Imaging features of rosette-forming glioneuronal tumours (RGNTs): A Series of seven cases

Eur Radiol. 2016 Jan;26(1):262-70. doi: 10.1007/s00330-015-3808-y. Epub 2015 May 28.

Abstract

Objective: Rosette-forming glioneuronal tumours (RGNTs) are a recently described, rare, distinct nosological entity of the glioneuronal family. We describe imaging findings (CT and MRI) in seven patients with RGNTs.

Materials and methods: This retrospective study includes seven RGNT patients (4 male, 3 female; age range: 7-42 years; mean age: 25 years) diagnosed and treated at our institute. MR studies were performed on 3 T and 1.5-T clinical MR systems. All patients were reviewed by two experienced neuroradiologists and imaging findings were tabulated.

Results: Five tumours were located in the posterior fossa, and two were in the pineal region. One of the tumours demonstrated multiple satellite lesions, which involved the midbrain, pons, medulla as well as the cervical cord. Tumours located in the pineal region compressed the 3rd ventricle/aqueduct and extended below the tentorium cerebelli. All the tumours demonstrated enhancement, and susceptibility was evident in six of the seven patients. CSF dissemination was present in two patients.

Conclusion: RGNTs are usually solid-cystic tumours and frequently demonstrate peripheral/heterogeneous enhancement upon post-contrast study. Haemorrhage is a common feature which may not be evident on CT. Cerebrospinal fluid (CSF) dissemination is a feature and appropriate imaging should be performed whenever an RGNT is suspected.

Key points: CT and MRI findings of seven RGNT cases were retrospectively reviewed. RGNTs are predominantly posterior fossa tumours. RGNTs are typically T1 hypointense and T2 hyperintense. Haemorrhage and peripheral/heterogeneous enhancement are common features of RGNTs. CSF dissemination is a feature of RGNTs and requires appropriate imaging.

Keywords: CSF dissemination; Haemorrhage; Histopathology; Imaging; Rosette forming glioneuronal tumour.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Adult
  • Cerebral Ventricle Neoplasms / diagnosis*
  • Child
  • Female
  • Fourth Ventricle / diagnostic imaging
  • Fourth Ventricle / pathology
  • Ganglioglioma / diagnosis*
  • Humans
  • Magnetic Resonance Imaging / methods*
  • Male
  • Retrospective Studies
  • Spinal Cord / diagnostic imaging
  • Spinal Cord / pathology
  • Tomography, X-Ray Computed / methods*
  • Young Adult