Despite mostly indolent course and favorable postoperative outcome long-term follow-up studies are needed to identify the most appropriate therapeutic strategies to minimize surgical morbidity and neurologic injury in patients with RGNT.
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| http://dx.doi.org/10.1002/ccr3.4355 | DOI Listing |
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A glioneuronal tumor with neurocytic rosettes harboring FGFR1 internal tandem duplication - A report of a unique case.
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Department of Neurosurgery and Neurooncology, 1st Medical Faculty, Charles University, Military University Hospital Prague, Prague, Czech Republic.
Rosette-forming glioneuronal tumors (RGNTs) with FGFR1 tyrosine kinase domain internal tandem duplication (FGFR1 ITD) is exceedingly rare, with only a few cases reported in the literature. Hereby we present a case of a tumor with RGNT morphology occurring in area of septum pellucidum of 43-year-old male. The tumor showed FGFR1 ITD, no PIK3CA, PIK3R1 or NF1 alterations and inconclusive methylation profile with match for class of "low-grade glial/glioneuronal/neuroepithelial tumors".
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Biomedicines
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Department of Neurosurgery, Zhongshan Hospital, Fudan University, Shanghai 200030, China.
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Department of Neurosurgery, HClinic, Ramallah, Palestine, Palestine.
Introduction: The association between primary brain tumors, such as glioneuronal tumors, with autosomal-dominant polycystic kidney disease (ADPKD) remains poorly understood, with only two cases reported excluding this one. This case of an ADPKD patient diagnosed with a rosette-forming glioneuronal tumor highlights an exceptionally rare potential association warranting further investigation.
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