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Main text Ganglioglioma is a WHO grade I glioneuronal tumor composed of neoplastic glial cells and dysmorphic ganglion cells [13]. They account for up to 2% of central nervous system neoplasms, typically occur in the temporal lobes, and are frequently epileptogenic [7]. A majority have alterations that activate the mitogen-activated protein kinase (MAPK) signaling pathway. Following complete resection, recurrence or anaplastic progression has been reported in rare cases [1, 5, 7]. Anaplastic ganglioglioma is a WHO grade III neoplasm with a poor prognosis that may either arise de novo or secondary to the malignant transformation of a previous ganglioglioma [10, 11, 14]. We present the case of a 71-year-old man with a history of a right temporal mass that was resected and diagnosed as “ganglioneuroma” according to pathology reports from approximately 30 years previous. The patient presented to our institution with recurrent seizures. Neuroimaging revealed an enhancing mesial right temporal lobe mass, invading the right suprasellar and ambient cisterns and the right cerebral peduncle medial to the previous resection cavity. Interval imaging 5months later showed increasing size and development of ring enhancement (Fig. 1a). He underwent a right pterional craniotomy for tumor debulking. Hematoxylin and eosin stained tumor sections showed that the dominant pattern featured a highly cellular, compact neoplasm with pleomorphic spindled cells with eosinophilic, fibrillary cytoplasm arranged in a sheetlike or fascicular growth pattern, with focal nodules of large cells with bizarre pleomorphic nuclei (Fig. 1b). Some tumor cells showed abundant amphophilic cytoplasm and enlarged nuclei with vesicular chromatin, prominent nucleoli, and occasional binucleation (Fig. 1b, inset). Focal necrosis with peripheral macrophage accumulation was observed. The mitotic index was 5 mitoses per 10 highpower fields. In some regions, the tumor transitioned to lower cellularity with haphazard clusters of dysmorphic ganglion-like cells, eosinophilic granular bodies, and blood vessels with perivascular lymphocytes (Fig. 1c); abnormal cell body positivity for neurofilament protein was also encountered (Figs. 1c, inset). Other low-cellularity regions showed a nodular growth pattern and numerous vacuolecontaining ganglion-like cells, which were OLIG2 positive and NeuN negative (Fig. 1d-f). Other immunohistochemical stains showed that the cellular, pleomorphic component was GFAP positive, with scattered cells positive for neuron-specific enolase, and the Ki67 index was approximately 10%. ATRX expression was retained in tumor nuclei in both components, p53 staining was positive in only scattered tumor nuclei, and an IDH1-R132H stain was negative for mutant protein expression. Extravascular CD34 staining was seen in the low-grade gangliogliomalike component, highlighting occasional cells with ramified branching processes. CD34 staining in the high-grade component only highlighted small vessels, and was negative for extravascular staining. Collagen IV was negative for tumor cell associated staining in all components, highlighting only vessels and a focal fibrotic nodule. The features supported a diagnosis of a high grade glioneuronal tumor, most consistent with anaplastic ganglioglioma,

An unusual recurrent high-grade glioneuronal tumor with MAP2K1 mutation and CDKN2A/B homozygous deletion