Aim of the study

Rosette-forming glioneuronal tumour (RGNT) of the fourth ventricle is an uncommon tumour. The management is not consensual. Most of the published cases show stable outcome with and without gross total resection and are regarded as having a relatively indolent behaviour.

Material and methods

We present a 32-year-old man with a tumour in the fourth ventricle. He underwent midline suboccipital craniectomy with gross total removal.

Results

The histopathological diagnosis was RGNT grade I. Four years later he presented a radiological progression and received stereotactic radiosurgery. At the last follow-up seven years after surgery, the MRI showed no recurrence.

Conclusions

RGNT should be considered in the differential diagnosis of a posterior fossa tumour and has to be differentiated from other lesions for its indolent course and favourable prognosis. Surgical procedures should be carefully performed to avoid serious surgical morbidities. Stereotactic radiosurgery treatment appears to be a useful treatment in recurrence episodes.