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.
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
Treatments
No FDA-approved treatments are currently listed for Protoplasmic astrocytoma.
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Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Protoplasmic astrocytoma.
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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.