Adult brain tumors: Difference between revisions
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* Astrocytic tumors: An increased risk of astrocytic tumors has been observed in patients who receive therapeutic radiation therapy for pituitary adenomas, craniopharyngioma, pineal parenchymal tumors, germinoma, and tinea capitis. In addition, children who receive prophylactic radiation therapy of the CNS for acute lymphoblastic leukemia have an increased risk of developing astrocytomas. Recurrent lesions often signal histologic progression to a higher grade; this malignant progression is associated with a cumulative acquisition of multiple genetic alterations. | * Astrocytic tumors: An increased risk of astrocytic tumors has been observed in patients who receive therapeutic radiation therapy for pituitary adenomas, craniopharyngioma, pineal parenchymal tumors, germinoma, and tinea capitis. In addition, children who receive prophylactic radiation therapy of the CNS for acute lymphoblastic leukemia have an increased risk of developing astrocytomas. Recurrent lesions often signal histologic progression to a higher grade; this malignant progression is associated with a cumulative acquisition of multiple genetic alterations. | ||
:* Pilocytic astrocytoma: Pilocytic astrocytoma (WHO grade I) is a grossly circumscribed, slow-growing, often cystic tumor that occurs primarily in children and young adults.[5] Histologically, pilocytic astrocytomas are composed of varying proportions of compacted bipolar cells with Rosenthal fibers and loose-textured multipolar cells with microcysts and granular bodies. This tumor is the most common glioma in children and represents 10% of cerebral and 85% of cerebellar astrocytic tumors. Occurring throughout the neuraxis, the preferred sites include the optic nerve, optic chiasm/hypothalamus, thalamus and basal ganglia, cerebral hemispheres, cerebellum, and brain stem. Pilocytic astrocytoma is the principal CNS tumor associated with neurofibromatosis type 1 (NF1). No specific cytogenetics or molecular genetics exist with this tumor. This tumor is infrequently fatal. | :* Pilocytic astrocytoma: Pilocytic astrocytoma (WHO grade I) is a grossly circumscribed, slow-growing, often cystic tumor that occurs primarily in children and young adults.[5] Histologically, pilocytic astrocytomas are composed of varying proportions of compacted bipolar cells with Rosenthal fibers and loose-textured multipolar cells with microcysts and granular bodies. This tumor is the most common glioma in children and represents 10% of cerebral and 85% of cerebellar astrocytic tumors. Occurring throughout the neuraxis, the preferred sites include the optic nerve, optic chiasm/hypothalamus, thalamus and basal ganglia, cerebral hemispheres, cerebellum, and brain stem. Pilocytic astrocytoma is the principal CNS tumor associated with neurofibromatosis type 1 (NF1). No specific cytogenetics or molecular genetics exist with this tumor. This tumor is infrequently fatal. | ||
:* Diffuse astrocytoma (including fibrillary, protoplasmic, and gemistocytic). | :* Diffuse astrocytoma (including fibrillary, protoplasmic, and gemistocytic): Diffuse astrocytoma (WHO grade II), also known as low-grade diffuse astrocytoma, is characterized by slow growth and infiltration of neighboring brain structures.[6] Histologically, diffuse astrocytomas are composed of well-differentiated fibrillary or gemistocytic neoplastic astrocytes. This type of tumor typically affects young adults and has a tendency for malignant progression to anaplastic astrocytoma and, ultimately, glioblastoma. Diffuse astrocytomas represent 35% of all astrocytic brain tumors.[7] They may be located in any region of the CNS but most commonly develop in the cerebrum. Three histologic variants include: fibrillary astrocytoma, gemistocytic astrocytoma, and protoplasmic astrocytoma. These types of tumors may occur in patients with inherited TP53 germline mutations (Li-Fraumeni syndrome). TP53 (also known as p53) mutations have been reported in more than 60% of the cases. The most common chromosomal alteration seen in diffuse astrocytoma is the deletion of chromosome band 17p13.1.[7] The mean survival time after surgical intervention is in the range of 6 to 8 years, with considerable individual variation. | ||
:* Anaplastic astrocytoma. | :* Anaplastic astrocytoma. | ||
:* Glioblastoma (including giant cell glioblastoma, and gliosarcoma). | :* Glioblastoma (including giant cell glioblastoma, and gliosarcoma). | ||
Revision as of 21:00, 11 January 2009
| Adult brain tumors |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Associate Editor in Chief: Cafer Zorkun, M.D., Ph.D. [2]
Please Take Over This Page and Apply to be Editor-In-Chief for this topic: There can be one or more than one Editor-In-Chief. You may also apply to be an Associate Editor-In-Chief of one of the subtopics below. Please mail us [3] to indicate your interest in serving either as an Editor-In-Chief of the entire topic or as an Associate Editor-In-Chief for a subtopic. Please be sure to attach your CV and or biographical sketch.
Classification & Grading
The WHO grading of CNS tumors establishes a malignancy scale based on histologic features of the tumor. 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. Some tumor types tend to progress to higher grades of malignancy.
- WHO grade III includes lesions with histologic evidence of malignancy, generally in the form of mitotic activity, clearly expressed infiltrative capabilities, and anaplasia.
- WHO grade IV includes lesions that are mitotically active, necrosis-prone, and generally associated with a rapid preoperative and postoperative evolution of disease.
Brain Tumors in Adult Population
Neuroepithelial tumors
Glial tumors
- Astrocytic tumors: An increased risk of astrocytic tumors has been observed in patients who receive therapeutic radiation therapy for pituitary adenomas, craniopharyngioma, pineal parenchymal tumors, germinoma, and tinea capitis. In addition, children who receive prophylactic radiation therapy of the CNS for acute lymphoblastic leukemia have an increased risk of developing astrocytomas. Recurrent lesions often signal histologic progression to a higher grade; this malignant progression is associated with a cumulative acquisition of multiple genetic alterations.
- Pilocytic astrocytoma: Pilocytic astrocytoma (WHO grade I) is a grossly circumscribed, slow-growing, often cystic tumor that occurs primarily in children and young adults.[5] Histologically, pilocytic astrocytomas are composed of varying proportions of compacted bipolar cells with Rosenthal fibers and loose-textured multipolar cells with microcysts and granular bodies. This tumor is the most common glioma in children and represents 10% of cerebral and 85% of cerebellar astrocytic tumors. Occurring throughout the neuraxis, the preferred sites include the optic nerve, optic chiasm/hypothalamus, thalamus and basal ganglia, cerebral hemispheres, cerebellum, and brain stem. Pilocytic astrocytoma is the principal CNS tumor associated with neurofibromatosis type 1 (NF1). No specific cytogenetics or molecular genetics exist with this tumor. This tumor is infrequently fatal.
- Diffuse astrocytoma (including fibrillary, protoplasmic, and gemistocytic): Diffuse astrocytoma (WHO grade II), also known as low-grade diffuse astrocytoma, is characterized by slow growth and infiltration of neighboring brain structures.[6] Histologically, diffuse astrocytomas are composed of well-differentiated fibrillary or gemistocytic neoplastic astrocytes. This type of tumor typically affects young adults and has a tendency for malignant progression to anaplastic astrocytoma and, ultimately, glioblastoma. Diffuse astrocytomas represent 35% of all astrocytic brain tumors.[7] They may be located in any region of the CNS but most commonly develop in the cerebrum. Three histologic variants include: fibrillary astrocytoma, gemistocytic astrocytoma, and protoplasmic astrocytoma. These types of tumors may occur in patients with inherited TP53 germline mutations (Li-Fraumeni syndrome). TP53 (also known as p53) mutations have been reported in more than 60% of the cases. The most common chromosomal alteration seen in diffuse astrocytoma is the deletion of chromosome band 17p13.1.[7] The mean survival time after surgical intervention is in the range of 6 to 8 years, with considerable individual variation.
- Anaplastic astrocytoma.
- Glioblastoma (including giant cell glioblastoma, and gliosarcoma).
- Pleomorphic xanthoastrocytoma.
- Subependymal giant cell astrocytoma.
- Oligodendroglial tumors.
- Oligodendroglioma.
- Anaplastic oligodendroglioma.
- Mixed gliomas.
- Oligoastrocytoma.
- Anaplastic oligoastrocytoma.
- Ependymal tumors.
- Myxopapillary ependymoma.
- Subependymoma.
- Ependymoma (including cellular, papillary, clear cell, and tanycytic).
- Anaplastic ependymoma.
- Neuroepithelial tumors of uncertain origin.
- Astroblastoma.
- Chordoid glioma of the third ventricle.
- Gliomatosis cerebri.
Neuronal and mixed neuronal-glial tumors (some glial component may be present)
- Gangliocytoma.
- Ganglioglioma.
- Desmoplastic infantile astrocytoma / ganglioglioma.
- Dysembryoplastic neuroepithelial tumor.
- Central neurocytoma.
- Cerebellar liponeurocytoma
- Paraganglioma.
Nonglial tumors
- Embryonal tumors.
- Ependymoblastoma.
- Medulloblastoma.
- Supratentorial primitive neuroectodermal tumor (PNET).
- Choroid plexus tumors.
- Choroid plexus papilloma.
- Choroid plexus carcinoma.
- Pineal parenchymal tumors.
- Pineoblastoma.
- Pineocytoma.
- Pineal parenchymal tumor of intermediate differentiation.
Meningeal tumors
- Meningioma.
- Hemangiopericytoma.
- Melanocytic lesion.
Germ cell tumors
- Germinoma.
- Embryonal carcinoma.
- Yolk-sac tumor (endodermal-sinus tumor).
- Choriocarcinoma.
- Teratoma.
- Mixed germ cell tumor.
Tumors of the sellar region
- Pituitary adenoma.
- Pituitary carcinoma.
- Craniopharyngioma.
Tumors of uncertain histogenesis
- Capillary hemangioblastoma.
Primary CNS lymphoma
Tumors of peripheral nerves that affect the CNS
- Schwannoma.
Metastatic tumors