Paediatric-type diffuse low-grade gliomas: a clinically and biologically distinct group of tumours with a favourable outcome

Fabbri, Viscardo Paolo; Caporalini, Chiara; Asioli, Sofia; Buccoliero, Annamaria

doi:10.32074/1591-951x-828

Cited by 15 publications

(14 citation statements)

References 29 publications

(53 reference statements)

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“…Diffuse low‐grade gliomas, MAPK pathway‐altered are described throughout the neuroaxis, and most commonly in the cerebral hemispheres. On imaging, diffuse low‐grade gliomas, MAPK pathway‐altered, may have a more diffuse pattern than pilocytic astrocytomas, but there are heterogeneous imaging findings reported given the broad spectrum of histological features 54,55 . Representative cases from two different patients are shown in Fig.…”

Section: Specific Molecular and Imaging Featuresmentioning

confidence: 99%

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The 2021 WHO Classification for Gliomas and Implications on Imaging Diagnosis: Part 2—Summary of Imaging Findings on Pediatric‐Type Diffuse High‐Grade Gliomas, Pediatric‐Type Diffuse Low‐Grade Gliomas, and Circumscribed Astrocytic Gliomas

Park

Vollmuth

Foltyn-Dumitru

et al. 2023

Magnetic Resonance Imaging

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The fifth edition of the World Health Organization (WHO) classification of central nervous system tumors published in 2021 advances the role of molecular diagnostics in the classification of gliomas by emphasizing integrated diagnoses based on histopathology and molecular information and grouping tumors based on genetic alterations. This Part 2 review focuses on the molecular diagnostics and imaging findings of pediatric-type diffuse high-grade gliomas, pediatric-type diffuse low-grade gliomas, and circumscribed astrocytic gliomas. Each tumor type in pediatric-type diffuse high-grade glioma mostly harbors a distinct molecular marker. On the other hand, in pediatric-type diffuse low-grade gliomas and circumscribed astrocytic gliomas, molecular diagnostics may be extremely complicated at a glance in the 2021 WHO classification. It is crucial for radiologists to understand the molecular diagnostics and imaging findings and leverage the knowledge in clinical practice. Evidence Level: 3 Technical Efficacy: Stage 3.

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Section: Specific Molecular and Imaging Featuresmentioning

confidence: 99%

“…On imaging, diffuse low-grade gliomas, MAPK pathway-altered, may have a more diffuse pattern than pilocytic astrocytomas, but there are heterogeneous imaging findings reported given the broad spectrum of histological features. 54,55 Representative cases from two different patients are shown in Fig. 11.…”

Section: Polymorphous Low-grade Neuroepithelial Tumormentioning

confidence: 99%

The 2021 WHO Classification for Gliomas and Implications on Imaging Diagnosis: Part 2—Summary of Imaging Findings on Pediatric‐Type Diffuse High‐Grade Gliomas, Pediatric‐Type Diffuse Low‐Grade Gliomas, and Circumscribed Astrocytic Gliomas

Park

Vollmuth

Foltyn-Dumitru

et al. 2023

Magnetic Resonance Imaging

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“…The aforementioned genetic alterations represent potential therapeutic targets, and the use of agents such as fibroblast growth factor (FGFR) or pan-RAF inhibitors should be considered in cases of residual or progressive disease. 2,63 Pediatric-Type Diffuse High-Grade Gliomas…”

Section: Pediatric-type Diffuse Low-grade Gliomasmentioning

confidence: 99%

“…61 62 Polymorphous low-grade neuroepithelial tumor of the young and diffuse low-grade glioma, MAPK pathway-altered are partially cystic tumors found in the cerebral hemispheres and defined my MAPK pathway alterations. 63 Similar to other pLGG, the initial management of pediatric-type diffuse LGG consists of MSR, which is curative in the majority of cases. The aforementioned genetic alterations represent potential therapeutic targets, and the use of agents such as fibroblast growth factor (FGFR) or pan-RAF inhibitors should be considered in cases of residual or progressive disease.…”

Section: Pediatric-type Diffuse Low-grade Gliomasmentioning

confidence: 99%

Brain Tumors in Adolescents and Young Adults: A Review

Rousseau,

Bennett,

Lim-Fat

2023

Semin Neurol

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Brain tumors account for the majority of cancer-related deaths in adolescents and young adults (AYAs), defined as individuals aged 15 to 39. AYAs constitute a distinct population in which both pediatric- and adult-type central nervous system (CNS) tumors can be observed. Clinical manifestations vary depending on tumor location and often include headaches, seizures, focal neurological deficits, and signs of increased intracranial pressure. With the publication of the updated World Health Organization CNS tumor classification in 2021, diagnoses have been redefined to emphasize key molecular alterations. Gliomas represent the majority of malignant brain tumors in this age group. Glioneuronal and neuronal tumors are associated with longstanding refractory epilepsy. The classification of ependymomas and medulloblastomas has been refined, enabling better identification of low-risk tumors that could benefit from treatment de-escalation strategies. Owing to their midline location, germ cell tumors often present with oculomotor and visual alterations as well as endocrinopathies. The management of CNS tumors in AYA is often extrapolated from pediatric and adult guidelines, and generally consists of a combination of surgical resection, radiation therapy, and systemic therapy. Ongoing research is investigating multiple agents targeting molecular alterations, including isocitrate dehydrogenase inhibitors, SHH pathway inhibitors, and BRAF inhibitors. AYA patients with CNS tumors should be managed by multidisciplinary teams and counselled regarding fertility preservation, psychosocial comorbidities, and risks of long-term comorbidities. There is a need for further efforts to design clinical trials targeting CNS tumors in the AYA population.

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“…Antonelli and Poliani 1 discuss the current classification of diffuse gliomas in adults, including the role of ancillary molecular testing in identifying distinct tumor types (e.g., EGFR amplification, TERT promoter mutation, and +7/10 in glioblastoma, IDH wildtype) and grading (homozygous CDKN2A/B homozygous deletion in IDH mutant astrocytomas). Fabbri et al 2 cover the pediatric counterpart of diffuse low-grade gliomas which include four distinct histo-molecular entities, namely diffuse astrocytoma MYB or MYBL1 altered, angiocentric glioma, polymorphous low-grade neuroepithelial tumour of the young (PLNTY) and diffuse low-grade glioma MAPK pathway-altered. Gianno et al 3 focus on the issue of pediatric diffuse high grade gliomas giving practical information for their diagnosis discussing advantages and limits of the multiple molecular tests utilized to define the single entities of this complex tumor family.…”

mentioning

confidence: 99%