Gemistocytic astrocytoma

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ORPHA:251604C71.9
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Overview

Gemistocytic astrocytoma is a variant of diffuse astrocytoma, a type of brain tumor (glioma) that arises from astrocytes, the star-shaped glial cells that support neurons in the central nervous system. It is characterized by the predominance of gemistocytes — large, plump astrocytes with abundant eosinophilic cytoplasm and eccentric nuclei — comprising at least approximately 20% of the tumor cell population. Historically classified as a World Health Organization (WHO) grade II astrocytoma, gemistocytic astrocytoma is notable for its relatively higher tendency to progress to higher-grade malignancies (anaplastic astrocytoma or glioblastoma) compared to other diffuse astrocytoma subtypes. Under the revised WHO 2016 and 2021 classifications, these tumors are now further characterized by their IDH mutation status, which has significant prognostic implications. Gemistocytic astrocytomas primarily affect the cerebral hemispheres, though they can occur in other regions of the brain. Symptoms depend on tumor location and size and may include headaches, seizures, focal neurological deficits (such as weakness, speech difficulties, or visual changes), cognitive impairment, and personality changes. Increased intracranial pressure may cause nausea, vomiting, and papilledema. The disease predominantly affects adults, typically presenting in the third to fifth decades of life. Treatment generally follows the approach used for diffuse astrocytomas and includes maximal safe surgical resection when feasible, followed by radiation therapy and/or chemotherapy (commonly temozolomide), particularly in cases with unfavorable prognostic features or evidence of progression. Due to the recognized propensity for malignant transformation, close clinical and radiological surveillance is essential. Prognosis varies depending on factors such as IDH mutation status, extent of resection, patient age, and clinical performance status, but the gemistocytic variant is generally considered to carry a somewhat less favorable prognosis than other low-grade diffuse astrocytomas due to its higher rate of progression.

Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Gemistocytic astrocytoma.

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No actively recruiting trials found for Gemistocytic astrocytoma at this time.

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Specialists

1 foundView all specialists →
PM
Patrick A. Thompson, MD
Specialist
PI on 2 active trials

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Gemistocytic astrocytoma.

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Community

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Caregiver Resources

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Common questions about Gemistocytic astrocytoma

What is Gemistocytic astrocytoma?

Gemistocytic astrocytoma is a variant of diffuse astrocytoma, a type of brain tumor (glioma) that arises from astrocytes, the star-shaped glial cells that support neurons in the central nervous system. It is characterized by the predominance of gemistocytes — large, plump astrocytes with abundant eosinophilic cytoplasm and eccentric nuclei — comprising at least approximately 20% of the tumor cell population. Historically classified as a World Health Organization (WHO) grade II astrocytoma, gemistocytic astrocytoma is notable for its relatively higher tendency to progress to higher-grade malign

How is Gemistocytic astrocytoma inherited?

Gemistocytic astrocytoma follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Gemistocytic astrocytoma typically begin?

Typical onset of Gemistocytic astrocytoma is adult. Age of onset can vary across affected individuals.

Which specialists treat Gemistocytic astrocytoma?

1 specialists and care centers treating Gemistocytic astrocytoma are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.