Review
Year : 2025 | Volume : 9 | Issue : 2 | Page : 62-77
Central Nervous System Embryonal Tumours Through Time: From Historical Concepts to Integrated Morphological and Molecular Insights
Review
Ishita Pant 1, Sujata Chaturvedi2, Ajay Chaudhary3, Monali chaturvedi4
1,2,4 Institute of Human Behaviour and Allied Sciences, Delhi, India, 3 Atal Bihari Vajpayee Institute of Medical Sciences and Dr. Ram Manohar Lohia Hospital, Delhi, India
Address for correspondence:
Department of Pathology, Institute of Human Behaviour and Allied Sciences, Dilshad Garden, Delhi-110095, India
Email: ishitapant@gmail.com
Abstract
Central nervous system (CNS) embryonal tumours are a heterogeneous and aggressive group of neoplasms that predominantly affect children and adolescents. These tumours have long posed diagnostic and therapeutic challenges due to overlapping morphological features and variable biological behaviour. Traditionally, they were classified into medulloblastomas and supratentorial primitive neuroectodermal tumours (PNETs) based on histology. However, the term “PNET” lacked biological specificity and reproducibility, leading to diagnostic ambiguity and limited clinical utility. The 2016 World Health Organisation (WHO) classification represented a paradigm shift by eliminating the designation “PNET” and incorporating molecular parameters into tumour classification, reflecting the limitations of morphology alone. Building on this framework, the 2021 WHO classification adopts an integrated diagnostic approach that combines histopathology, immunohistochemistry, and DNA methylation profiling. This refined classification has significant clinical relevance by enabling more accurate prognostication, risk stratification, and the development of tailored molecularly targeted therapies. This review provides a comprehensive overview of the 2021 WHO classification of CNS embryonal tumours, with emphasis on the integration of histopathological and molecular features. reflecting the limitations of morphology alone. Building on this framework, the 2021 WHO classification adopts an integrated diagnostic approach that combines histopathology, immunohistochemistry, and DNA methylation profiling. This refined classification has significant clinical relevance by enabling more accurate prognostication, risk stratification, and the development of tailored molecularly targeted therapies. This review provides a comprehensive overview of the 2021 WHO classification of CNS embryonal tumours, with emphasis on the integration of histopathological and molecularfeatures.
Acknowledgment
Nil
Conflict of interest
Nil
Source of funding
Nil
How to cite this article: Ishita Pant, Sujata Chaturvedi, Ajay Chaudhary, Monali chaturvedi, Central Nervous System Embryonal Tumours Through Time: From Historical Concepts to Integrated Morphological and Molecular Insights. Int J Clinicopathol Correl. 2025; 9(2):62-77.10.56501/Int.J.Clin.Pathol.Correl..v9.i2.ijcc0001.
