Paediatric-type diffuse high-grade gliomas in the 5th CNS WHO Classification

Gianno, Francesca; Giovannoni, Isabella; Cafferata, Barbara; Diomedi-Camassei, Francesca; Minasi, Simone; Barresi, Sabina; Buttarelli, Francesca Romana; Alesi, Viola; Cardoni, Antonello; Antonelli, Manila; Puggioni, Chiara; Colafati, Giovanna Stefania; Carai, Andrea; Vinci, Maria; Mastronuzzi, Angela; Miele, Evelina; Alaggio, Rita; Giangaspero, Felice; Rossi, Sabrina

doi:10.32074/1591-951x-830

Cited by 20 publications

(24 citation statements)

References 65 publications

(114 reference statements)

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“…The tumor family defined as “pediatric-type diffuse high-grade gliomas” represents one of the main changes in the fifth WHO Classification of CNS Tumors ( WHO Classification of Tumours Editorial Board, 2021 ; Gianno et al, 2022a ). The term “pediatric-type” has been introduced to distinguish these tumors from the adult-type counterpart.…”

Section: Pediatric-type Diffuse High-grade Gliomasmentioning

confidence: 99%

“…The number of possible differential diagnoses is high. The application of 2021 CNS WHO diagnostic criteria is greatly helpful for managing differential diagnoses, although some critical issues may be raised ( Gianno et al, 2022a ). First, the characteristic genetic alterations of the three pHGG H3/IDH WT subtypes are not exclusive of this entity and may be frequently found in other CNS tumors.…”

Section: Pediatric-type Diffuse High-grade Gliomasmentioning

confidence: 99%

“…The GAB1 and YAP1 protein immunostaining in the cytoplasm identifies the SHH-activated group. The medulloblastoma WNT and SHH groups both have cytoplasmic positivity for filamin A. Non-WNT/non-SHH tumors are immunonegative for GAB1 and YAP1 ( Gianno et al, 2022b ). Nevertheless, the gold standard for assessing the status of a medulloblastoma subgroup is DNA methylation profiling ( Schwalbe et al, 2017 ).…”

Section: Embryonal Tumorsmentioning

confidence: 99%

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Pediatric CNS tumors and 2021 WHO classification: what do oncologists need from pathologists?

d’Amati,

Bargiacchi,

Rossi

et al. 2024

Front. Mol. Neurosci.

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The fifth edition of the WHO Classification of Tumors of the Central Nervous System (CNS), published in 2021, established new approaches to both CNS tumor nomenclature and grading, emphasizing the importance of integrated diagnoses and layered reports. This edition increased the role of molecular diagnostics in CNS tumor classification while still relying on other established approaches such as histology and immunohistochemistry. Moreover, it introduced new tumor types and subtypes based on novel diagnostic technologies such as DNA methylome profiling. Over the past decade, molecular techniques identified numerous key genetic alterations in CSN tumors, with important implications regarding the understanding of pathogenesis but also for prognosis and the development and application of effective molecularly targeted therapies. This review summarizes the major changes in the 2021 fifth edition classification of pediatric CNS tumors, highlighting for each entity the molecular alterations and other information that are relevant for diagnostic, prognostic, or therapeutic purposes and that patients’ and oncologists’ need from a pathology report.

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Section: Pediatric-type Diffuse High-grade Gliomasmentioning

confidence: 99%

Section: Pediatric-type Diffuse High-grade Gliomasmentioning

confidence: 99%

Section: Embryonal Tumorsmentioning

confidence: 99%

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Pediatric CNS tumors and 2021 WHO classification: what do oncologists need from pathologists?

d’Amati,

Bargiacchi,

Rossi

et al. 2024

Front. Mol. Neurosci.

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“…Diffuse midline gliomas, H3 K27-altered can be classified into distinct subclassifications based on the histone H3 variant [ 110 , 111 , 112 ]. The first subtype encompasses H3.3 - mutant cases [ 112 ].…”

Section: Diffuse Midline Glioma H3 K27-alteredmentioning

confidence: 99%

The 2021 World Health Organization Central Nervous System Tumor Classification: The Spectrum of Diffuse Gliomas

Gue,

Lakhani

2024

Biomedicines

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The 2021 edition of the World Health Organization (WHO) classification of central nervous system tumors introduces significant revisions across various tumor types. These updates, encompassing changes in diagnostic techniques, genomic integration, terminology, and grading, are crucial for radiologists, who play a critical role in interpreting brain tumor imaging. Such changes impact the diagnosis and management of nearly all central nervous system tumor categories, including the reclassification, addition, and removal of specific tumor entities. Given their pivotal role in patient care, radiologists must remain conversant with these revisions to effectively contribute to multidisciplinary tumor boards and collaborate with peers in neuro-oncology, neurosurgery, radiation oncology, and neuropathology. This knowledge is essential not only for accurate diagnosis and staging, but also for understanding the molecular and genetic underpinnings of tumors, which can influence treatment decisions and prognostication. This review, therefore, focuses on the most pertinent updates concerning the classification of adult diffuse gliomas, highlighting the aspects most relevant to radiological practice. Emphasis is placed on the implications of new genetic information on tumor behavior and imaging findings, providing necessary tools to stay abreast of advancements in the field. This comprehensive overview aims to enhance the radiologist’s ability to integrate new WHO classification criteria into everyday practice, ultimately improving patient outcomes through informed and precise imaging assessments.

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“…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. Bertero et al 4 report the advances in the classification of ependymal neoplasms, which merging anatomic, histologic, immunohistochemical, sequencing, and methylation profiling has significantly improved the prognostic stratification of patients harboring such neoplasms.…”

mentioning

confidence: 99%