From a Polymorphous Low-Grade Neuroepithelial Tumor to a Glioblastoma in an Adult Patient with FGFR3-TACC3 Fusion: A Case Report and Literature Review of the Molecular Profile.
Simple Summary: Polymorphous low-grade neuroepithelial tumor (PLNTY) is a recently recognized low-grade epilepsy-associated neoplasm characterized by MAPK pathway alterations that frequently involve FGFR3, but malignant transformation has not been well documented. We report a rare adult case of PLNT...
| Publicado en: | Current Oncology Vol. 31; no. 3; pp. 165 - 175 |
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| Autores principales: | , , , , , , , , , , |
| Formato: | Journal Article |
| Publicado: |
MDPI
Mar2026
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| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=192598820&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 192598820 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 11980052 5EKK jtl: Current Oncology issn: 11980052 maglogo: N pubinfo: dt: Mar2026 vid: 31 iid: 3 pid: 97109 pub: MDPI artinfo: ui: 192598820 10.3390/curroncol33030165 192598820 ppf: 165 ppct: 10 formats: fmt: – @attributes: type: T – @attributes: type: P tig: atl: From a Polymorphous Low-Grade Neuroepithelial Tumor to a Glioblastoma in an Adult Patient with FGFR3-TACC3 Fusion: A Case Report and Literature Review of the Molecular Profile. aug: au: Gurrieri, Lorena Riva, Nada Tomassini, Alessia Ghigi, Giulia Naccarato, Maurizio Cenni, Patrizia Bartolini, Daniela Cavatorta, Chiara Tosatto, Luigino Dall'Agata, Monia Ridolfi, Laura affil: Clinical and Experimental Oncology, Immunotherapy, Rare Cancers and Biological Resource Center, IRCCS Istituto Romagnolo per lo Studio dei Tumori (IRST) "Dino Amadori", Via P. Maroncelli 40, 47014 Meldola, Italy sug: ab: Simple Summary: Polymorphous low-grade neuroepithelial tumor (PLNTY) is a recently recognized low-grade epilepsy-associated neoplasm characterized by MAPK pathway alterations that frequently involve FGFR3, but malignant transformation has not been well documented. We report a rare adult case of PLNTY progressing to glioblastoma, highlighting the molecular evolution of the tumor. Histopathological evaluation and immunohistochemistry were performed at initial diagnosis and at recurrence, and targeted next-generation sequencing was used to assess genomic alterations, with particular focus on FGFR3 status. The primary lesion showed morphological and molecular features consistent with PLNTY, including FGFR3 alteration. At recurrence, the tumor demonstrated high-grade histological features and additional molecular changes consistent with glioblastoma, suggesting clonal evolution. This case underscores the importance of comprehensive molecular profiling in low-grade glioneuronal tumors. Further studies are needed to clarify the mechanisms driving malignant transformation and to determine whether FGFR3 alterations may represent potential therapeutic targets or biomarkers of progression. From an epidemiological perspective, polymorphous low-grade neuroepithelial tumor (PLNTY) represents a small proportion of brain tumors encountered in epilepsy surgery series. Their rarity and relatively recent recognition likely contribute to underdiagnosis and poor prognosis. In terms of histopathological features, they are similar to oligodendrogliomas. Molecular analyses can be used to show the fusion between fibroblast growth factor receptor (FGFR3) and transforming acidic coiled coil (TACC) proteins, which most commonly results in progression towards glioblastoma (GBM). We report a case of a 62-year-old man who underwent left frontal craniotomy to remove a frontal mass. Histologically, the glial lesion consisted of elements associated with oligodendroglia-like features. Immunohistochemistry was positive for glial fibrillary acidic protein (GFAP), oligodendrocyte transcription factor 2 (OLIG2), and α-thalassemia X-linked mental retardation syndrome (ATRX) nuclear expression, but negative for isocitrate dehydrogenase 1 (IDH1) and BRAF-V600E. Next-generation sequencing showed the FGFR-TACC3 fusion, and taken together, these findings supported the final diagnosis of PLNTY. During follow-up, the patient underwent a second neurosurgery, where histological evaluation indicated a GMB. This article presents clinical and radiological data, morphology, immunohistochemistry, molecular features, and treatment to enhance the clinical and pathological understanding of PLNTY with FGFR3-TACC3 fusion for all professionals involved in medical decisions. pubtype: Academic Journal doctype: Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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