Fibrillary astrocytoma

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ORPHA:251601C71.9
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3Specialists8Treatment centers

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Overview

Fibrillary astrocytoma is a type of diffuse astrocytoma, classified as a World Health Organization (WHO) grade II brain tumor. It is the most common subtype of low-grade diffuse astrocytoma and arises from astrocytes, the star-shaped glial cells that support neurons in the central nervous system. The tumor is characterized by increased cellularity of fibrillary astrocytes with mild nuclear atypia and a diffusely infiltrative growth pattern, meaning it spreads into surrounding brain tissue without a clear border. Fibrillary astrocytomas can occur in any region of the brain but are most commonly found in the cerebral hemispheres, particularly the frontal and temporal lobes. Key symptoms depend on the tumor's location and size and may include seizures (often the presenting symptom), headaches, cognitive changes, personality or behavioral alterations, focal neurological deficits such as weakness or speech difficulties, and increased intracranial pressure. Because these tumors grow slowly, symptoms may develop gradually over months to years before diagnosis. However, fibrillary astrocytomas carry a risk of malignant progression to higher-grade astrocytomas (anaplastic astrocytoma or glioblastoma) over time. The current treatment landscape involves a multimodal approach. Surgical resection is the primary treatment, with maximal safe resection associated with improved outcomes. For tumors that cannot be completely removed or that show progression, adjuvant radiation therapy and chemotherapy (commonly temozolomide) may be employed. Molecular markers, including IDH1/IDH2 mutation status and 1p/19q codeletion status, are now integral to classification and prognosis under the updated WHO classification of central nervous system tumors. IDH-mutant tumors generally carry a more favorable prognosis. Regular surveillance with MRI imaging is essential for monitoring disease progression. Despite treatment, recurrence is common, and long-term management remains a significant clinical challenge.

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 Fibrillary astrocytoma.

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

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Specialists

3 foundView all specialists →
PB
Patricia Baxter
Specialist
PI on 1 active trial10 Fibrillary astrocytoma publications
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 Fibrillary astrocytoma.

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Community

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

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Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Common questions about Fibrillary astrocytoma

What is Fibrillary astrocytoma?

Fibrillary astrocytoma is a type of diffuse astrocytoma, classified as a World Health Organization (WHO) grade II brain tumor. It is the most common subtype of low-grade diffuse astrocytoma and arises from astrocytes, the star-shaped glial cells that support neurons in the central nervous system. The tumor is characterized by increased cellularity of fibrillary astrocytes with mild nuclear atypia and a diffusely infiltrative growth pattern, meaning it spreads into surrounding brain tissue without a clear border. Fibrillary astrocytomas can occur in any region of the brain but are most commonly

How is Fibrillary astrocytoma inherited?

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

At what age does Fibrillary astrocytoma typically begin?

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

Which specialists treat Fibrillary astrocytoma?

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