Recurrent Papillary Glioneuronal Tumor

Eric A. Goethe, Michael Youssef, Akash J. Patel, Ali Jalali, J. Clay Goodman, Jacob J. Mandel

Research output: Contribution to journalArticlepeer-review

1 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)127-130
Number of pages4
JournalWorld neurosurgery
Volume128
DOIs
StatePublished - Aug 2019
Externally publishedYes

Keywords

  • Papillary glioneuronal tumor
  • Recurrent tumor

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

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