Papillary glioneuronal tumor

Papillary glioneuronal tumor (PGNT) is a rare, low-grade brain tumor classified by the World Health Organization as a grade I mixed neuronal-glial neoplasm. It predominantly arises in the cerebral hemispheres, most commonly in the temporal lobe, though it can occur in other supratentorial locations. The tumor is characterized by a distinctive biphasic histological architecture featuring pseudopapillary structures lined by a single layer of small, flat glial cells overlying hyalinized vascular cores, intermixed with collections of neurocytes and ganglion cells. PGNT primarily affects the central nervous system and typically presents as a well-circumscribed, cystic mass, sometimes with a mural nodule. Patients most commonly present with seizures, headaches, and symptoms related to increased intracranial pressure such as nausea and vomiting. Visual disturbances, focal neurological deficits, and cognitive changes may also occur depending on the tumor's location and size. Some cases are discovered incidentally on neuroimaging performed for unrelated reasons. The tumor affects both children and adults, with a broad age range at diagnosis, though it appears to have a slight predilection for younger adults. The primary treatment for papillary glioneuronal tumor is surgical resection. Given its generally well-circumscribed nature and low-grade behavior, gross total resection is often achievable and is typically associated with an excellent prognosis and low recurrence rates. In cases where complete resection is not possible, subtotal resection may still provide favorable outcomes, though close follow-up with serial neuroimaging is recommended. The role of adjuvant radiation therapy or chemotherapy is not well established due to the rarity of the tumor, and these modalities are generally reserved for the uncommon cases that demonstrate aggressive behavior or recurrence. Rare instances of malignant transformation have been reported in the literature.

Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Source: openFDA + DailyMed · NDA / BLA labels with structured indications · refreshed weekly

Source: ClinicalTrials.gov · synced daily · phases, status, and PI names normalized at ingest

Source: NPI Registry + PubMed · trial PI roles cross-referenced with ClinicalTrials.gov · ranked by match score (publications + PI activity + community signal)

Source: NORD Rare Disease Centers + NIH Undiagnosed Diseases Network (UDN) · centers verified active within last 12 months

Source: PubMed + NIH RePORTER + openFDA + clinical-journal RSS · last 30 days · disease-tagged at ingest by AI extraction with human QC

Common questions about Papillary glioneuronal tumor