Supratentorial intraventricular rosette-forming glioneuronal tumors – Case report and review of treatment paradigms

BACKGROUND: Rosette-forming glioneuronal tumors (RGNT) are slow-growing WHO Grade I tumors that are characterized by mixed histology and rosette formation. Although typically located in the posterior fossa, these tumors can rarely originate elsewhere. Here, we describe the fourth case in literature where an RGNT was localized to the lateral ventricles and detail the treatment approach. CASE DESCRIPTION: A 41-year-old male presented with a 10 day history of gradually worsening headaches and mild gait difficulty. Computed tomography and magnetic resonance imaging (MRI) identified a heterogeneously enhancing 6.0 cm left lateral ventricular cystic mass with hydrocephalus. An interhemispheric transcallosal approach was performed for tumor debulking. The mass was emanating from the roof of the left lateral ventricle. Sub-total resection (STR) was achieved. Pathology showed a glioneuronal neoplasm with vague neurocytic rosettes and loose perivascular pseudorosettes. Tumor vessels were thickly hyalinized and contained eosinophilic granular bodies and Rosenthal fibers. Tumor stained positive for GFAP, S-100, OLIG2, and SOX10, and patchy positive for epithelial membrane antigen (EMA), D2-40, CD99, and p16. Neurocytic rosettes and perivascular structures stained positive for synaptophysin. The patient was discharged home uneventfully and remained intact at his 6-month follow-up visit. Long-term care included MRI surveillance with repeat surgery being considered in case of progression. CONCLUSION: In this report, we describe the fourth case of an RGNT being isolated to the lateral ventricles and the first where it stained positive for EMA and D2-40. Our patient’s uneventful recovery after STR indicates that surgery alone continues to be a viable initial treatment option.