What is Papillary Glioneuronal Tumour (PGNT)?
Papillary Glioneuronal Tumour (PGNT) is a rare, low-grade central nervous system (CNS) tumour characterised by a mix of glial and neuronal components.
Causes
The exact cause of PGNT remains unknown. However, genetic alterations, including PRKCA gene fusions, have been identified in some cases. No environmental risk factors have been linked to its development.
Signs and Symptoms
Common symptoms of Papillary Glioneuronal Tumour include:
- Seizures
- Headaches
- Focal neurological deficits (e.g., weakness, numbness, or difficulty with coordination)
Diagnosis
PGNT is diagnosed through:
- Imaging studies (MRI): Typically revealing well-circumscribed, cystic lesions with solid components.
- Histopathological examination: Confirming papillary structures with both glial and neuronal components.
Treatment
Treatment options for PGNT include:
- Surgery: The primary treatment, involving tumour removal along with affected surrounding tissue.
- Radiation therapy: Rarely required but may be considered for recurrent or residual tumours.
- Chemotherapy: Used in rare cases where the tumour is advanced or spreading.
- Targeted therapy: Experimental treatments being evaluated in clinical studies.
Prognosis
PGNT has an excellent prognosis:
- 10-year survival rate exceeds 90% when complete surgical removal is achieved.
- Recurrence is rare, and malignant transformation is extremely uncommon.
You can help us with your donation:
Without a Ribbon is a charity that works hard to aid those who suffer from rare cancers. You can help our cause in a variety of ways:
Donations — Without a Ribbon is grateful when we receive every donation. Giving to Without a Ribbon helps us to provide ongoing support, organise the annual gathering and subsidise the costs of our Warriors attending these conferences.
Sponsorship — If you wish to sponsor our charity, please contact us using this form.
Volunteering — We are always looking for volunteers to help with different aspects of running our charity. So, if you are looking for volunteer work, please feel free to contact us.