Astrocytoma classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

There are two broad classes of classification identified within astrocytomas, based on histology, as to whether the zones of infiltration of cancer cells are narrow or diffuse.

Classification

Within the astrocytomas, there are two broad classes recognized in literature, those with:

  • Narrow zones of infiltration (mostly invasive tumors; e.g., pilocytic astrocytoma, subependymal giant cell astrocytoma, pleomorphic xanthoastrocytoma), that often are clearly outlined on diagnostic images
  • Diffuse zones of infiltration (e.g., low-grade astrocytoma, anaplastic astrocytoma, glioblastoma), that share various features, including the ability to arise at any location in the CNS, but with a preference for the cerebral hemispheres; they occur usually in adults; and an intrinsic tendency to progress to more advanced grades.[1]

Grading

Astrocytomas have great variation in their presentation. The World Health Organization acknowledges the following grading system for astrocytomas:

  • Grade 1pilocytic astrocytoma - primarily pediatric tumor, with median age at diagnosis of 12
  • Grade 2 — diffuse astrocytoma
  • Grade 3 — anaplastic (malignant) astrocytoma
  • Grade 4glioblastoma multiforme (most common)
  • The WHO-grading scheme is based on the appearance of certain characteristics: atypia, mitosis, endothelial proliferation, and necrosis. These features reflect the malignant potential of the tumor in terms of invasion and growth rate. Tumors without any of these features are grade I, and those with one of these features (usually atypia) are grade II. Tumors with 2 criteria and tumors with 3 or 4 criteria are WHO grades III and IV, respectively. Thus, the low-grade group of astrocytomas are grades I and II. According to the WHO data the lowest grade astrocytomas (grade I) make up only 2% of recorded astrocytomas, grade II 8%, and the higher grade anaplastic astrocytomas (grade III) 20%. The highest graded astrocytoma (grade IV GBM) is the most common primary nervous system cancer and second most frequent brain tumor after brain metastasis. Despite the low incidence of astrocytomas compared to other human cancers, mortality is significant, as the higher grades (III & IV) present high mortality rates (mainly due to late detection of the neoplasm).
  • Various types of astrocytomas are given these WHO grades:
WHO Grade Astrocytomas Description
I Consist of slow growing astrocytomas, benign, and associated with long-term survival. Individuals with very slow growing tumors where complete surgical removal by stereotactic surgery is possible may experience total remission.[2] Even if the surgeon is not able to remove the entire tumor, it may remain inactive or be successfully treated with radiation.
II
  • Low-grade (fibrillary astrocytoma) astrocytoma
  • Mixed oligoastrocytoma
Consist of relatively slow-growing astrocytomas, usually considered benign that sometimes evolve into more malignant or as highergrade tumors. They are prevalent in younger people who often present with seizures. Median survival varies with the cell type of the tumor. Grade 2 astrocytomas are defined as being invasive gliomas, meaning that the tumor cells penetrate into the surrounding normal brain. People with oligodendrogliomas (which might share common cells of origin[3]) have better prognoses than those with mixed oligoastrocytomas, who in turn have better prognoses than patients with (pure) low-grade astrocytomas. Other factors which influence survival include age (younger the better) and performance status (ability to perform tasks of daily living). Due to the infiltrative nature of these tumors, recurrences are relatively common. Depending on the patient, radiation or chemotherapy after surgery is an option. Individuals with grade 2 astrocytoma have a 5-year survival rate of about 34% without treatment and about 70% with radiation therapy.[2] The median survival time is 4 years.[2]
III Anaplastic astrocytoma The standard initial treatment is to remove as much of the tumor as possible without worsening neurologic deficits. Radiation therapy has been shown to prolong survival and is a standard component of treatment. Individuals with grade 3 astrocytoma have a median survival time of 18 months without treatment (radiation and chemotherapy).[2] There is no proven benefit to adjuvant chemotherapy or supplementing other treatments for this kind of tumor. Although temozolomide is effective for treating recurrent anaplastic astrocytoma, its role as an adjuvant to radiation therapy has not been fully tested.
IV Glioblastoma multiforme (GBM) Consists of Glioblastoma multiforme (GBM), which is the most common and most malignant primary brain tumor. Primary GBM grow and spread to other parts of the brain quickly; they can become very large before producing symptoms, which often begin abruptly with seizures.[4] Less than 10% form more slowly following degeneration of low-grade astrocytoma or anaplastic astrocytoma. These are called secondary GBM and are more common in younger patients (mean age 45 versus 62 years).[3] "Surgical removal remains the mainstay of treatment, provided that unacceptable neurologic injury can be avoided. The extremely infiltrative nature of this tumor makes complete surgical removal impossible. Although radiotherapy rarely cures glioblastoma, studies show that it doubles the median survival of patients, compared to supportive care alone."[4] The prognosis is worst for these grade 4 gliomas. Few patients survive beyond 3 years. Individuals with grade 4 astrocytoma have a median survival time of 17[2] weeks without treatment, 30[2] weeks with radiation, and 37[2] weeks with surgical removal of most of the tumor followed by radiation therapy. Long-term survival (at least five years) falls well under 3%.[5][6]

See Also


References

  1. http://emedicine.medscape.com/article/283453-overview
  2. 2.0 2.1 2.2 2.3 2.4 2.5 2.6 mdguidelines.com > Astrocytoma Retrieved on Mars 26, 2010
  3. 3.0 3.1 Ohgaki, Hiroko; Kleihues, Paul (2009). "Genetic alterations and signaling pathways in the evolution of gliomas". Cancer Science. 100 (12): 2235–41. doi:10.1111/j.1349-7006.2009.01308.x. PMID 19737147.
  4. 4.0 4.1 quoted from http://www.mayoclinic.org/glioma/glioblastoma.html[full citation needed]
  5. Buckner, Jan C.; Brown, Paul D.; O'Neill, Brian P.; Meyer, Fredric B.; Wetmore, Cynthia J.; Uhm, Joon H. (2007). "Central Nervous System Tumors". Mayo Clinic Proceedings. 82 (10): 1271–86. doi:10.4065/82.10.1271. PMID 17908533.
  6. Central Brain Tumor Registry of the United States, http://www.cbtrus.org/

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