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Rosette-Forming Glioneuronal Tumor

Last Updated: May 8, 2021

Open Table of Contents: Rosette-Forming Glioneuronal Tumor

Figure 1: (Left) This mildly complex, circumscribed T2-FLAIR hyperintense rosette-forming glioneuronal tumor fills and expands the fourth ventricle, contributing to hydrocephalus in this patient, but causes little edema. (Right) The tumor demonstrates a heterogeneous internal pattern of enhancement.

Figure 1: (Left) This mildly complex, circumscribed T2-FLAIR hyperintense rosette-forming glioneuronal tumor fills and expands the fourth ventricle, contributing to hydrocephalus in this patient, but causes little edema. (Right) The tumor demonstrates a heterogeneous internal pattern of enhancement.

BASIC DESCRIPTION

  • Uncommon, slow-growing, and benign tumor that usually arises in the posterior fossa

PATHOLOGY

  • WHO grade I
  • Composed of pseudorosette-forming neurocytes and astrocytes
  • No malignant transformation

CLINICAL FEATURES

  • Affects young adults (mean age, 30 years)
  • 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 after total resection; 90% 5-year survival rate

IMAGING FEATURES

  • General
    • Mixed solid-cystic tumor ± calcification, hemorrhage; may be solid
    • Minimal peritumoral edema
    • Majority arise in fourth ventricle or midline cerebellum
    • Pineal, cerebellopontine angle cistern, or hemispheric locations are uncommon
  • CT
    • Solid-cystic midline posterior fossa mass
  • MRI
    • T1WI: hypointense 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

IMAGING RECOMMENDATIONS

  • MRI with contrast

For more information, please see the corresponding chapter in Radiopaedia.

Contributor: Rachel Seltman, MD

DOI: https://doi.org/10.18791/nsatlas.v1.03.01.37

References

Hsu C, Kwan G, Lau Q, et al. Rosette-forming glioneuronal tumour: imaging features, histopathological correlation and a comprehensive review of literature. Br J Neurosurg 2012;26:668–673. doi.org/10.3109/02688697.2012.655808.

Schlamann A, von Bueren AO, Hagel C, 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. doi.org/10.1371/journal.pone.0101211.

Osborn AG, Salzman KL, Jhaveri MD. Diagnostic Imaging (3rd ed). Elsevier, Philadelphia, PA; 2016.

Smith AB, Smirniotopoulos JG, Horkanyne-Szakaly I. From the radiologic pathology archives: intraventricular neoplasms: radiologic-pathologic correlation. Radiographics 2013;33:21–43. doi.org/10.1148/rg.331125192.

Zhang J, Babu R, McLendon RE, et al. A comprehensive analysis of 41 patients with rosette-forming glioneuronal tumors of the fourth ventricle. J Clin Neurosci 2013;20:335–341. doi.org/10.1016/j.jocn.2012.09.003.

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