Atypical papillary glioneuronal tumor

Jesús Vaquero1, Santiago Coca

  • 1Department of Neurosurgery, Puerta de Hierro Hospital, Autonomous University, San Martin de Porres, 4, Madrid 28035, Spain. jvaqueroc@telefonica.net

Journal of Neuro-Oncology
|February 8, 2007
PubMed

Insights

A rare papillary glioneuronal tumor (PGNT) with aggressive features showed favorable outcomes after surgery and radiotherapy. This case highlights that high-grade histological findings in PGNT do not always predict poor prognosis.

Area of Science:

  • Neuro-oncology
  • Neurosurgery
  • Pathology

Background:

  • Papillary glioneuronal tumors (PGNTs) are rare primary brain tumors.
  • While typically considered low-grade, some PGNTs exhibit atypical features.
  • Understanding the behavior of atypical PGNTs is crucial for treatment planning.

Observation:

  • A 34-year-old male presented with a 3-month history of headaches.
  • MRI revealed a large cystic tumor in the right frontoparietal lobe.
  • Pathological examination identified a papillary glioneuronal tumor with mitotic activity and a 15% Ki-67 index.

Findings:

  • The patient underwent radical surgery followed by radiotherapy.
  • Five years post-treatment, the patient remains symptom-free with no evidence of recurrence or residual tumor.
  • This outcome suggests a potentially indolent course despite aggressive histological markers.

Implications:

  • This case supports the existence of an atypical PGNT variant with high proliferative activity.
  • Histological indicators of malignancy in PGNTs may not correlate with short-term unfavorable outcomes.
  • Aggressive treatment modalities can be effective even in PGNTs with concerning pathological features.