What is Rosette-forming glioneuronal tumor?
Rosette-forming glioneuronal tumor (RGNT) is a rare, slow-growing brain tumor classified by the World Health Organization (WHO) as a grade I mixed glioneuronal neoplasm. It predominantly arises in the fourth ventricle and aqueduct region of the brain, though cases in other locations such as the pineal region, cerebellum, thalamus, and spinal cord have been reported. The tumor is characterized by a distinctive biphasic architecture consisting of neurocytic rosettes (Homer Wright-type rosettes and perivascular pseudorosettes) and a glial component resembling pilocytic astrocytoma. Key symptoms are primarily related to the tumor's location and its tendency to obstruct cerebrospinal fluid flow, leading to obstructive hydrocephalus. Patients commonly present with headaches, nausea, vomiting, dizziness, gait disturbances (ataxia), and visual disturbances including diplopia and papilledema. Some patients may experience seizures, cranial nerve palsies, or progressive neurological deficits depending on the tumor's exact location and size. The onset of symptoms is often insidious, developing over months to years. The primary treatment for rosette-forming glioneuronal tumor is surgical resection, which is often curative when complete removal is achieved. Given its benign nature (WHO grade I), the prognosis is generally favorable. In cases where complete resection is not possible due to the tumor's location near critical brain structures, subtotal resection followed by observation with serial imaging is typically recommended. The role of adjuvant radiation therapy or chemotherapy remains unclear and is generally not indicated for this tumor type. Recurrence after surgery is uncommon but has been reported, necessitating long-term follow-up with neuroimaging.
Also known as:
- Inheritance
- Sporadic
- Usually appears on its own, not inherited from a parent
- Age of Onset
- Adult
- Begins in adulthood (age 18 or older)
Treatments
Source: openFDA + DailyMed · NDA / BLA labels with structured indications · refreshed weekly
No FDA-approved treatments are currently listed for Rosette-forming glioneuronal tumor.
View clinical trials →Clinical Trials
View all trials with filters →Source: ClinicalTrials.gov · synced daily · phases, status, and PI names normalized at ingest
No actively recruiting trials found for Rosette-forming glioneuronal tumor at this time.
New trials open frequently. Follow this disease to get notified.
Source: NPI Registry + PubMed · trial PI roles cross-referenced with ClinicalTrials.gov · ranked by match score (publications + PI activity + community signal)
Treatment Centers
8 centersSource: NORD Rare Disease Centers + NIH Undiagnosed Diseases Network (UDN) · centers verified active within last 12 months
Children's Hospital Colorado Rare Disease Program ↗
Children's Hospital Colorado
📍 Aurora, CO
👤 Boston Children's Hospital Rare Disease Program
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDBoston Children's Hospital Rare Disease Program ↗
Boston Children's Hospital
📍 Boston, MA
👤 Boston Children's Hospital Rare Disease Program
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
🏨 Children'sAnn & Robert H. Lurie Children's Hospital Genetics ↗
Lurie Children's Hospital
📍 Chicago, IL
👤 Boston Children's Hospital Rare Disease Program
🏥 NORDCincinnati Children's Hospital Medical Center ↗
Cincinnati Children's
📍 Cincinnati, OH
👤 Boston Children's Hospital Rare Disease Program
🏨 Children'sNationwide Children's Hospital Rare Disease Center ↗
Nationwide Children's Hospital
📍 Columbus, OH
👤 Boston Children's Hospital Rare Disease Program
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
Travel Grants
No travel grants are currently matched to Rosette-forming glioneuronal tumor.
Community
No community posts yet. Be the first to share your experience with Rosette-forming glioneuronal tumor.
Start the conversation →Latest news about Rosette-forming glioneuronal tumor
Source: PubMed + NIH RePORTER + openFDA + clinical-journal RSS · last 30 days · disease-tagged at ingest by AI extraction with human QC
No recent news articles for Rosette-forming glioneuronal tumor.
Follow this condition to be notified when news becomes available.
Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
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 Rosette-forming glioneuronal tumor
What is Rosette-forming glioneuronal tumor?
Rosette-forming glioneuronal tumor (RGNT) is a rare, slow-growing brain tumor classified by the World Health Organization (WHO) as a grade I mixed glioneuronal neoplasm. It predominantly arises in the fourth ventricle and aqueduct region of the brain, though cases in other locations such as the pineal region, cerebellum, thalamus, and spinal cord have been reported. The tumor is characterized by a distinctive biphasic architecture consisting of neurocytic rosettes (Homer Wright-type rosettes and perivascular pseudorosettes) and a glial component resembling pilocytic astrocytoma. Key symptoms
How is Rosette-forming glioneuronal tumor inherited?
Rosette-forming glioneuronal tumor follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Rosette-forming glioneuronal tumor typically begin?
Typical onset of Rosette-forming glioneuronal tumor is adult. Age of onset can vary across affected individuals.
Which specialists treat Rosette-forming glioneuronal tumor?
2 specialists and care centers treating Rosette-forming glioneuronal tumor are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.
Frequently asked questions about Rosette-forming glioneuronal tumor
Auto-generated from canonical disease facts (Orphanet, OMIM, ClinicalTrials.gov, openFDA, NPPES). Not a substitute for clinical guidance.
What is Rosette-forming glioneuronal tumor?
Rosette-forming glioneuronal tumor is a rare disease catalogued in international rare-disease ontologies (Orphanet ORPHA:251975). It is typically inherited as sporadic. Age of onset is generally adult. For verified primary sources, see the UniteRare Rosette-forming glioneuronal tumor page.
How is Rosette-forming glioneuronal tumor inherited?
Rosette-forming glioneuronal tumor follows sporadic inheritance. Genetic counseling is recommended for affected families to understand recurrence risk in offspring and the likelihood of unaffected siblings being carriers. Variants in the underlying gene(s) may be identified via clinical genetic testing.
Are there FDA-approved treatments for Rosette-forming glioneuronal tumor?
Approved treatments for Rosette-forming glioneuronal tumor are tracked from openFDA and DailyMed primary sources. Many rare diseases have no specific FDA-approved therapy; for those, supportive care and management of complications form the basis of clinical care. Orphan-drug-designation status is noted where applicable.
Are there clinical trials for Rosette-forming glioneuronal tumor?
Active clinical trials for Rosette-forming glioneuronal tumor are tracked daily from ClinicalTrials.gov. Trial availability changes frequently; check the UniteRare trial listings for the current count and recruitment status. Sponsors of rare-disease research often welcome inquiries even when a trial is not actively recruiting at a given moment.
How do I find a specialist for Rosette-forming glioneuronal tumor?
UniteRare lists 2 verified clinicians with documented expertise in Rosette-forming glioneuronal tumor, sourced from ClinicalTrials.gov principal-investigator records, PubMed publication histories, and the NPPES NPI registry. Filter by state or browse our state-specific specialist pages for nearby options.
See full Rosette-forming glioneuronal tumor page for complete clinical details, sources, and verified-specialist listings.
Cite this page
Select a citation format above to view and copy.