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Article Abstract

Papillary glioneuronal tumors (PGNTs) are classified by the World Health Organization (WHO) as Grade I neoplasms, with only sporadic reports of anaplastic variants demonstrating aggressive clinical behavior and distinct histopathological characteristics. This study presents two cases of anaplastic PGNT, including one that ultimately progressed to glioblastoma (WHO Grade IV). The first case involved a 47-year-old female patient without a history of seizures. Magnetic resonance imaging (MRI) revealed an irregular mass containing multiple cysts and a mural nodule in the left parietal lobe. Histopathological examination confirmed the diagnosis of anaplastic PGNT. Nineteen months later, follow-up brain MRI demonstrated a recurrent mass at the prior surgical site. During the second resection, histological analysis identified glioblastoma arising from the glial component of the original tumor. The second case concerned a 7-year-old boy presenting with progressive headache. MRI showed a solid-cystic mass in the right frontal lobe accompanied by marked peritumoral edema. Postoperative pathological examination revealed anaplastic PGNT with extensive necrosis. MRI characteristics including prominent peritumoral edema, ring-enhancing cyst walls, restricted diffusion, and elevated lipid/lactate peaks may predict the aggressive nature of anaplastic PGNT. Furthermore, this case series suggests that anaplastic PGNTs harbor malignant potential to transform into more aggressive neoplasms.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC12381771PMC
http://dx.doi.org/10.3389/fonc.2025.1598058DOI Listing

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Papillary glioneuronal tumors (PGNTs) are classified by the World Health Organization (WHO) as Grade I neoplasms, with only sporadic reports of anaplastic variants demonstrating aggressive clinical behavior and distinct histopathological characteristics. This study presents two cases of anaplastic PGNT, including one that ultimately progressed to glioblastoma (WHO Grade IV). The first case involved a 47-year-old female patient without a history of seizures.

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Objective: Papillary glioneuronal tumors (PGNT) is well-recognized in the literature, although reports usually have not attempted a critical analysis of their characteristics. We report two PGNT and perform a comprehensive review of the published cases, aiming to clarify their clinical, imaging and histopathological features.

Material And Methods: We have reviewed all glioneuronal tumors diagnosed in our laboratory over the last 10 years and found 2 cases PGNTs along with their clinical, imaging and surgical data.

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The fourth edition of the WHO classification of tumours of the CNS was published in 2007. Six new entities were codified: angiocentric glioma (AG); papillary glioneuronal tumour (PGNT); rosette-forming glioneuronal tumour of the fourth ventricle (RGNT); papillary tumour of the pineal region (PTPR); spindle cell oncocytoma of the adenohypophysis (SCO); and pituicytoma. Furthermore, six histological variants of well-known brain tumours have been added, partially because they show different biological behaviour and/or prognosis: pilomyxoid astrocytoma; atypical choroid plexus papilloma; medulloblastoma with extensive nodularity; anaplastic medulloblastoma; extraventricular neurocytoma; non-specific variant of dysembryoplastic neuroepithelial tumour (DNT).

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Papillary glioneuronal tumor: not always a benign tumor?

Clin Neuropathol

February 2009

Service Central d'Anatomie et de Cytologie Pathologiques, APHP, Hôpital Lariboisière-Université Paris VII, France.

Papillary glioneuronal tumor (PGNT) is a variant of ganglioglioma, characterized by a pseudopapillary structure with a single pseudostratified layer of small, cuboidal, GFAP-positive cells around hyalinized blood vessels. To date, less than 30 cases have been described with a usually benign course. We report two additional cases: a clinically, radiologically and histopathologically typical tumor in a 38-year-old man and an atypical tumor with histopathological features of anaplasia in a 74-year-old woman.

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