Rosette-forming glioneuronal tumor outside the fourth ventricle: a case-based update

Area of Science:

• Neuro-oncology
• Surgical Pathology

Background:

• Rosette-forming glioneuronal tumors (RGNTs) are rare neoplasms.
• Originally thought to be confined to the fourth ventricle, RGNTs present in diverse anatomical locations.
• RGNTs lacking fourth ventricle involvement are particularly uncommon.

Purpose of the Study:

• To review the literature on RGNTs outside the fourth ventricle.
• To analyze the clinical presentation, treatment, and outcomes of these rare tumors.
• To add a case of cerebellar hemisphere RGNT to the existing data.

Main Methods:

• Comprehensive literature review identifying 32 cases of RGNTs not involving the fourth ventricle.
• Inclusion of an additional case of a cerebellar hemisphere RGNT.
• Analysis of treatment modalities and patient outcomes.

Main Results:

• Surgical treatment yielded an excellent outcome with no reported mortality.
• Only two patients required adjuvant therapy for tumor progression.
• The analyzed RGNT was WHO grade I with a biphasic histopathology.

Conclusions:

• RGNTs outside the fourth ventricle demonstrate a favorable oncological outcome following surgical resection.
• Despite good prognosis, long-term surveillance is necessary to monitor for potential recurrence or metastatic disease.
• Surgical management is the primary treatment modality for these tumors.