Recurrent Papillary Glioneuronal Tumor

World Neurosurg. 2019 Aug:128:127-130. doi: 10.1016/j.wneu.2019.04.266. Epub 2019 May 9.


Background: Papillary glioneuronal tumors (PGNTs) are rare World Health Organization grade I neoplasms that are characterized by a benign course and excellent response to surgical resection. A few reports exist of tumors with more aggressive clinical and histologic features. In this report we detail the case of an unusually aggressive PGNT in a 67-year-old woman.

Case description: The patient had a 3-year history of seizures and was diagnosed with a frontoparietal mass on imaging. She underwent subtotal resection with a histologic diagnosis of PGNT. Less than a year after surgery, the patient experienced recurrence of disease and underwent reresection and adjuvant radiation treatment. The patient's disease continued to progress despite radiation treatment, so adjuvant temozolomide was initiated. Molecular testing was performed and revealed a TERT promotor mutation, an FGFR3-TACC3 oncogenic fusion, and a copy number loss in CDKN2A/CDKN2B.

Conclusions: PGNTs, while typically benign, can rarely recur after surgery. Molecular testing should be performed on all PGNTs to help possibly identify more aggressive tumors and potentially reveal novel treatment options.

Keywords: Papillary glioneuronal tumor; Recurrent tumor.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Brain Neoplasms / diagnostic imaging
  • Brain Neoplasms / genetics
  • Brain Neoplasms / pathology*
  • Brain Neoplasms / therapy
  • Cyclin-Dependent Kinase Inhibitor Proteins / genetics
  • DNA Copy Number Variations
  • Female
  • Glioma / diagnostic imaging
  • Glioma / genetics
  • Glioma / pathology*
  • Glioma / therapy
  • Humans
  • Magnetic Resonance Imaging
  • Microtubule-Associated Proteins / genetics
  • Mutation
  • Neoplasm Recurrence, Local / diagnostic imaging
  • Neoplasm Recurrence, Local / pathology*
  • Neoplasm Recurrence, Local / therapy
  • Neurosurgical Procedures
  • Oncogene Fusion / genetics
  • Promoter Regions, Genetic / genetics
  • Radiotherapy
  • Receptor, Fibroblast Growth Factor, Type 3 / genetics
  • Somatosensory Cortex / diagnostic imaging
  • Somatosensory Cortex / pathology*
  • Somatosensory Cortex / surgery
  • Telomerase / genetics
  • Tomography, X-Ray Computed


  • Cyclin-Dependent Kinase Inhibitor Proteins
  • Microtubule-Associated Proteins
  • TACC3 protein, human
  • FGFR3 protein, human
  • Receptor, Fibroblast Growth Factor, Type 3
  • TERT protein, human
  • Telomerase