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Published on: May 17, 2024
Atypical papillary glioneuronal tumor
1Department of Neurosurgery, Puerta de Hierro Hospital, Autonomous University, San Martin de Porres, 4, Madrid 28035, Spain. jvaqueroc@telefonica.net
Abstract:
We describe a 34-year-old man who presented with headaches for about 3 months. Magnetic Resonance Imaging (MRI) revealed a large cystic tumor, involving the right frontoparietal lobe region. Pathological study revealed a papillary glioneuronal tumor (PGNT) with mitotic activity and a Ki-67 labeling index of approximately 15%. Five years after radical surgery and radiotherapy, the patient is symptom-free, without tumor recurrence or residual tumor. This case supports the existence of an atypical variant of PGNT, with mitotic activity and high proliferative index, and suggests that in these tumors, histological findings of malignity not necessarily indicate a short-term unfavorable behavior.
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.
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