Overview
Embryonal tumor of neuroepithelial tissue is a broad category of rare, aggressive central nervous system (CNS) tumors that arise from primitive or undifferentiated neuroepithelial cells. These tumors predominantly occur in children and include several subtypes such as embryonal tumor with multilayered rosettes (ETMR), medulloepithelioma, CNS neuroblastoma, CNS ganglioneuroblastoma, and atypical teratoid/rhabdoid tumor, among others. They primarily affect the brain and, less commonly, the spinal cord, and are classified as high-grade (WHO grade IV) malignancies due to their rapid growth and tendency to disseminate through the cerebrospinal fluid. Key symptoms depend on tumor location but commonly include signs of increased intracranial pressure such as headaches, nausea, vomiting, and lethargy. Children may present with seizures, focal neurological deficits, developmental regression, visual disturbances, gait abnormalities, or cranial nerve palsies. In infants, an enlarging head circumference may be an early sign. The clinical course is often aggressive, with rapid symptom progression. Treatment typically involves a multimodal approach including maximal safe surgical resection, followed by chemotherapy and, depending on the patient's age and tumor subtype, craniospinal radiation therapy. Prognosis varies significantly by subtype, patient age, extent of resection, and presence of metastatic disease at diagnosis. Despite advances in treatment, many subtypes carry a guarded prognosis, and ongoing clinical trials are investigating novel targeted therapies and immunotherapies. Long-term survivors require careful monitoring for treatment-related late effects including neurocognitive impairment, endocrine dysfunction, and secondary malignancies.
Sporadic
Usually appears on its own, not inherited from a parent
Childhood
Begins in childhood, roughly ages 1 to 12
FDA & Trial Timeline
7 eventsImmVira Pharma Co. Ltd — PHASE1, PHASE2
John M. Buatti — NA
Kuopio University Hospital — NA
Fondazione IRCCS Policlinico San Matteo di Pavia
Duke University — PHASE1
Cellectar Biosciences, Inc. — PHASE1
Institut de Recherches Internationales Servier — PHASE3
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Embryonal tumor of neuroepithelial tissue.
View clinical trials →Clinical Trials
View all trials with filters →No actively recruiting trials found for Embryonal tumor of neuroepithelial tissue at this time.
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Rare Disease Specialist
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 Embryonal tumor of neuroepithelial tissue.
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Start the conversation →Latest news about Embryonal tumor of neuroepithelial tissue
Disease timeline:
New recruiting trial: Investigating Mechanistic Predictors of Interpatient Variability and Temozolomide (TMZ) Induced Haematological Toxicity for Glioma Patients
A new clinical trial is recruiting patients for Embryonal tumor of neuroepithelial tissue
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A new clinical trial is recruiting patients for Embryonal tumor of neuroepithelial tissue
New recruiting trial: Trilateral Retinoblastoma: Incidence and Outcomes
A new clinical trial is recruiting patients for Embryonal tumor of neuroepithelial tissue
New recruiting trial: MRE Scan for the Assessment of Differences in Tissue Stiffness Between Radiation Necrosis and Recurrent Glioma in Patients With Previously Treated Gliomas
A new clinical trial is recruiting patients for Embryonal tumor of neuroepithelial tissue
New recruiting trial: 18F-Fluciclovine PET-MRI in High-grade Glioma
A new clinical trial is recruiting patients for Embryonal tumor of neuroepithelial tissue
New recruiting trial: Evaluation of Diagnostic Performances of 18F-FDOPA PET KInetics
A new clinical trial is recruiting patients for Embryonal tumor of neuroepithelial tissue
New recruiting trial: Feasibility of Aspirate Tissue Monitoring in Neuro-oncological Surgery
A new clinical trial is recruiting patients for Embryonal tumor of neuroepithelial tissue
New recruiting trial: Study of GC101 TIL in Brain Glioma (Soochow2)
A new clinical trial is recruiting patients for Embryonal tumor of neuroepithelial tissue
New recruiting trial: T2 Star Magnetic Resonance Imaging and Biomarker Blood Testing to Predict the Change and Progress of Malignant Gliomas
A new clinical trial is recruiting patients for Embryonal tumor of neuroepithelial tissue
New recruiting trial: Three miRNA Signatures in Glioma: From Molecular Mechanisms to Potential Clinical Application
A new clinical trial is recruiting patients for Embryonal tumor of neuroepithelial tissue
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Common questions about Embryonal tumor of neuroepithelial tissue
What is Embryonal tumor of neuroepithelial tissue?
Embryonal tumor of neuroepithelial tissue is a broad category of rare, aggressive central nervous system (CNS) tumors that arise from primitive or undifferentiated neuroepithelial cells. These tumors predominantly occur in children and include several subtypes such as embryonal tumor with multilayered rosettes (ETMR), medulloepithelioma, CNS neuroblastoma, CNS ganglioneuroblastoma, and atypical teratoid/rhabdoid tumor, among others. They primarily affect the brain and, less commonly, the spinal cord, and are classified as high-grade (WHO grade IV) malignancies due to their rapid growth and ten
How is Embryonal tumor of neuroepithelial tissue inherited?
Embryonal tumor of neuroepithelial tissue follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Embryonal tumor of neuroepithelial tissue typically begin?
Typical onset of Embryonal tumor of neuroepithelial tissue is childhood. Age of onset can vary across affected individuals.
Which specialists treat Embryonal tumor of neuroepithelial tissue?
25 specialists and care centers treating Embryonal tumor of neuroepithelial tissue are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.