Figure 1: This mildly complex, circumscribed T2-FLAIR hyperintense rosette-forming glioneuronal tumor fills and expands the fourth ventricle (left), contributing to hydrocephalus in this patient, but causes little edema. The tumor demonstrates a heterogeneous internal pattern of enhancement (right).
- Uncommon, slow-growing, and benign tumor usually arising in the posterior fossa
- WHO grade I
- Composed of pseudorosette-forming neurocytes and astrocytes
- No malignant transformation
- Affects young adults (mean 30 years old)
- Female gender predilection (2:1)
- Commonly presents with signs/symptoms of increased intracranial pressure secondary to obstructive hydrocephalus
- Headache, nausea, ataxia, vertigo
- Treatment: surgical resection
- Prognosis: recurrence uncommon following total resection; 5-year survival 90%
- Mixed solid-cystic tumor ± calcification, hemorrhage; may be solid
- Minimal peritumoral edema
- Majority arise in 4th ventricle or midline cerebellum
- Pineal, CPA cistern, or hemispheric locations are uncommon
- Solid-cystic midline posterior fossa mass
- T1WI: hypo- to isointense
- T2WI: usually hyperintense with cystic or bubbly appearance; ± flow voids
- FLAIR: heterogeneously hyperintense
- T2*/GRE/SWI: black signal blooming in foci of calcification, hemorrhage
- T1WI+C: variable enhancement
Hsu C, et al. Rosette-forming glioneuronal tumour: Imaging features, histopathological correlation and a comprehensive review of literature. Br J Neurosurg. 2012;26:668-673.
Schlamann A, et al. An individual patient data meta-analysis on characteristics and outcome of patients with papillary glioneuronal tumor, rosette glioneuronal tumor with neuropil-like islands and rosette forming glioneuronal tumor of the fourth ventricle. PLoS One. 2014;9:e101211.
Osborn AG, Salzman KL, Jhaveri MD. Diagnostic Imaging (3rd ed). Philadelphia, PA: Elsevier, 2016.
Smith AB, et al. From the radiologic pathology archives: intraventricular neoplasms: radiologic-pathologic correlation. Radiographics. 2013;33:21-43.
Zhang J, et al. A comprehensive analysis of 41 patients with rosette-forming glioneuronal tumors of the fourth ventricle. J Clin Neurosci. 2013;20:335-341.
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