Related Experiment Video
Updated: May 14, 2026

Visualization of Chondrocyte Intercalation and Directional Proliferation via Zebrabow Clonal Cell Analysis in the Embryonic Meckel’s Cartilage
Published on: October 21, 2015
From Histology to Multi-Omics: Review of Chordoma Classification and Its Clinical Implications
Szymon Piotr Baluszek1, Paulina Kober2, Mateusz Bujko1
1Laboratory of Molecular Oncology, Maria Skłodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, Poland.
Abstract:
Chordoma is a rare malignant neoplasm of the axial skeleton, arising from notochordal remnants. No approved systemic therapies exist, and the 10-year overall survival is below 60%. Accurate molecular and pathological classification is a prerequisite for improved prognostication and the identification of actionable therapeutic targets; however, molecular classification of chordoma remains significantly less advanced than that of other neoplasms. This narrative review synthesizes proposed classification frameworks for chordoma across histological, radiological, surgical, genomic, epigenomic, transcriptomic, and proteomic domains. PubMed and CENTRAL were searched on 1 February 2026 using five queries: 'chordoma classification', 'chordoma DNA sequencing', 'chordoma RNA sequencing', 'chordoma methylation', and 'chordoma copy number'. Original research articles describing more than one patient and reporting a classification or subtyping framework were included; review articles, case reports, and non-English publications were excluded. Sample size and the use of a validation dataset were identified for each study. Results were synthesized qualitatively. A total of 108 studies encompassing 6349 individuals were included. Across six domains, four cross-cutting themes with prognostic and potential theranostic value emerged: copy number alterations, particularly CDKN2A/B loss; SWI/SNF complex dysfunction; stroma-tumor ratio; and immune microenvironment heterogeneity.
Insights
This review synthesizes chordoma classification frameworks, identifying key molecular and pathological features. These findings aim to improve prognostication and guide the development of targeted therapies for this rare axial skeleton cancer.
Area of Science:
- Oncology
- Genetics
- Pathology
Background:
- Chordoma is a rare axial skeleton malignancy with poor survival rates and no approved systemic therapies.
- Accurate molecular and pathological classification is crucial for prognostication and identifying therapeutic targets.
- Current molecular classification of chordoma lags behind other neoplasms.
Purpose of the Study:
- To synthesize proposed classification frameworks for chordoma across multiple domains.
- To identify cross-cutting themes with prognostic and theranostic value.
- To advance the understanding of chordoma biology for improved patient outcomes.
Main Methods:
- Narrative review of studies from PubMed and CENTRAL databases.
- Searched using keywords related to chordoma classification and molecular analysis (DNA sequencing, RNA sequencing, methylation, copy number).
- Included original research articles with >1 patient and classification/subtyping frameworks; excluded reviews, case reports, and non-English articles.
Main Results:
- Synthesized data from 108 studies involving 6349 individuals.
- Identified four cross-cutting themes with prognostic and theranostic potential: copy number alterations (e.g., CDKN2A/B loss), SWI/SNF complex dysfunction, stroma-tumor ratio, and immune microenvironment heterogeneity.
- Results were synthesized qualitatively across histological, radiological, surgical, genomic, epigenomic, transcriptomic, and proteomic domains.
Conclusions:
- Established classification frameworks highlight key molecular and pathological features of chordoma.
- Identified themes like CDKN2A/B loss and SWI/SNF dysfunction offer prognostic and potential therapeutic insights.
- Further research into these domains can improve chordoma classification, prognostication, and treatment strategies.
Related Concept Videos
Classification of Epithelial Tissues: Overview
Based on the number of cell layers,...
Classification of Bones
Long and Short Bones
The appendicular skeleton, particularly the upper and lower limbs, is primarily made of long and short bones. The long...
Classification of Connective Tissues
Connective Tissue Proper
Connective tissue proper is the most abundant class of connective tissues. As its name implies, it predominantly connects different tissues in the body. Depending on the cell types, ground substance, viscosity, and fiber types in the ECM, connective tissue proper is further categorized into loose and dense.
Classification of Epithelial Tissues: Stratified Epithelium
Tumor Progression
Colon cancer is one of the best-documented examples of tumor progression. Early mutation in the APC gene in colon cells causes a small growth on the colon wall called a polyp. With time, this polyp grows into a benign, pre-cancerous tumor. Further...