Rare Hematology News

Disease Profile

Astroblastoma

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

Infancy

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ICD-10

C71.9

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Cerebral astroblastoma

Categories

Rare Cancers

Summary

Astroblastoma is a rare glial tumor usually located in the cerebral hemisphere of the brain.[1] It can occur in individuals of any age, but occurs predominantly in children and young adults.[2] Signs and symptoms depend on the location and size of the tumor but most commonly include headaches and seizures.[1] Because these tumors are rare, there is little known regarding their behavior and how they should be managed.[3] Gross total resection (complete removal) of the tumor is recommended and is associated with a good prognosis for low-grade tumors. Adjuvant therapy, including radiation and/or chemotherapy, is recommended for high-grade and recurrent cases (although its effectiveness is controversial).[1][4]

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Learn more

    These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

    In-Depth Information

    • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
    • PubMed is a searchable database of medical literature and lists journal articles that discuss Astroblastoma. Click on the link to view a sample search on this topic.

      Selected Full-Text Journal Articles

        References

        1. Agarwal V, Mally R, Palande DA, Velho V. Cerebral astroblastoma: A case report and review of literature. Asian J Neurosurg. April 2012; 7(2):98-100. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3410171/. Accessed 9/4/2012.
        2. John D. Port, Daniel J. Brat, Peter C. Burger and Martin G. Pomper. Astroblastoma: Radiologic-Pathologic Correlation and Distinction from Ependymoma. AJNR. February 2002; 23:243-247.
        3. Sughrue ME et al. Clinical features and post-surgical outcome of patients with astroblastoma. J Clin Neurosci. June 2011; 18(6):750-754.
        4. Andrew D Norden, Milan G Chheda, Patrick Y Wen. Uncommon Brain Tumors. UpToDate. Waltham, MA: UpToDate; July 2012;