Gliome de bas grade avec altération de MYBL1 : observation d’une nouvelle entité tumorale pédiatrique

Appay, Romain; Tauziède‐Espariat, Arnault; Silva, Karen; Fritih, Radia; Scavarda, Didier; Delteil, Clémence; Varlet, Pascale; Figarella‐Branger, Dominique

doi:10.1016/j.annpat.2020.07.003

Cited by 2 publications

(1 citation statement)

References 9 publications

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“…The tumor spectrum of established LEAT entities is broad and has significantly increased according to the fourth WHO classification update (Table 1; Blümcke et al, 2016;Slegers and Blumcke, 2020;Louis et al, 2021). However, except for the established tumors of GG and DNET, other tumors in LEATs are not yet well-recognized due to their rather low incidences, especially from a single center report with limited cases (Blümcke et al, 2016;Blumcke et al, 2017), and thus they are variably reported in the surgical series of LEATs (Luyken et al, 2003;Wessling et al, 2015;Radhakrishnan et al, 2016;Giulioni et al, 2017;Vogt et al, 2018;Ristić et al, 2020), including angiocentric glioma (AG) (Ni et al, 2015;Wang et al, 2020), papillary glioneuronal tumor (PGNT) (Bridge et al, 2013;Pages et al, 2015;Hou et al, 2019), multinodular and vacuolating neuronal tumor (MVNT) (Gonzalez-Quarante et al, 2018;Pekmezci et al, 2018a;Thom et al, 2018;Choi et al, 2019;Gökçe, 2020), isomorphic astrocytoma/isomorphic diffuse glioma (IDG) (Wefers et al, 2020;Appay et al, 2021), pilocytic astrocytoma (PA) (Blümcke et al, 2016), and sometimes including pleomorphic xanthoastrocytoma (PXA) (Weber et al, 2007), diffuse low-grade gliomas (DLGGs) of diffuse astrocytoma (DA) and oligodendroglioma (d-OT) or oligoastrocytoma (d-OA) (Luyken et al, 2003;Vogt et al, 2018;Ius et al, 2020), and the newly diagnosed entity of "polymorphous low-grade neuroepithelial tumor of the young (PLNTY)" (Huse et al, 2017;Louis et al, 2021). Although shared clinical features are found in these lesions, arguments still exist in the categorization of which tumor entities are true LEATs…”

Section: The Spectrum Of Brain Tumors In Leatmentioning

confidence: 99%

Low-grade epilepsy-associated neuroepithelial tumors: Tumor spectrum and diagnosis based on genetic alterations

et al. 2023

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Brain tumors can always result in seizures when involving the cortical neurons or their circuits, and they were found to be one of the most common etiologies of intractable focal seizures. The low-grade epilepsy-associated neuroepithelial tumors (LEAT), as a special group of brain tumors associated with seizures, share common clinicopathological features, such as seizure onsets at a young age, a predilection for involving the temporal lobe, and an almost benign course, including a rather slow growth pattern and thus a long-term history of seizures. Ganglioglioma (GG) and dysembryoplastic neuroepithelial tumor (DNET) are the typical representatives of LEATs. Surgical treatments with complete resection of tumors and related epileptogenic zones are deemed the optimal way to achieve postoperative seizure control and lifetime recurrence-free survival in patients with LEATs. Although the term LEAT was originally introduced in 2003, debates on the tumor spectrum and the diagnosis or classification of LEAT entities are still confusing among epileptologists and neuropathologists. In this review, we would further discuss these questions, especially based on the updated classification of central nervous system tumors in the WHO fifth edition and the latest molecular genetic findings of tumor entities in LEAT entities.

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Section: The Spectrum Of Brain Tumors In Leatmentioning

confidence: 99%

Low-grade epilepsy-associated neuroepithelial tumors: Tumor spectrum and diagnosis based on genetic alterations

et al. 2023

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Clinical characteristics and detection of MYB-QKI fusions in patients with angiocentric glioma

Li,

Aihemaitiniyazi,

Zhang

et al. 2024

Neurol Sci

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Purpose Angiocentric glioma (AG), a benign tumor identified within the last two decades, was officially included in the 2007 WHO Classification of Tumors of the Central Nervous System, WHO grade I. The tumor is relatively rare, with only approximately 100 cases reported. We aim to complement the characteristics and long-term prognosis of AG, as well as to detect MYB-QKI fusions. Methods The characteristics of all cases collected between 1 March 2009 and 1 March 2023 at the Beijing Sanbo Brain Hospital, Capital Medical University, were summarized and analyzed. Additionally, all fourteen patients were tested for MYB-QKI fusions. Results AG more predominantly occurs in adolescents (median age 16.5-year-old), and commonly presents with drug-resistant epilepsy. AG is frequently localized in the supratentorial regions and only one patient is in the brainstem. Brain parenchyma atrophy, and stalk-like signs can observe in imaging. Pathologically, tumor cells are perivascular pseudorosettes, presenting immunoreactivity for GFAP, S-100, Vimentin, “dot-like” staining for EMA, and low proliferative activity. Focal cortex dysplasia was observed in four patients. Twelve of fourteen (85.7%) patients were found with MYB-QKI fusions. Completely surgical resection typically has a satisfactory prognosis with long-term follow-up. Conclusion AG is a rare benign tumor with a favorable prognosis after complete resection, characterized by refractory epilepsy, frequently occurring in adolescents. MYB-QKI fusions were detected in most AG patients, as a good defining genetic alteration pathologically. The potential presence of focal cortical dysplasia (FCD) may affect the prognosis of epilepsy.

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Gliome de bas grade avec altération de MYBL1 : observation d’une nouvelle entité tumorale pédiatrique

Cited by 2 publications

References 9 publications

Low-grade epilepsy-associated neuroepithelial tumors: Tumor spectrum and diagnosis based on genetic alterations

Low-grade epilepsy-associated neuroepithelial tumors: Tumor spectrum and diagnosis based on genetic alterations

Clinical characteristics and detection of MYB-QKI fusions in patients with angiocentric glioma

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