Protoplasmic astrocytoma

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ORPHA:251598C71.9
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1Specialists8Treatment centers

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Overview

Protoplasmic astrocytoma is a rare subtype of diffuse astrocytoma (WHO grade II), a low-grade brain tumor arising from astrocytes, which are star-shaped glial cells that support neurons in the central nervous system. This tumor is classified under the ICD-10 code C71.9 (malignant neoplasm of brain, unspecified). Protoplasmic astrocytomas are characterized by a low cellularity, mucoid or microcystic degeneration, and tumor cells with few, thin processes. They are among the rarest variants of diffuse astrocytomas and tend to occur in the cerebral cortex and temporal lobes. Because they are brain tumors, they can affect neurological function depending on their location and size. Patients may present with seizures, headaches, cognitive changes, personality alterations, focal neurological deficits, or signs of increased intracranial pressure. Symptoms often develop insidiously over months to years due to the slow-growing nature of the tumor. Diagnosis is typically made through neuroimaging (MRI) and confirmed by histopathological examination of tissue obtained via biopsy or surgical resection. Treatment generally involves maximal safe surgical resection when feasible, which is the primary therapeutic approach. Depending on the extent of resection, patient age, and molecular characteristics of the tumor, adjuvant therapies such as radiation therapy and/or chemotherapy (commonly temozolomide) may be considered. Despite being classified as low-grade, protoplasmic astrocytomas carry a risk of progression to higher-grade gliomas over time. Under the updated WHO 2021 classification of central nervous system tumors, the histological subtyping of diffuse astrocytomas (including the protoplasmic variant) has been largely replaced by molecular classification based on IDH mutation status and other genetic markers. Long-term follow-up with serial imaging is essential for monitoring disease 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 Protoplasmic astrocytoma.

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No actively recruiting trials found for Protoplasmic 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 Protoplasmic 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.

Mental Health Support

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Family & Caregiver Grants

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 Protoplasmic astrocytoma

What is Protoplasmic astrocytoma?

Protoplasmic astrocytoma is a rare subtype of diffuse astrocytoma (WHO grade II), a low-grade brain tumor arising from astrocytes, which are star-shaped glial cells that support neurons in the central nervous system. This tumor is classified under the ICD-10 code C71.9 (malignant neoplasm of brain, unspecified). Protoplasmic astrocytomas are characterized by a low cellularity, mucoid or microcystic degeneration, and tumor cells with few, thin processes. They are among the rarest variants of diffuse astrocytomas and tend to occur in the cerebral cortex and temporal lobes. Because they are brain

How is Protoplasmic astrocytoma inherited?

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

At what age does Protoplasmic astrocytoma typically begin?

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

Which specialists treat Protoplasmic astrocytoma?

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