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Review
. 2024 Apr 12:15:130.
doi: 10.25259/SNI_681_2023. eCollection 2024.

New CNS tumor classification: The importance in pediatric neurosurgical practice

Ramiro José Del Río  1 , Santiago Ezequiel Cicutti  1 , Daniel C Moreira  2 , Javier Danilo Gonzalez Ramos  1
Affiliations
Review

New CNS tumor classification: The importance in pediatric neurosurgical practice

Ramiro José Del Río et al. Surg Neurol Int. .

Abstract

Background: The management of the central nervous system (CNS) tumors in the pediatric population is crucial in neurosurgical practice. The World Health Organization (WHO) has evolved its classification of CNS tumors from the 19th century to the 5th edition, published in 2021, incorporating molecular advancements. This transition from morphology to molecular characterization is ongoing.

Methods: This manuscript analyzes the modifications introduced in the 5th edition of WHO's CNS tumor classification, particularly focusing on pediatric tumor families. The paper integrates clinical, morphological, and molecular information, aiming to guide pediatric neurosurgeons in their daily practice and interdisciplinary discussions.

Results: The 5th edition of the WHO classification introduces a hybrid taxonomy that incorporates both molecular and histological components. The terminology shifts from "entity" to "type" and "subtype," aiming to standardize terminology. Tumor grading experiences changes, integrating molecular biomarkers for prognosis. The concept of integrated layered diagnosis is emphasized, where molecular and histological information is combined systematically.

Conclusion: The 5th edition of the WHO CNS classification signifies a paradigm shift toward molecular characterization. The incorporation of molecular advances, the layered diagnostic approach, and the inclusion of clinical, morphological, and molecular information aim to provide comprehensive insights into pediatric CNS tumors. This classification offers valuable guidance for pediatric neurosurgeons, aiding in precise diagnosis and treatment planning for these complex neoplasms.

Keywords: Brain tumors; Central nervous system; Pediatric central nervous system tumors; Pediatric neurooncology; Pediatrics; World Health Organization classification.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1:
Figure 1:
Two examples of diagnostic layers based on Harlem consensus in tumors often found in the pediatric population. SHH: Sonic Hedgehog
Figure 2:
Figure 2:
Pediatric-type diffuse gliomas: (a) MRI FLAIR, axial section of a 7-year-old patient with an angiocentric glioma (pediatric-type diffuse low-grade glioma), grade 1. (b) MRI T1 with gadolinium, axial section of a 12-year-old patient with a diffuse hemispheric glioma H3 G34-mutant, grade 4 (formerly glioblastoma). (c) MRI T2 axial section of a 7-year-old patient with diffuse midline glioma H3 K27-altered, grade 4 (former diffuse intrinsic pontine glioma). MRI: Magnetic resonance imaging, FLAIR: Fluid attenuated inversion recovery.
Figure 3:
Figure 3:
Circumscribed astrocytic gliomas: (a) MRI T1 with gadolinium, axial section of a 2-year-old patient suffering from a posterior fossa pilocytic astrocytoma, WHO grade 1. (b) MRI T1 with gadolinium, axial section of a 1-year-old patient with a suprasellar pilocytic astrocytoma, WHO grade 1. (c) MRI T2, axial section of a 13-year-old patient with pleomorphic xanthoastrocytoma, WHO grade 3 (former anaplastic pleomorphic xanthoastrocytoma). MRI: Magnetic resonance imaging, WHO: World Health Organization.
Figure 4:
Figure 4:
Classification of ependymomas according to the WHO classification 2021: This tumor type is categorized based on both its histological location and molecular characteristics. WHO: World Health Organization. ZFTA: Zinc finger translocation associated (previously known as C11orf95), YAP: Yes-associated protein, PF: Posterior Fossa, MYCN: Myelocytomatosis viral oncogene neuroblastoma derived homolog.
Figure 5:
Figure 5:
Ependymomas: (a) MRI T2, Axial section. 2-year-old patient with ependymoma PFA, WHO grade 3. (b) MRI T2, Axial section of a 2-year-old patient with a supratentorial ependymoma ZFTA fusion-positive, WHO grade 3. (c) MRI T1 with gadolinium, Sagittal section. 12-year-old patient with a spinal myxopapillary ependymoma, WHO grade 2. MRI: Magnetic resonance imaging, WHO: World Health Organization, PFA: Posterior fossa type A. ZFTA: Zinc finger translocation associated (previously known as C11orf95).
Figure 6:
Figure 6:
Medulloblastoma diagnosis algorithm used for classification into molecular groups according to WHO classification 2021. WHO: World Health Organization. YAP: Yes-associated protein, GAB: Grb2-associated binding protein.
Figure 7:
Figure 7:
Embryonal tumors: (a) MRI T2, an axial section of an 11-year-old boy with a histologically defined medulloblastoma (desmoplastic), WHO grade 4. (b) MRI T1 with gadolinium, an axial section of an 8-year-old patient with a molecularly and histologically defined hemispheric medulloblastoma. Subtype: desmoplastic nodular. SHH TP53-mutant. WHO grade 4. (c) MRI T2, an axial section of a 2-year-old patient with a CNS tumor with BCOR internal tandem duplications. WHO grade 4. MRI: Magnetic resonance imaging, WHO: World Health Organization, SHH: Sonic hedgehog, CNS: Central nervous system. BCOR: BCL6 corepressor.
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