20plexus%20carcinomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Neurocytomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Dysembryoplastic%20neuroepithelial%20tumorhttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Lipomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Hemangioblastomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Hamartomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Teratomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Neuromahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Neurofibromahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Cystshttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Colloid%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Arachnoid%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Dermoid%20or%20epidermoid%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Dermoid%20or%20epidermoid%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Rathke's%20cleft%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pineal%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Chondroma


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Chordoma

Sarcomas

Gliosarcoma

Chondrosarcoma

RhabdomyosarcomaK. Primary Central Nervous System Lymphoma (PCNSL)

II. Metastatic brain tumors and carcinomatous meningitis

This information has been provided by the AANS/CNS Section on TumorsA listing of most of the tumors that occur in the brain or its surroundingstructures is presented here. Sometimes a tumor may have more than one
http://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Chordomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Sarcomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Gliosarcomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Chondrosarcomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Rhabdomyosarcomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Metastatic%20brain%20tumorshttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Chordomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Sarcomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Gliosarcomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Chondrosarcomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Rhabdomyosarcomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Metastatic%20brain%20tumors


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name. The descriptions of how the tumors behave refer to the most commonpatterns since there are always exceptional cases (both better and worse).Higher grade malignancies are, in general, expected to grow faster thanlower grade malignancies.The comments on tumor treatment usually refer to conventional therapy

(surgery, radiation and cytotoxic chemotherapy) and may not includepromising new, investigational or experimental treatments. Radiosurgerymay be an option for most of these tumors and its use depends upon tumorsize, location and the individual patient's case. If a tumor continues to growdespite treatment, additional surgery may be recommended. This alsodepends on the individual patient.Clinical trials are available for many of these tumors, particularly if they aremalignant or recurrent. Specific tumors are listed alphabetically in the pagesto follow. Discussions ofspinal cord tumors , familial syndromes and theremote effects of carcinoma follow the alphabetical listing.It should be noted that this information is provided for educational purposes

only, and should not be considered to be medical advice. Questions about aspecific patient should be referred to a qualified surgeon or other physician.

I. Primary tumors of the brain

A. Gliomas

Lowest grade tumors

Pilocytic astrocytoma

Subependymal giant cell astrocytoma

Protoplasmic astrocytoma

Ganglioglioma

Xanthomatous astrocytoma Subependymoma

Lower grade malignancies

Fibrillary (gemistocytic, protoplasmic) astrocytoma

Ependymoma

Oligodendroglioma

Mixed oligo-astrocytoma

Optic nerve glioma

Higher-grade malignancies Anaplastic astrocytoma

Anaplastic oligodendroglioma

Anaplastic mixed glioma

Highest-grade malignancies

Glioblastoma multiforme

Gliosarcoma
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Gliomatosis cerebri

B. Meningioma

Benign

Atypical

MalignantC. Primitive neuroectodermal tumors (PNET)

Medulloblastoma

Ependymoblastoma

Pineoblastoma

D. Pituitary tumors

Pituitary adenoma

Pituitary carcinoma

Craniopharyngioma Rathke's cleft cyst

E. Pineal Tumors

Pineal cyst

Pineocytoma

Pineoblastoma

Germinoma

Mixed germ cell tumor

Pineal gliomas

Pineal teratomaF. Choroid plexus tumors

Choroid plexus papilloma

Choroid plexus carcinoma

G. Other, more benign primary tumors

Neurocytoma

Dysembroplastic neuroepithelial tumor (DNT)

Lipoma

Hemangioblastoma Hamartoma

Teratoma

H. Tumors of nerves and/or nerve sheaths

Neuroma

Schwannoma

Neurofibroma
http://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Gliomatosis%20cerebrihttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Meningiomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Primitive%20neuroectodermal%20tumorhttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Medulloblastomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Ependymoblastomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pineoblastomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pituitary%20adenomashttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pituitary%20carcinomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Craniopharyngiomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Rathke's%20cleft%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pineal%20tumorshttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pineal%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pineocytomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pineoblastomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Germinomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Choroid%20plexus%20papillomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Choroid%20plexus%20carcinomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Neurocytomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Dysembryoplastic%20neuroepithelial%20tumorhttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Lipomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Hemangioblastomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Hamartomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Teratomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Neuromahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Neurofibromahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Gliomatosis%20cerebrihttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Meningiomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Primitive%20neuroectodermal%20tumorhttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Medulloblastomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Ependymoblastomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pineoblastomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pituitary%20adenomashttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pituitary%20carcinomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Craniopharyngiomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Rathke's%20cleft%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pineal%20tumorshttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pineal%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pineocytomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pineoblastomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Germinomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Choroid%20plexus%20papillomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Choroid%20plexus%20carcinomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Neurocytomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Dysembryoplastic%20neuroepithelial%20tumorhttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Lipomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Hemangioblastomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Hamartomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Teratomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Neuromahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Neurofibroma


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I. Cysts

Colloid cyst

Arachnoid cysts

Dermoid

Epidermoid Rathke's cleft cyst

Pineal cyst

J. Other primary tumors, including skull base

Chondroma

Chordoma

Sarcomas

Gliosarcoma

Chondrosarcoma Rhabdomyosarcoma

K. Primary Central Nervous System Lymphoma (PCNSL)

II. Metastatic brain tumors and carcinomatous meningitis

PRIMARY TUMORS OF THE BRAIN

Acoustic schwannomaThis is a schwannoma (i.e. tumor of the nerve sheath cells -- see "schwannoma ".) which arises in the region of the eighth cranial nerve. It islocated in the posterior fossa (the lower, back part of the cranial cavity,above the neck) in the angle between the cerebellum and pons. It is usually

found in adults who develop hearing loss on one side. Tinnitus, a buzzing orringing sound, may also be present; other symptoms are also possible. Thetumor is benign, grows slowly, and is treated with surgery or radiosurgery. Itis also known as an acoustic neuroma. There is an Acoustic NeuromaAssociation, which can be reached at www.ANAUSA.org .Anaplastic astrocytomaThis tumor is a locally aggressive, destructive form of astrocytoma. Itspreads into, or "infiltrates"normal brain, and is considered malignant. It may
http://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Cystshttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Colloid%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Arachnoid%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Dermoid%20or%20epidermoid%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Dermoid%20or%20epidermoid%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Rathke's%20cleft%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pineal%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Chondromahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Chordomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Sarcomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Gliosarcomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Chondrosarcomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Rhabdomyosarcomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Metastatic%20brain%20tumorshttp://www.neurosurgerytoday.org/media/fact/classification.asp#schwannomahttp://www.anausa.org/http://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Cystshttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Colloid%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Arachnoid%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Dermoid%20or%20epidermoid%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Dermoid%20or%20epidermoid%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Rathke's%20cleft%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Pineal%20cysthttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Chondromahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Chordomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Sarcomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Gliosarcomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Chondrosarcomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Rhabdomyosarcomahttp://www.aans.org/en/Media/Fact%20Sheets/Classification%20of%20Brain%20Tumors.aspx#Metastatic%20brain%20tumorshttp://www.neurosurgerytoday.org/media/fact/classification.asp#schwannomahttp://www.anausa.org/


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occur anywhere within the central nervous system (CNS). Conventionaltreatment options include surgery and radiation therapy. Even after thesetreatments, it may recur. About half of the patients can be expected to havea partial response to chemotherapy. Experimental therapies are oftenrecommended if the tumor continues to progress despite these standard

treatments.Anaplastic mixed gliomaThis is an infiltrating glioma that grows relatively rapidly and contains morethan one type of tumor cell -- an aggressive combination of an astrocytomaand an oligodendroglioma. Conventional treatment options include surgeryand radiation therapy. The response of this tumor to chemotherapy isintermediate between anaplastic astrocytoma and anaplasticoligodendroglioma.Anaplastic oligodendrogliomaAn anaplastic oligodendroglioma is an aggressive (malignant) type ofoligodendroglioma. Conventional treatment options include surgery and

radiation therapy. Also, most of these tumors would be expected to respondto chemotherapy. A combination of procarbazine, CCNU and vincristine,called "PCV" is used. Recent evidence suggests that specific chromosomalabnormalities may predict the response to chemotherapy.Arachnoid cystThis is a sac of cerebrospinal fluid (CSF), which has been "trapped" outside orinside the brain. It might also be called a "leptomeningeal cyst". Some ofthese cysts may date to birth; others may arise or be diagnosed after a headinjury. Usually such a cyst produces no symptoms and is found bycoincidence when a patient gets scanned for a headache or after a headinjury. However, the fluid (CSF) might be able to enter the sac more easily

than exit, so the cyst might grow over time. On rare occasions, these cystsmay cause loss of neurologic function due to stretching normal nerve cells orputting pressure on the brain, producing seizures, or even causing ahemorrhage if a blood vessel is stretched across the cyst.When surgery is warranted, either endoscopic (i.e. through an "endoscope"or small tube) or open surgery can be used, depending on the surgeon'schoice and the patient's situation. The goal of the surgery is to allow for alarger passage to be created (called a fenestration) between the cyst and thenormal CSF compartments. Alternatively, the collecting fluid is sometimesdiverted by a tube (called a shunt) to another body cavity. Even aftertreatment, the cyst may not appear to change in size, since the brain often

adopts the shape permanently. Similar structures that arise off theventricular system have been called ependymal cysts.AstrocytomaAn astrocytoma is a tumor that arises from astrocytes, which are cells thatsupport and nourish the neurons of the brain. Astrocytes are one type of glia;astrocytomas are a main category of the gliomas, the tumors arising fromthe glia. There are many types of astrocytomas -- please refer to the specifictype, such as pilocytic astrocytoma or anaplastic astrocytoma.


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Butterfly gliomaA tumor of the glial cells (see "glioma") that has spread across the corpuscallosum (which connects the two sides of the brain) so that it involves bothof the cerebral hemispheres. Surgery is rarely helpful, other than fordiagnosis (biopsy).

Brain stem gliomaA tumor arising from the glial cells and located in the brainstem, the deepestportion of the brain. Children are often affected by this tumor. Severaldifferent pathologic types are possible; the type determines how fast thetumor will grow. Surgery, stereotactic surgery, radiation and/orchemotherapy may be used depending on the particular patient.Carcinomatous meningitisSometimes a cancer may spread to the space around the brain or spinalcord, which contains the meninges. Here, there are no barriers to furtherspread throughout this space. The function of individual nerves and/or nerveroots (serving the head and body) becomes impaired. Progression is usually

relentless despite radiation and chemotherapy, even when the drugs aredelivered directly into the spinal fluid. These therapies are often used toattempt to stop the disease, however. This disease is also calledleptomeningeal cancer.ChondromaA chondroma is a tumor that arises from cartilage, usually arising at the baseof the skull. It usually grows slowly, and can become very large. The tumorcan be treated by surgical removal.ChondrosarcomaThis is a rare, malignant tumor arising from cartilage. It may occur in theskull at the joints between bones, or elsewhere in the skeleton. It is locally

invasive, but rarely spreads to other parts of the body. Treatment consists ofsurgical removal, which may be followed by radiation therapy.ChordomaA chordoma is a tumor that comes from a part of the spine or skull -- calledthe notochord -- that is left over from fetal life. It occurs at the base of theskull, or at the end of the spine. Although this tumor is relatively growsrelatively slowly and appears benign under the microscope, the behavior ismore like that of a malignant tumor because it has a tendency to recur andspread. Treatments include surgery, radiation therapy, radiosurgery and insome cases, chemotherapy.Choroid plexus carcinoma

This is a malignant variety of the choroid plexus papilloma (see below). Itusually occurs in children, and can invade normal brain. A few patients havebeen cured with complete surgical removal of the tumor. Otherwise, radiationand high dose chemotherapy may be used to attempt control this tumor,because it is highly aggressive and can often be fatal.Choroid plexus papillomaThis is a benign tumor that arises within the brain's ventricles from the cells(in the "choroid plexus") that make spinal fluid. A choroid plexus papilloma


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generally becomes apparent during infancy or early childhood. It may causeover-production (or blockage) of spinal fluid, resulting in an accumulation ofthis fluid within the ventricular cavities of the brain -- this is called"hydrocephalus". The tumor is treated with surgical removal. In rareinstances, the tumor may re-grow or spread throughout the nervous system.

Colloid cystThis benign cyst arises in the third ventricle and is usually filled with thick,mucous-like, fluid. Thought to be present since birth, the cyst graduallygrows and eventually blocks the normal flow the cerebral spinal fluid (CSF)from the lateral ventricles into the third ventricle. This is one type ofhydrocephalus. If it occurs gradually, the patient may develop headache,double vision, difficult walking, memory problems or other difficulties.Sometimes a colloid cyst may produce an abrupt fluid blockage that maycause sudden coma or even death without prior warning. For this reason,many surgeons consider the discovery of a colloid cyst an indication forsurgery.

Management options include stereotactic aspiration, endoscopic removal(surgery through a small tube called and "endoscope") or microsurgicalremoval. Removing the cyst without causing brain damage can be difficult.Treatment should be individualized and take into account the experience ofthe surgeon.CraniopharyngiomaThis is a cystic tumor that arises just above the pituitary gland. It is usuallyfirst diagnosed at one of two ages -- childhood or the elderly. It growsrelatively slowly. The principle threat is to vision (as the tumor may compressthe optic nerves) and to the function of the pituitary gland. When very large,this tumor may threaten neurologic function or life itself due to pressure on

the brain. It may also block the drainage of spinal fluid, causinghydrocephalus. Surgery is usually considered the first line treatment becausea craniopharyngioma can sometimes be cured at the first operation if it iscompletely removed. Complete removal may not be possible however,because of "fingers" of the tumor that invades into adjacent brain structures.In some cases, simple drainage of the cyst fluid and/or injection ofradioactive fluid can control the symptoms and growth. Radiosurgery orradiation therapy may be used in some cases. Although this is a relativelybenign tumor, the progressive growth may be difficult to control and somepatients die of this disease.Cysts

These are not true tumors in the sense of being neoplasms (i.e. "newgrowths") or cancers; nonetheless they may expand over time puttingpressure on vital brain or spinal structures. They are characterized by theaccumulation of fluid or some other substance within a confining layer. Cystsmay occur in isolation, or as part of a true tumor (such as a glioma,metastasis, or hemangioblastoma). Please see the specific type of brain cyst(or tumor) for further information.Dermoid or epidermoid cyst


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These benign growths are due to left over (or implanted) skin tissue (dermis)within the head or spinal canal. The skin tissue grows, producing pearlyscales that would be analogous to the shedding of dead skin. As this materialhas nowhere to go (unlike on the surface of the skin where it would be shed),it accumulates and eventually forms a mass. Treatment is surgical, although

it may not be possible to cure the cyst, as often the skin cells are firmlyadherent to small nerves and blood vessels. Epidermoid cysts contain justthe outer layer of skin and its waste, while dermoids also include deeperlayers of skin tissue that produce hair.Dysembryoplastic neuroepithelial tumor (DNT)This recently described tumor presents with intractable epilepsy in infancyand early childhood. It is usually located in the frontal or temporal lobe of thebrain. A complete surgical resection can cure both the tumor and theseizures. Possible use of radiation is unclear at this point.EpendymoblastomaSee primitive neuroectodermal tumors (PNETs).

EpendymomaThis tumor arises from the ependymal cells lining the cerebrospinal fluid sacs(ventricles) of the brain. It commonly affects children but can occur at anyage. An ependymoma is a low-grade malignancy that is best treated byremoving as much as is safely possible by surgery. Sometimes the tumorcells can spread through the spinal fluid. Radiation therapy is sometimesused. It has not yet been determined whether or not chemotherapy is usefulin these tumors. A myxopapillary ependymoma is a benign tumor that occursin the spine.EpidermoidSee dermoid cyst.

Fibrillary astrocytomaThis is a tumor that grows relatively slowly, arising from astrocytes, the glialcells that nourish and support the neurons within the brain. The tumor cellsof astrocytomas mix and coexist with normal brain tissue. While oftenreferred to as "benign" tumors, they are more accurately considered low-grade malignancies because they have the potential to, and usually do, recuror turn into high-grade malignancies over time. They are often found inyoung adults but can occur at any age. Other types of astrocytomas in thiscategory are the gemistocytic and protoplasmic varieties.Treatment is dictated by the tumor's location and patient's symptoms.Surgery may benefit patients whose tumors are located in parts of the brain

that are not functionally important, those with large tumors exerting pressureon the brain, or those that cause seizures. Because tumor cells grow into thenormal brain and may extend an inch or more from the visible edge of thetumor, not every cell can be removed surgically. Cells left behind can lead totumor recurrence. Because of this, follow-up scans may be needed.The role of radiation therapy is controversial for low-grade astrocytomas.Although radiation therapy may lead to longer survival, the side effects ofradiation, such as impaired thinking and memory, limit its practical use in
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people with these tumors. In addition, the inability to then use radiationtreatments when the tumor progresses has led many doctors to deferradiation treatments in patients who are not having symptoms and whosetumors are not actively growing.Gangliocytoma

This tumor arises from nerve cells and may also be called a ganglioneuroma.It usually is found in children and is rarely malignant. It may cause seizuresand is usually treated by surgical removal.GangliogliomaThis tumor contains nerve cells and glial (supportive) cells and typicallyproduces seizures beginning in childhood. The seizures may be difficult tocontrol with medicine. Surgical removal is often feasible and is associatedwith good long-term tumor and seizure control for most patients.Germ cell tumorThere are several types of germ cell tumors, including the germinoma (seebelow), embryonal carcinoma, choriocarcinoma, and yolk sac (endodermal

sinus) tumors. They are relatively uncommon, and usually occur in youngadults. Usual locations are near the pineal gland or in the suprasellar region(above the pituitary gland). These tumors may spread through the spinalfluid. Sometimes "tumor markers" (in the blood and or spinal fluid) are usedto make the diagnosis. Treatment may include biopsy, chemotherapy and/orradiation therapy.GerminomaThis tumor looks just like ovarian or testicular cancer under the microscope.It is the most common of the germ cell tumors of the brain. It may spread or"seed" through the spinal fluid. About one third of tumors in the pineal regionare germinomas; however, this tumor can occur in many locations within the

brain. This tumor may cause headaches, visual problems, hormonaldisturbances and blockage of spinal fluid (hydrocephalus). It is treated withsurgery (often a biopsy for making the diagnosis), radiotherapy andsometimes chemotherapy. The germinoma is very responsive to radiationtherapy, but doctors may attempt to avoid the use of radiation in the veryyoung.Glioblastoma multiformeThis is the most common primary (i.e. from the brain itself) brain tumoraffecting adults. Unfortunately, it is a rapidly growing, destructive tumor thatmay lead to death within months. Under the microscope, the tumor has thefeatures of an anaplastic astrocytoma with the addition of areas of dead

tissue (necrosis). Necrosis occurs because the tumor cells grow faster thannew blood vessels can be produced to nourish the tumor cells. Surgery,radiation and chemotherapy are first line treatments that may prolong life toa year or so. Not all patients are candidates for surgical removal of thetumor, for example if the tumor has already spread into vital structures or toboth sides of the brain (e.g. "butterfly glioma").It is extremely rare for this tumor to be completely removed by surgery. Thisis because at the time of first diagnosis, it has usually already spread deep


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into the brain. Removing the part of the tumor that "lights up" on the MRI orCT scan does not mean that all the malignant cells have been removed.While radiation therapy does help most patients to live a bit longer,chemotherapy only benefits about a quarter of patients. Another treatmentthat has been approved by the U.S. Food and Drug Administration (FDA) for

these tumors is the surgical implantation of wafers containing chemotherapydirectly into the area of the tumor. Radiosurgery has also been used.Immunotherapy, gene therapy and other experimental treatments arecurrently under investigation for this very serious disease.GliomaThis is a general term for any tumor that arises from tissues of the brainother than nerve cells and blood vessels -- i.e. the "glia". There are manytypes of gliomas (see scheme above). The behavior of a glioma may rangefrom truly benign to highly malignant, depending on exactly what type it is,and the individual patient.Gliomatosis cerebri

The best way to think of gliomatosis cerebri is as a diffuse, infiltrating high-grade astrocytoma without a definite mass. The tumor cells are intermixedwith normal tissue throughout the majority of the brain. Gliomatosis cerebridoes not respond well to radiation or chemotherapy, although these may beused to attempt to slow the course of the disease. Surgery other than biopsyis also usually not an option.GliosarcomaThis tumor has the characteristics of a glioblastoma, but also includesmalignant tissue that is not glial in origin (sarcoma) -- which may come, forinstance, from the membranes that cover the brain. The tumor tends to beeven more resistant to conventional treatments (surgery, radiation therapy

and chemotherapy) than glioblastoma. Small tumors might be partiallycontrolled by radiosurgery.HamartomaNot a neoplasm in the usual sense, this "mass" may either be an overgrowthof tissue at its native site, or tissue growing normally but in the wrong place.A common site is in the hypothalamus where it may produce seizures. Iftreatment is needed at all, surgery or radiosurgery are used.HemangioblastomaThese are usually benign tumors of blood vessels, often found in the lowerpart of the brain (posterior fossa). They may be associated with cysts.Although usually solitary, multiple hemangioblastomas can occur in Von

Hippel-Lindau disease (see below), a hereditary disorder that may also beassociated with tumors of the retina, pancreas and/or kidney. This tumor maycause dysfunction of the cerebellum (causing difficulty with walking and/orco-ordination, for instance). It also may block the drainage of spinal fluid,leading to hydrocephalus. Treatment is usually by surgical removal, and canbe curative. There are some reports of successful treatment withradiosurgery. Radiation therapy or radiosurgery may also be used if thetumor cannot be removed completely.


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HemangiopericytomaThis is a fairly rare tumor that arises from the same cells as a meningioma. Ittends to recur and spread, even to locations outside the nervous system.Treatment usually includes surgery and radiation therapy. After thesetreatments, patients need to be followed with scans for local recurrence, and

also with studies to evaluate other organs, such as the liver and lungs.LipomaThis is a benign tumor composed of fat cells (adipose tissue). Lipomas in thehead rarely cause symptoms and are often diagnosed coincidentally. Thosein the spine may cause the spinal cord to become stretched or "tethered".Management, if necessary, is usually surgical.LymphomaThis is a tumor arising from lymphatic tissue (like the lymph nodes) -- whichis a main component of the body's immune system. Although lymphomasfrom elsewhere in the body may spread to the brain, most cases oflymphoma affecting the brain originate within the brain itself. Such a tumor

is called a "primary CNS lymphoma". Unless a tumor is large enough to belife threatening from its mass, treatment usually does not include surgery(except for biopsy). Symptoms may be due to increased intracranialpressure, or due to where the tumor (or tumors) are located more specificallywithin the brain. Traditionally, radiation was the preferred treatment,however, early chemotherapy now appears to result in longer and bettersurvivals. One treatment for lymphoma involves disruption of the "blood-brain barrier", so that higher doses of chemotherapy can be delivered.In many cases, CNS lymphoma occurs in patients with AIDS (acquiredimmunodeficiency syndrome) or in people infected with HIV (humanimmunodeficiency virus). Such patients do not respond to treatment in the

same way as patients without AIDS/HIV.MedulloblastomaSee primitive neuroectodermal tumors (PNETs). A medulloblastoma is a PNETthat arises in or near the fourth ventricle. It is a fast-growing, invasive tumor,which may spread through the spinal fluid. Children are usually affected,boys more commonly than girls. Treatment includes surgical removal, staging(see PNET), and radiation and/or chemotherapy, depending on the patient'sage.MeningiomaA meningioma is a tumor that arises from the membranes that cover thebrain and surround the central nervous system (i.e. the "linings" of the skull

and spine). It is the most common benign brain tumor in adults.Meningiomas usually grow slowly; some may not grow at all and the doctormay choose to follow possible growth of the tumor with scans done overtime. Symptoms may include headache, seizure, loss of brain function (suchas weakness, incoordination, sensory problems), visual problems, hearing orswallowing difficulties, loss of smell or taste, or other problems depending onthe location and behavior of the mass. This tumor may grow to be quite largebefore it produces symptoms. The explanation for this is that some


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meningiomas grow so slowly that the brain can gradually shift or adapt tothe presence of the tumor. On the other hand, meningiomas can be foundcoincidentally on scans that are being done for other reasons. Removing asmall meningioma, for example, wouldn't necessarily be expected to curesomeone's headaches.

The preferred treatment for a meningioma is usually considered to besurgery if the lesion can be largely removed at sufficiently low risk. A morecomplete removal is associated with a lower risk of recurrence orprogression. The surgical removal usually includes bone and/or dura (one ofthe layers of the meninges) into which the tumor has spread. Replacement ofthese structures may be needed. Radiation therapy, radiosurgery and/orgrowth-modifying drugs may be considered as treatment options forincompletely-removed tumors or at time of tumor recurrence or progression.A small fraction of meningiomas may be aggressive or malignant. Malignantmeningiomas are treated with surgery, radiation therapy and possiblychemotherapy.

Metastatic brain tumorsAbout 1 in 4 patients with cancer will develop tumors that spread to thecentral nervous system (CNS), most commonly through the blood stream tothe brain. Tumors that often spread to the brain include those originating inthe lung, kidney (renal cell carcinoma), or breast, and also melanoma.However, almost any cancer has this potential. Metastatic tumors typicallyarise where the white and gray matter of the brain meet. The symptomsdepend upon the function of the affected part of the brain, but also caninclude headache or seizures -- or no symptoms at all, when first detected.The results of treatment for metastatic brain tumors was once considered tobe bleak, with survival on the order of several weeks. It has been

convincingly shown, however, that aggressive surgical managementcombined with radiation treatment can lead to a substantially betteroutcome in some patients, both in terms of survival and quality of life.Control of a single metastasis to the head is better when surgery is combinedwith radiation therapy, in comparison to either treatment alone. The benefitof aggressive management of multiple brain metastases is less clear, yetdepending on the particular patient, surgery is sometimes considered whenthere are life-threatening tumors, especially if the patient otherwise is ingood condition.Radiosurgery has taken on an increasingly important role in the managementof brain metastases. Control is thought by many to be comparable to that

achieved by surgery, particularly when combined with conventional radiationtreatments. Radiosurgery may also prove effective in controlling sometumors that are resistant to conventional radiation (such as melanoma andkidney cancer). The equivalence of radiosurgery to surgery for brainmetastases has not been proven, however, and there are those who believethat conventional surgery is superior just as there are those who areadvocates of radiosurgery. Treatment should be individualized for eachpatient, and the patient's medical condition and extent of cancer elsewhere


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in the body must be considered.Mixed oligo-astrocytoma (mixed glioma)This tumor shares the microscopic appearance and behavior of bothastrocytomas and oligodendrogliomas (see separate listings). Treatmentoptions include surgery, radiation and/or chemotherapy.

NeurinomaNote: sometimes schwannomas or neurofibromas are referred to asneuromas or neurinomas -- see descriptions of these other tumors.NeuroblastomaA neuroblastoma usually occurs outside the central nervous system. Rarely,it can occur within the brain, more commonly in children. It usually growsrapidly, causing seizures and other neurologic symptoms. It may also spreadthrough the spinal fluid. Some may call it a PNET. Surgery, radiation therapyand/or chemotherapy may be used to attempt to control it.NeurocytomaThese tumors usually arise in the fluid sacs (ventricles) of the brain and often

affect young or middle aged adults. Because of this, it may causehydrocephalus. Although usually slowly-growing and benign, someneurocytomas may be malignant. This tumor is sometimes called a "centralneurocytoma". Maximal surgical resection is the treatment of choice; thetumor may be vascular (i.e. contain many blood vessels). Radiation therapyis usually reserved for progressive or more malignant-appearingneurocytomas.NeurofibromaThis is a tumor of a nerve, which mixes with normal nerve tissue. It usuallyoccurs in the nerves outside the spine and head. Since it is mixed withnormal nerve tissue, surgical removal would be expected to lead to loss of

function of that nerve. If left untreated, however, the tumor may continue togrow, or possibly turn into malignant tumor over time. Note: sometimesschwannomas or neurofibromas are referred to as neuromas or neurinomas.NeuromaThese are benign growths of abnormal nerve tissue that usually occur at thesite of a nerve injury. Neuromas may be quite painful and treatment isusually medical. Surgery can be considered for cases where pain fails torespond to medical management. Note: sometimes schwannomas orneurofibromas are referred to as neuromas or neurinomas.OligodendrogliomaThese tumors are thought to arise from the oligodendrocytes, which are the

cells that wrap around nerve cells and act as a form of electrical insulationfor conducting the nerve impulses. Recent evidence suggests that they mayactually arise from progenitor cells that are immature oligodendrocytes.These tumors also tend to occur in young adults and may contain calciumdeposits that appear on brain scans. They tend to be slower growing thanlow-grade astrocytomas, but have the potential to turn into more aggressivetumors. Treatment is usually surgical and radiation therapy may berecommended. Chemotherapy is considered for progressive tumors, and


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usually recommended for anaplastic (i.e. more malignant)oligodendrogliomas. (Also see anaplastic oligodendroglioma).Optic nerve gliomaOptic nerve gliomas occur in about 10% of patients with neurofibromatosistype I (NF I -- see below), usually during childhood. They may involve the

optic nerve, optic chiasm and/or optic tract, which are various parts of thevisual system. The tumor usually does not spread to other parts of the brain,unless it is a higher grade (more malignant) glioma. In those without NF I,they may occur at any age. These tumors may be treated with radiationand/or chemotherapy; surgery is also sometimes used depending on thepatient.Pilocytic astrocytomaThis tumor is usually found in a child or young adult. The name comes fromthe "hair-like" appearance of the tumor cells under the microscope. Thetumor is typically slowly- growing and often can be cured by completesurgical removal. Some pilocytic astrocytomas, however, may behave more

aggressively or be surgically inaccessible. Treatment with radiation mayeventually lead to malignant progression (more rapid growth, and braininvasion).Pineal cystThis is generally considered to be a benign lesion, rarely causing symptoms.A pineal cyst is often discovered coincidentally when a patient is scanned foran unrelated reason such as a headache.Pineal tumorsThese are growths that occur in the region of the pineal gland, situated deepwithin the brain. They may obstruct the cerebrospinal fluid (CSF) pathways,causing hydrocephalus, which is treated with a surgery to "shunt" the fluid

that is accumulating. Because they are difficult to reach safely, indirectmeans of diagnosing these lesions may be recommended, such as specialblood and/or spinal fluid tests ("marker" studies -- see germ cell tumors) or atrial of low dose radiation. A biopsy, or removal of the tumor might also berecommended, depending on the patient and test results. Also see thespecific type of pineal tumor, e.g. pineocytoma.PineoblastomaThis is a PNET of the pineal gland (see PNET). It tends to grow rapidly andchemotherapy and radiation therapy is usually recommended.PineocytomaThis is a tumor derived from pineal gland tissue, but it grows more slowly

than a pineoblastoma. It is treated with surgery and radiotherapy;radiosurgery has also been used.Pituitary adenomasThese are common benign tumors of the pituitary gland. It is said that up to10 percent of people will have a pituitary adenoma (which might never havecaused a problem) by the time of their death. The pituitary gland isconsidered the "master gland" of the body; it produces hormones thatregulate the other glands. Some tumors secrete one or more of these


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hormones in excess. Such so-called secretory pituitary adenomas are usuallyfound due to hormonal imbalances that affect bodily functions. They may berelatively small when detected.Syndromes of secreting pituitary adenomas:

Name Hormone SymptomsProlactinoma Prolactin Women: breast milkproduction, change inmenstrual period.Men: breastenlargement,impotence.

Cushing's disease ACTH & Cortisone Weight gain, highblood pressure

Acromegaly Growth Hormone Enlarging tissue andorgans, diabetes,

gigantismHyperthyroidism TSH& Thyroid

Weight loss,irritability, heatintolerance

Treatment of a secretory pituitary adenoma is directed not only at controllingtumor growth, but also at eliminating hormone over-production. Trueprolactinomas may often be successfully treated with medicine alone,however many tumors may cause small elevations of blood prolactin but notrespond to this treatment. Other secreting tumors (or prolactinomas that donot respond well to medical therapy) are treated with surgery, radiosurgery,

standard radiation therapy alone, or some combination. If unchecked, somesecreting pituitary adenomas may prove fatal.In contrast, non-secreting pituitary adenomas are rarely detected until theygrow large enough to compress the optic nerves, resulting in some loss ofvision. Again, surgery, radiosurgery, standard radiation therapy alone or acombination of these is used for treatment. These tumors tend to recur andmay be quite difficult to control as they are often too large to be safely curedby surgery when found. Unchecked, patients may go blind, suffer otherneurologic loss or die from pressure or infection of the brain.Pituitary carcinomaThese are rare cancers of the pituitary gland. They are difficult to control

locally and are usually treated with surgery and some type of radiation(radiotherapy and/or radiosurgery). They may spread to other organs of thebody.Pituitary region tumorsIn addition to tumors of the pituitary gland itself, tumors or cysts may arisefrom nearby structures -- see the specific type of tumor, e.g. pituitaryadenoma.Primitive neuroectodermal tumor (PNET)


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This is a malignant tumor arising from cells that are believed to remain fromfetal brain development. They usually occur in early childhood but maybecome symptomatic in adult life. In children, they commonly occur in ornear a spinal fluid sac known as the fourth ventricle and are calledmedulloblastomas. Under the microscope, a PNET is seen to consist of

densely-packed small cells that are usually blue in color (when commontissue processing is performed). PNETs have a tendency to spread over thebrain and spinal cord by way of the spinal fluid. Treatment usually beginswith surgery. Radiation therapy is used in adults, along with chemotherapy.Young children are usually treated with chemotherapy alone, since radiationmay stunt intellectual development when given at an early age.PNET staging is an important consideration, since the extent of treatmentneeded depends on how widely the PNET has spread at the time ofdiagnosis. This "staging process" requires MRI scans of the brain and spinalcolumn, as well as examination of the spinal fluid under the microscope tolook for tumor cells.

Protoplasmic astrocytomaThis is a rare tumor which is typically slow-growing and displaces rather thanmixes in with (i.e. infiltrates) normal brain tissue. The tumor is thereforesomewhat better controlled by surgery than the more common low-gradeastrocytoma. Additional treatment may include radiation therapy orradiosurgery if the tumor progresses.Pseudotumor cerebri (benign intracranial hypertension)This condition does not involve a tumor at all, hence the use of the prefix"pseudo". It refers to an increase in pressure inside the head, which can leadto loss of vision and changes in the optic nerves -- findings which can also becaused by true brain tumors. The condition may due to high levels of vitamin

A, or to a major blood channel (sinus) shutting down inside the head (usuallydue to an infection or blood clotting disorder). In other cases the cause isunknown, but may be related to obesity. Treatment options include medicaltreatment of the pressure, a shunt to drain spinal fluid and lower thepressure, and/or optic nerve sheath decompression, to relieve pressure onthe optic nerves. For obese patients, weight loss is also needed.Rathke's cleft cystThis is a slowly-growing fluid-filled cyst, thought to be left over from the fetalstage. The theory is that cells that should have migrated to the nasal/throatarea became trapped in the region of the pituitary gland. Treatment includesobservation, surgery and/or radiosurgery.

RhabdomyosarcomaThis is a malignant tumor of the muscle cells. It may arise anywhere amuscle normally exists, such as near the eye.SarcomaA sarcoma is a malignant tumor arising from connective or structural tissuesuch as bones, cartilage or dura (one of the linings of the skull and spine).Sarcomas are generally malignant but encompass a range of behaviors. Theytend to be resistant to conventional treatments (such as surgery, radiation


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and chemotherapy). Surgery and sometimes radiosurgery may be useful.Please refer to the specific type of sarcoma, e.g. gliosarcoma.SchwannomaA schwannoma typically arises along a nerve, since it is comprised of cellsthat normally provide the "electrical insulation" for the nerve cells. Usually

benign, traditional management has consisted of surgical removal.Schwannomas often displace the remainder of the normal nerve, instead ofmixing in with it (see neurofiboma). Common locations include along thenerves in the head (especially the balance or "vestibular" nerve [also knownas an acoustic schwannoma]), in the spine, and, more rarely, along nervesthat go to the limbs. Some schwannomas in the head may be treated withradiosurgery instead of, or in addition to, conventional microsurgery. Rarelythe tumors may be malignant. Note: sometimes schwannomas orneurofibromas are referred to as neuromas or neurinomas.SubependymomaLike an ependymoma, this tumor also arises from tissue that lines the

ventricles. However, it more often occurs in elderly patients. The behavior isbenign. Unfortunately, subependymomas often arise from the brainstem andsurgeons may have to leave some tumor behind if they are to avoidneurologic damage.Subependymal giant cell astrocytomaGenerally a benign tumor, arising from tissue off the fluid sacs (ventricles) ofthe brain. This tumor is often seen in children and some adults with acondition called Tuberous Sclerosis. Tuberous Sclerosis is characterized byseizures, certain skin abnormalities of the face, and varying degrees ofmental retardation. Treatment usually consists of surgical removal, orobservation if it is not causing symptoms.

Skull base tumorsPlease refer to the specific tumor, such as chordoma, meningioma,etc.TeratomaA benign tumor, which is a complex accumulation of normal tissue, butgrowing in the wrong place. The tumor may include hair, teeth, muscle and avariety of other tissues. Treatment is surgical.Xanthomatous astrocytoma (pleomorphic xanthastrocytoma)This is a rare tumor usually seen in young adults, often found in a temporallobe after a seizure. It tends not to infiltrate (mix) with normal brain tissue,but may spread along the meninges. The tumor and further seizures are

usually controlled by its surgical removal, but it is intermediate in its' grade(i.e. degree of malignancy). Follow-up scans over time are recommended.Radiation, radiosurgery and/or re-operation may be suggested for a recurrenttumor.

ADDITIONAL INFORMATION

SPINAL TUMORSMany of the types of tumors described above (especially glioblastoma,


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astrocytoma and ependymoma) can affect the spinal cord (without beingpresent in the brain). Metastatic tumors often involve the bones of the spine,and/or the spinal cord. There are also tumors of the spinal nerves (such asneurofibromas or schwannomas), lining of the spinal canal (meningiomas),and spinal fluid compartment (meningeal carcinomatosis or gliomatosis).

Primary tumors may arise from the bones of the spine itself. Treatment isoften the same as for their cranial counterparts, although radiosurgery forthe spine is in its infancy and is generally considered investigational.FAMILIAL SYNDROMESIn some cases, tumors affecting the nervous system are part of aconstellation of problems as opposed to being an isolated tumor. In addition,these syndromes may be passed on to the children of the affected person.For autosomal dominant illnesses the risk of a child getting the disorder fromone affected parent is 1 in 2 or 50 per cent.NeurofibromatosisThis disease is also known as von Recklinghausen's Disease and is generally

divided into two types:Neurofibromatosis type I is characterized by tumors of the "peripheral"nerves that run throughout the limbs, trunk and head. These tumors mayinclude plexiform neurofibromas and/or schwannomas. It is associated withparticular patches of skin discoloration called "caf-au-lait" spots. In extremecases the skin tumors can be disfiguring. Abnormalities of other organs andbones may also occur. Although many cases occur spontaneously, theypersons afflicted with NF I can pass it on to their offspring as an autosomallydominant trait.Neurofibromatosis type II is often associated with schwannomas(neuromas) of both vestibular nerves. It often leads to deafness, due either

to the tumors or their treatment. Other tumors such as gliomas of the opticnerves or hypothalamus, ependymomas or multiple meningiomas arecommon. Both spontaneous and hereditary forms occur and the disease ispassed on as an autosomcal dominant disorder.Lindau SyndromeThis disease (also known as von Hippel-Lindau disease) is characterized bythe presence of multiple hemangioblastomas, usually in the cerebellum, andthe spinal cord, as well as tumors of the retina, pancreas and kidney. Surgerycan be used to treat both the brain and spinal cord lesions while radiosurgeryhas been reported to control some brain lesions. Inheritance is autosomaldominant. Information about the von Hippel-Lindau Family Alliance can be

viewed atwww.vhl.org .Tuberous SclerosisThis syndrome often presents in childhood and is characterized by seizures,mental retardation, specific facial lesions (hamartomas) and areas of skindiscoloration. Tumors of the lining of the ventricles (subependymal giant cellastrocytomas) may block flow of the cerebrospinal fluid leading to a backupof the fluid called hydrocephalus. The tumors and hydrocephalus are usuallytreated with surgery. Inheritance is autosomal dominant.
http://www.vhl.org/http://www.vhl.org/


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Remote effects of carcinomaSome tumors produce chemicals or hormones that can directly or indirectlycause nerve cells to die or malfunction without physically contacting thosenerve cells. These chemicals are carried through the blood stream to remoteareas. Although these remote effects of carcinoma are rare, they can be

incapacitating. Treatment usually requires effective control of the tumorproducing the substances.

cns tumor classification (full)

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