Rosette-forming glioneuronal tumor of the aqueduct of Sylvius: Case report and literature review

Background Rosette-forming glioneuronal tumors (RGNTs) are rare, low-grade tumors, with various CNS anatomical locations predominantly in fourth ventricle, cerebella and supratentorial ventricular system. The tumor most commonly occurs in young adults. The most common reported clinical symptoms associated with RGNTs have been headache and ataxia. Based on tumor location, surgical resection (total, subtotal or partial) or biopsy has been the first treatment option. Case presentation We present a 26-years old female case of RGNT originated in the aqueduct of Sylvius. Magnetic resonance imaging (MRI) revealed an aqueductal cystic lesion associated with a mild hydrocephalus of the supratentorial ventricles. An endoscopic third ventriculostomy with biopsy of the aqueductal cystic lesion was performed. Postoperative, there were no complications and MRI showed interval reduction in the size of aqueductal cystic lesion and resolution of the obstructive hydrocephalus. In conclusion This case reported a RGNT originated in the aqueduct of Sylvius associated with hydrocephalus. Although various surgical techniques have been used to treat an aqueductal RGNT, we showed that endoscopic third ventriculostomy with tumor biopsy may be an effective therapeutic option.