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Diffusely invasive supratentorial rosette-forming glioneuronal tumor: illustrative case
Brittany Owusu-Adjei1,2, Constance J Mietus1,2, Jeewoo Chelsea Lim1,2
11University of Massachusetts Chan Medical School, Worcester, Massachusetts.
Journal of Neurosurgery. Case Lessons
|October 23, 2023
Summary
Diffuse supratentorial rosette-forming glioneuronal tumors (RGNTs) are rare. Targeted therapy with FGFR inhibitors showed partial remission in a patient with RGNT, offering a potential treatment for advanced disease.
Area of Science:
- Neuro-oncology
- Rare tumors
- Genomic medicine
Background:
- Rosette-forming glioneuronal tumors (RGNTs) are rare neoplasms with glial and neurocytic elements.
- Typically found in the posterior fossa of young adults, RGNTs can present challenges due to diffuse supratentorial involvement.
Purpose of the Study:
- To report an extremely rare case of diffuse supratentorial RGNT.
- To investigate the genetic alterations and treatment response in this challenging presentation.
Main Methods:
- A 23-year-old female with hydrocephalus underwent surgical resection and biopsy of supratentorial lesions.
- Next-generation sequencing identified FGFR-1 and PIK3CA alterations.
- Treatment with the FGFR inhibitor erdafitinib was initiated.
Main Results:
- Pathology confirmed RGNT in both resected and biopsied lesions.
- Next-generation sequencing revealed FGFR-1 and PIK3CA gene alterations.
- The patient achieved partial remission with erdafitinib therapy.
Conclusions:
- Diffuse supratentorial RGNT is an exceptionally rare entity requiring tailored management.
- Surgical goals include diagnosis, safe resection, and hydrocephalus treatment.
- FGFR inhibitors represent a promising therapeutic option for progressive RGNT.
