Neuroradiological features of the polymorphous low-grade neuroepithelial tumor of the young: five new cases with a systematic review of the literature

Kurokawa, Mariko; Kurokawa, Ryo; Capizzano, Arístides A.; Baba, Akira; Ota, Yoshiaki; Pinarbasi, Emile; Johnson, Timothy D.; Srinivasan, Ashok; Moritani, Toshio

doi:10.1007/s00234-021-02879-5

Cited by 27 publications

(36 citation statements)

References 20 publications

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“… 8 Regarding the stalk‐like sign, we assume that it reflects several different pathologies, such as tumor infiltration along with the vessels extending from near the brain surface toward the ventricle, peritumoral gliosis caused by long‐standing tumor existence or seizure‐induced oxidative stress, and coexisting FCD, as is the case with polymorphous low‐grade neuroepithelial tumor of the young. 46 Indeed, the coexistence of AG and FCD was pathologically proven in three cases, 25 , 43 including two cases with stalk‐like signs. FCD may cause longstanding seizure/epilepsy, and conversely, a long seizure/epilepsy history may cause regional atrophy and brain parenchymal degeneration around the tumor.…”

Section: Discussionmentioning

confidence: 96%

Neuroimaging features of angiocentric glioma: A case series and systematic review

Kurokawa

Baba

Pinarbasi

et al. 2022

Journal of Neuroimaging

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Background and Purpose: Angiocentric gliomas (AGs) are epileptogenic low-grade gliomas in young patients. We aimed to investigate the MRI findings of AGs and systematically review previous publications and three new cases. Methods:We searched PubMed, Elsevier's abstract and citation database, and Embase databases and included 50 patients with pathologically proven AGs with analyzable preoperative MRI including 3 patients from our institution and 47 patients from 38 publications (median age, 13 years [range, 2-83 years]; 35 men). Two board-certified radiologists reviewed all images. The relationships between seizure/epilepsy history and MRI findings were statistically analyzed. Moreover, clinical and imaging differences were evaluated between supratentorial and brainstem AGs.Results: Intratumoral T1-weighted high-intensity areas, stalk-like signs, and regional brain parenchymal atrophy were observed in 23 out of 50 (46.0%), 10 out of 50 (20.0%), and 14 out of 50 (28.0%) patients, respectively. Intratumoral T1-weighted high-intensity areas were observed significantly more frequently in patients with stalk-like signs (positive, 9/10 vs. negative, 14/40, p = .0031) and regional atrophy (13/14 vs. 10/36, p = .0001).There were significant relationships between the length of seizure/epilepsy history and presence of intratumoral T1-weighted high-intensity area (median 3 years vs. 0.5 years, p = .0021), stalk-like sign (13.5 vs. 1 year, p < .0001), and regional atrophy (14 vs. 0.5 years, p < .0001). Patients with brainstem AGs (n = 7) did not have a seizure/epilepsy history and were significantly younger than those with supratentorial AGs (median, 5 vs. 13 years, p < .0001, respectively).Conclusions: Intratumoral T1-weighted high-intensity areas, stalk-like signs, and regional brain atrophy were frequent imaging features in AG. We also found that affected age was different between supratentorial and brainstem AGs.

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Section: Discussionmentioning

confidence: 96%

Neuroimaging features of angiocentric glioma: A case series and systematic review

Kurokawa

Baba

Pinarbasi

et al. 2022

Journal of Neuroimaging

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“…Therefore, gross total resection of the tumor may not be enough, and enlarged resection of the tumor with the epileptic zone (EZ) is required. FCD is known to coexist with LEATs (29), and nowadays, an increasing number of studies reported the coexistence of PLNTY and FCD (5,8,30). The double pathology of PLNTY may be more frequent in cases with long seizure duration and drugrefractory epilepsy (30).…”

Section: Discussionmentioning

confidence: 99%

“…FCD is known to coexist with LEATs (29), and nowadays, an increasing number of studies reported the coexistence of PLNTY and FCD (5,8,30). The double pathology of PLNTY may be more frequent in cases with long seizure duration and drugrefractory epilepsy (30). In our study, the peritumoral cortex was obtained in 2 patients (cases a, b) with ER, and peritumoral FCD was observed in case a with drug-refractory epilepsy, which was located in the occipital lobe.…”

Section: Discussionmentioning

confidence: 99%

Clinical, Radiological, Pathological Features and Seizure Outcome With Surgical Management of Polymorphous Low-Grade Neuroepithelial Tumor of the Young Associated With Epilepsy

et al. 2022

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ObjectivePolymorphous low-grade neuroepithelial tumor of the young (PLNTY) is a novel distinct epileptogenic neoplasm, and its clinical, imaging, histopathological, and molecular features were already known in the existing literature. We aimed to analyze the surgical management of PLNTY combined with these known characteristics.MethodsEight patients underwent surgical treatment in our center between December 2017 and December 2020, and the postoperative pathology was diagnosed as PLNTY. Their clinical data, imaging, pathological, molecular characteristics, and seizure outcome were retrospectively analyzed. Follow-up evaluations and a literature review were performed.ResultsThe 8 patients included 1 woman and 7 men, aged between 5 and 51 years old (mean = 31.6, median = 29). The preoperative symptoms of all 8 cases were seizures. Four tumors were situated in the temporal lobes, and one of the four extratemporal tumors was in the occipital lobe and three were in the frontal lobe. Enlarged and gross total resections were performed in 2 cases and the other 6 cases, respectively. All cases exhibited intense labeling of CD34, and absence of 1p/19q codeletion and IDH1 or IDH2 mutation. B-Raf proto-oncogene (BRAF) V600E mutation was presented in 4 (66.7%) of 6 detected cases. The postoperative seizure outcome of Engel class I was achieved in 6 cases (75%).ConclusionPLNTY represents distinctive histologic, immunophenotypic and biomolecular features, and has high epileptogenicity. Early surgical intervention and enlarged resection of PLNTY associated with epilepsy will help to improve the postoperative seizure-free rate.

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“…Polymorphous low-grade neuroepithelial tumor of the young (PLNTY) is also a WHO grade 1 tumor associated with seizures [49,50]. The PLNTY is characterized by alterations of the mitogen-activated protein kinase-pathway (MAPK pathway) such as a mutation of the gene for the rapid accelerated fibrosarcoma oncogene type B at position 600 (BRAF V600E) or even a gene fusion involving the gene for the fibroblast growth factor receptor 2 or 3 (FGFR2 or FGFR3) in combination with typical histological features, such as oligodendroglioma-like areas, a diffuse growth pattern, as well as the expression of cluster of differentiation 34 (CD34) and oligodendrocyte transcription factor 2 (OLIG2) [50][51][52][53].…”

Section: Pediatric-type Diffuse Low-grade Gliomasmentioning

confidence: 99%

“…The PLNTY is characterized by alterations of the mitogen-activated protein kinase-pathway (MAPK pathway) such as a mutation of the gene for the rapid accelerated fibrosarcoma oncogene type B at position 600 (BRAF V600E) or even a gene fusion involving the gene for the fibroblast growth factor receptor 2 or 3 (FGFR2 or FGFR3) in combination with typical histological features, such as oligodendroglioma-like areas, a diffuse growth pattern, as well as the expression of cluster of differentiation 34 (CD34) and oligodendrocyte transcription factor 2 (OLIG2) [50][51][52][53]. Despite the median age of 15.5 years [49], several patients with PLNTY up to the 6th decade of life have been reported [49,54,55]. Except for one tumor occurring in the third ventricle [52], all tumors described so far are supratentorial cortical or subcortical tumors with most cases localized within the temporal lobes [49,54,55].…”

Section: Pediatric-type Diffuse Low-grade Gliomasmentioning

confidence: 99%

Adult-type and Pediatric-type Diffuse Gliomas

et al. 2023

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The classification of diffuse gliomas into the adult type and the pediatric type is the new basis for the diagnosis and clinical evaluation. The knowledge for the neuroradiologist should not remain limited to radiological aspects but should be based additionally on the current edition of the World Health Organization (WHO) classification of tumors of the central nervous system (CNS). This classification defines the 11 entities of diffuse gliomas, which are included in the 3 large groups of adult-type diffuse gliomas, pediatric-type diffuse low-grade gliomas, and pediatric-type diffuse high-grade gliomas. This article provides a detailed overview of important molecular, morphological, and clinical aspects for all 11 entities, such as typical genetic alterations, age distribution, variability of the tumor localization, variability of histopathological and radiological findings within each entity, as well as currently available statistical information on prognosis and outcome. Important differential diagnoses are also discussed.

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Neuroradiological features of the polymorphous low-grade neuroepithelial tumor of the young: five new cases with a systematic review of the literature

Cited by 27 publications

References 20 publications

Neuroimaging features of angiocentric glioma: A case series and systematic review

Neuroimaging features of angiocentric glioma: A case series and systematic review

Clinical, Radiological, Pathological Features and Seizure Outcome With Surgical Management of Polymorphous Low-Grade Neuroepithelial Tumor of the Young Associated With Epilepsy

Adult-type and Pediatric-type Diffuse Gliomas

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