Supratentorial Ependymoma Yap1 Fusion Positive Cn377573
A rare brain tumor that forms in the supratentorial region with a YAP1 gene fusion. It can affect children and adults, and treatment typically involves surgery followed by radiation or chemotherapy.
Key facts at a glance
- ICD-10
- C80.1
- ICD-11
- 2A00.0
- Body system
- oncologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Headache
Seizures
Focal neurological deficits
Visual disturbances
Generalized symptoms
(oncologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
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
Aggressive but safe surgical removal of the tumor with preservation of neurological function. Extent of resection is a critical prognostic factor for outcomes in ependymoma.
Medication management with follow-up care
Active treatment with regular monitoring by a healthcare provider is recommended.
Radiation Therapy
Advanced conformal radiation therapy targeting the tumor bed with margin. May include proton beam therapy to minimize radiation exposure to surrounding healthy brain tissue, particularly important in younger patients.
Additional treatment options
Chemotherapy
Systemic chemotherapy, typically including agents such as vincristine with possible carboplatin or cyclophosphamide, particularly considered for younger children to delay radiation or for recurrent disease.
medicationLong-term Surveillance Imaging
Regular MRI surveillance for early detection of recurrence, typically every 3-6 months initially, with spacing intervals based on clinical course and tumor grade.
monitoringNeurocognitive Support
Comprehensive neurocognitive assessment and rehabilitation services to address learning difficulties, processing speed, and attention deficits that may result from tumor and treatment.
complementaryWhen 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 Supratentorial Ependymoma Yap1 Fusion Positive Cn377573?
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.
Louis DN, Wesseling P, Aldape KD, et al. cIMPACT-NOW update 7: advancing the molecular classification of ependymal tumors. Neuro Oncol. 2020.
Pajtler KW, Mack SC, Ramaswamy V, et al. The current consensus on the clinical management of intracranial ependymoma and its distinct molecular variants. Acta Neuropathol. 2017.
Rizzo D, Arcella A, Greco S, et al. YAP1 fusion-positive supratentorial ependymomas: clinicopathological characterization and response to therapy. J Neuropathol Exp Neurol. 2021.
Parker M, Mohankumar KM, Punchihewa C, et al. C11orf95-RELA fusions drive oncogenic NF-kB signaling in ependymoma. Nature. 2014.
Medication Comparisons
Compare medications used to treat Supratentorial Ependymoma Yap1 Fusion Positive Cn377573. 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 20, 2026. · Read our editorial policy