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Appendix 1: WHO Classification and Grading of Tumours of the Central Nervous System
Contents:
  1. Conclusions
  2. WHO releases updated Classification of Tumours of the Central Nervous System
  3. The WHO Classification of Tumours of the Central Nervous System: The Major Points of Revision
  4. World Health Organization (WHO) Classification of Adult Primary CNS Tumors
  5. Appendix 1: WHO Classification

Patients with higher-grade tumors have worse prognoses.

Conclusions

Standard treatment options for pineal astrocytic tumors include the following:. Standard treatment options for pilocytic astrocytomas include the following:. Controversy exists about the timing of radiation therapy after surgery. Some physicians use surgery alone if a patient has clinical factors that are considered low risk, such as age less than 40 years and the lack of contrast enhancement on a computed tomographic scan. The discovery of the isocitrate dehydrogenase IDH 1 and IDH2 mutations in diffuse gliomas has greatly helped to identify patients who are considered high risk. A number of large, retrospective studies has demonstrated that the IDH1 and IDH2 mutation is a powerful independent prognostic factor for improved survival.

Molecular correlative data from the RTOG trial, which would be informative about which patients benefited the most from the addition of PCV, have not yet been reported. Standard treatment options for anaplastic astrocytomas include the following:. A subset of anaplastic astrocytomas is aggressive; these tumors are frequently managed in the same way as glioblastomas, with surgery and radiation, and often with chemotherapy.

However, the optimal treatment for these tumors is not established. Two phase III randomized trials restricted to patients with anaplastic gliomas NCT and NCT are currently enrolling patients, but efficacy data are not available. It is not known whether the improved survival of patients with chemotherapy-treated glioblastoma can be extrapolated to patients with anaplastic astrocytomas.

Patients with anaplastic astrocytomas are appropriate candidates for clinical trials designed to improve local control by adding newer forms of treatment to standard treatment. Information about ongoing clinical trials is available from the NCI website. For patients with glioblastoma WHO grade IV , the cure rate is very low with standard local treatment.

WHO releases updated Classification of Tumours of the Central Nervous System

Standard treatment options for patients with newly diagnosed glioblastoma include the following:. The standard treatment for patients with newly diagnosed glioblastoma is surgery followed by concurrent radiation therapy and daily temozolomide, and then followed by six cycles of temozolomide.

The addition of bevacizumab to radiation therapy and temozolomide did not improve OS. There was significant crossover in both trials. The two trials had contradictory results in health-related quality of life HRQoL and neurocognitive outcomes studies.

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The reasons for these discrepancies are unclear. On the basis of these results, there is no definite evidence that the addition of bevacizumab to standard therapy is beneficial for all newly diagnosed glioblastoma patients. Certain subgroups may benefit from the addition of bevacizumab, but this is not yet known.

Glioblastoma patients are appropriate candidates for clinical trials designed to improve local control by adding newer forms of treatment to standard treatment. Patients who have oligodendrogliomas WHO grade II generally have better prognoses than do patients who have diffuse astrocytomas. Standard treatment options for oligodendrogliomas include the following:.

The WHO Classification of Tumours of the Central Nervous System: The Major Points of Revision

Controversy exists concerning the timing of radiation therapy after surgery. A study EORTC of patients with low-grade gliomas who had surgery and were randomly assigned to either radiation therapy or watchful waiting, did not show a difference in OS between the two groups. For low-grade WHO grade II tumors that are considered high risk , radiation therapy followed by six cycles of PCV chemotherapy is a recommended option based on the long-term follow-up results of RTOG, a randomized trial for high-risk, low-grade gliomas.

Notably, the RTOG study enrolled patients with a mixed variety of tumors, including astrocytomas, oligodendrogliomas, and mixed oligoastrocytomas; in a retrospective subset analysis, only the oligodendroglial tumors appeared to benefit from the addition of PCV. The discovery of the IDH1 and IDH2 mutations, which are independent prognostic factors for significantly improved survival in diffuse gliomas, has greatly helped to identify patients who are considered high risk. Molecular correlative data from the RTOG trial, which would be informative about which patients benefited most from the addition of PCV, have not yet been reported.

Patients with anaplastic oligodendrogliomas WHO grade III have a low cure rate with standard local treatment, but their prognoses are generally better than are the prognoses of patients with anaplastic astrocytomas. Two phase III randomized trials restricted to patients with anaplastic gliomas NCT and NCT are currently enrolling patients; however, efficacy data are not yet available.

These patients are appropriate candidates for clinical trials designed to improve local control by adding newer forms of treatment. Standard treatment options for anaplastic oligodendrogliomas include the following:. Evidence surgery followed by radiation therapy with or without chemotherapy :. The combination of radiation and chemotherapy is not known to be superior in outcome to sequential modality therapy. Therefore, assessment of these molecular markers may aid management decisions for anaplastic oligodendrogliomas. Testing for these known, strong, prognostic molecular markers should be performed, which may help to guide the assessment of risk and subsequent management.

Standard treatment options for grades I and II ependymal tumors include the following:. Patients with anaplastic ependymomas WHO grade III have variable prognoses that depend on the location and extent of disease. Frequently, but not invariably, patients with anaplastic ependymomas have worse prognoses than do those patients with lower-grade ependymal tumors.


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Standard treatment options for anaplastic ependymomas include the following:. Medulloblastoma occurs primarily in children, but may also occur in adults. Pineocytomas are slow-growing tumors and prognosis varies. Pineoblastomas grow more rapidly and patients with these tumors have worse prognoses. Pineal parenchymal tumors of intermediate differentiation have unpredictable growth and clinical behavior.

Standard treatment options for pineal parenchymal tumors include the following:. WHO grade I meningiomas are usually curable when they are resectable. With the increasing use of sensitive neuroimaging tools, there has been more detection of asymptomatic low-grade meningiomas.

World Health Organization (WHO) Classification of Adult Primary CNS Tumors

Most appear to show minimal growth and can often be safely observed while therapy is deferred until growth or the development of symptoms. Standard treatment options for grades II and III meningiomas and hemangiopericytomas include the following:. The prognoses and treatment of patients with germ cell tumors—which include germinomas, embryonal carcinomas, choriocarcinomas, and teratomas—depend on tumor histology, tumor location, presence and amount of biological markers, and surgical resectability.

Standard treatment options for craniopharyngiomas include the following:. Patients who have CNS tumors that are either infrequently curable or unresectable should be considered candidates for clinical trials.

Appendix 1: WHO Classification

Heavy-particle radiation, such as proton-beam therapy, carries the theoretical advantage of delivering high doses of ionizing radiation to the tumor bed while sparing surrounding brain tissue. The data are preliminary for this investigational technique, and are not widely available. Novel biologic therapies under clinical evaluation for patients with CNS tumors include the following:[ 36 ]. Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients.

The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria.

General information about clinical trials is also available. Surgery and radiation therapy are the primary modalities used to treat tumors of the spinal axis; therapeutic options vary according to the histology of the tumor. Patients who have spinal axis tumors that are either infrequently curable or unresectable should be considered candidates for clinical trials. The most common primary tumors with brain metastases and the percentage of patients effected are as follows:[ 1 , 2 ]. Brain involvement can occur with cancers of the nasopharyngeal region by direct extension along the cranial nerves or through the foramina at the base of the skull.

The diagnosis of brain metastases in cancer patients is based on the following:. A physical examination may show objective neurological findings or only minor cognitive changes. The presence of multiple lesions and a high predilection of primary tumor metastasis may be sufficient to make the diagnosis of brain metastasis.

Basic introduction to CNS TUMORS

A lesion in the brain should not be assumed to be a metastasis just because a patient has had a previous cancer; such an assumption could result in overlooking appropriate treatment of a curable tumor. Computed tomography scans with contrast or MRIs with gadolinium are quite sensitive in diagnosing the presence of metastases. Positron emission tomography scanning and spectroscopic evaluation are new strategies to diagnose cerebral metastases and to differentiate the metastases from other intracranial lesions.

In the case of a solitary lesion or a questionable relationship to the primary tumor, a brain biopsy via resection or stereotactic biopsy may be necessary.


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  7. The optimal therapy for patients with brain metastases continues to evolve. Because most cases of brain metastases involve multiple metastases, a mainstay of therapy has historically been whole-brain radiation therapy WBRT ; however, stereotactic radiosurgery has come into increasingly common use. The role of radiosurgery continues to be defined. Stereotactic radiosurgery in combination with WBRT has been assessed.