Yap1 Mamld1 Fusion Positive Supratentorial Ependymoma C5670480
A rare brain tumor occurring in the upper part of the brain, driven by a specific genetic fusion. It primarily affects children. Treatment typically involves surgery and radiation therapy.
Key facts at a glance
- ICD-10
- G43.909
- ICD-11
- 8A00.0
- Body system
- neurologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Headache
Focal motor weakness
Visual field deficits
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Seizures
(neurologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Maximal Safe Surgical Resection
Microsurgical tumor resection performed by experienced neurosurgeons with goal of maximal safe resection while preserving neurological function. Extent of resection is strongly correlated with progression-free survival.
Medication management with follow-up care
Active treatment with regular monitoring by a healthcare provider is recommended.
Adjuvant Radiation Therapy
Focal conformal radiation therapy to the tumor bed and any residual disease, typically administered at doses of 54-60 Gy. May include proton beam therapy for pediatric patients to reduce long-term effects.
Additional treatment options
Adjuvant Chemotherapy
Systemic chemotherapy, typically platinum-based regimens, administered either concurrently with radiation or sequentially for higher-risk disease. May include temozolomide or other agents based on molecular tumor characteristics.
medicationSerial MRI Surveillance
Regular magnetic resonance imaging at defined intervals to detect early recurrence, typically every 3 months initially, then extending to every 6 months and annually based on stability.
monitoringRehabilitation and Functional Recovery
Comprehensive neurorehabilitation including physical, occupational, and speech therapy as needed based on tumor location and treatment effects. Focuses on recovery of motor function, cognitive rehabilitation, and return to daily activities.
physical_therapyWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Yap1 Mamld1 Fusion Positive Supratentorial Ependymoma C5670480?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Pajtler KW, Witt H, Sill M, et al. Molecular Classification of Ependymal Tumors across All CNS Compartments, Histopathological Grades, and Age Groups. Cancer Cell. 2015.
Louis DN, Perry A, Wesseling P, et al. The 2021 WHO Classification of Tumors of the Central Nervous System: a summary. Neuro Oncol. 2021.
Parker M, Mohankumar KM, Punchline C, et al. C11orf95-RELA fusions drive oncogenic NF-kappaB signaling in ependymoma. Nature. 2014.
RuppNJ, Campos B, Weyerbacher JC, et al. Supratentorial Ependymoma: Molecular Insights and Classification. Curr Neurol Neurosci Rep. 2020.
Medication Comparisons
Compare medications used to treat Yap1 Mamld1 Fusion Positive Supratentorial Ependymoma C5670480. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 27, 2026. · Read our editorial policy