Chordoma FactFile

Overview of Chordoma

Malignancy: very rare malignant

Type of bone tumour: primary

Grade: slow-growing/low grade

Location: It affects the vertebral bodies.

The vertebral bodies are the bony area of the spine rather than the jelly disc, though they contain vestigial notochord within the nucleus pulposus.

The notochord is the early stage of the spine in a baby, which normally gets replaced by bone.

Small areas of the notochord remain in adults.

An alternative image presenting the structure of the vertebrae. The notochord is found in the nucleus pulposus, but it appears to be inflamed. This is just an example.

50% grow in the lower end of the spinal vertebrae (sacrum)

The layers of the embryo being formed (endoderm, ectoderm and mesoderm). The presence of the notochord can also be observed.

Other locations of the chordoma.

It may occur in the skull (30%), like the clivus.

The clivus is a bony structure at the base of the skull that supports the brainstem and connects the skull with the spine.

Mobile spine and other parts of the bone (20%).

Extraskeletal tumours are rare.

Location of clivus in the brain and other structures of the nasal passage.

Onset: Mean age is 60 years.

Appearance:

Chordomas are infiltrative.

They destroy the bony portion of the spine and invade surrounding neural spaces.

Types Of Chordoma

Risk Factors Of Chordoma

A) Genetics:

Example One of the genes that increases the risk of Chordoma: Brachyury

Brachyury (T) is a transcription factor of the T-box family.

It binds to target genes in the nucleus, facilitates development, and in some cases disease.

A mutated gene or its duplication of Brachyury is linked with the rare bone cancer (chordoma)

Overexpression of the mutated gene is linked to epithelial tumours that line the gastrointestinal tract (oesophagus, stomach, small intestine), urogenital (kidney, bladder, testis), respiratory (lungs), and the female gynaecological system (uterus, ovaries).

Recent evidence indicates tumour-promoting and tumour suppressor properties. TBX3 promotes tumour via epithelial-mesenchymal transition (EMT) during metastasis.

Overexpression of Brachyury causes resistance to anti-cancer therapy and confers stem cell traits on cancer cells.

This can be detected using high levels of mesenchymal markers (fibronectin, N-cadherin, vimentin) and loss of epithelial markers (E-cadherin, plakoglobin) through immunohistochemistry testing.

(Wansleben et al., 2014)

Other risk factors of chordoma

B) Gender

More in males than females

C) Ethnicity:

Not applicable – no influence on ethnicity.

Appearance Under Microscope

Cell Morphology:

Under the microscope, they commonly have physaliphorous cells (vacuolated or soap-bubbled cells). It is also found in adenocarcinomas and chondrosarcomas. This can be distinguished using immunohistochemistry markers.

Vacuoles are membrane-bound sacs in the cytoplasm of the cells.

The membrane is selectively permeable and is called a tonoplast.

The role of the tonoplast is to control movement of substances in and out of the vacuole and depends on the size.

Inside the vacuole, there are a variety of nutrients, ions, water, enzymes and in some cases waste products. They vary based on the type of cell, size, number, and physiological state.

The presence of a vacuole in animal and plant cells.

Multi-Vacuolated cells. Haemotoxylin and Eosin staining (Frontalcortex.com, 2009)

Illustration of the types of cells and their presence under the microscope using immunohistochemistry markers (Cima et al., 2021)

Infiltrative chordoma in position T10 affecting the vertebral body Dikti (n.d.)

References

Elsamadicy, A. A., Sayeed, S., Sherman, J. J. Z., Serrato, P., Ghanekar, S. D., Lo, S.-F. L., & Sciubba, D. M. (2025). The Association of Gender in the Management and Prognosis of Vertebral and Sacral Chordoma: A SEER Analysis. Journal of Clinical Medicine, 14(5), 1737. https://doi.org/10.3390/jcm14051737

Bădilă, A.E., Rădulescu, D.M., Niculescu, A.-G., Grumezescu, A.M., Rădulescu, M. and Rădulescu, A.R. (2021). Recent Advances in the Treatment of Bone Metastases and Primary Bone Tumors: An Up-to-Date Review. Cancers, [online] 13(16), p.4229. Doi: https://doi.org/10.3390/cancers13164229.

Biermann, J.S., Hirbe, A., Ahlawat, S., Bernthal, N.M., Binitie, O., Boles, S., Brigman, B., Callan, A.K., Cipriano, C., Cranmer, L.D., Davis, J., Donnelly, E., Ferguson, M., Graham, A., Groundland, J., Hess, M., Hiniker, S.M., Hoover-Regan, M.L., Hornick, J.L. and Jonard, B. (2025). Bone Cancer, Version 2.2025, NCCN Clinical Practice Guidelines in Oncology. Journal of the National Comprehensive Cancer Network, [online] 23(4). does: https://doi.org/10.6004/jnccn.2025.0017.

Cancer Research UK (2025a) Types of bone cancer. Available at: https://www.cancerresearchuk.org/about-cancer/bone-cancer/types (Accessed 3^rd April 2026)

Cima, L., Das, A., Venkatesh Dhanasekaran, Mirabassi, N. and Pagliuca, F. (2021). The “Make Surgical Pathology Easy” project: learning Pathology through tailored digital infographics – the case for renovation of an old teaching method. Pathologica, 113(4), pp.252–261. doi:10.32074/1591-951x-269.

Cowan, P., Launico, M. and Kahai, P. (2024) Anatomy, Bones. Available at: https://www.ncbi.nlm.nih.gov/books/NBK537199/ (Accessed: 9^th April 2026)

Ditki (n.d) Chordoma Available at: https://ditki.com/course/pathology/glossary/pathophysiologic-disorder/chordoma (Accessed: 28^th April 2026)

Frontal cortex (2009) Clival chordoma, H&E stain x 400. Available at: https://frontalcortex.com/?page=oll&topic=24&qid=1909 (Accessed: 28^th April 2026)

Gerrand, C., Amary, F., Anwar, H.A., Brennan, B., Dileo, P., Kalkat, M.S., McCabe, M.G., McCullough, A.L., Parry, M.C., Patel, A., Seddon, B.M., Sherriff, J.M., Tirabosco, R. and Strauss, S.J. (2024). UK guidelines for the management of bone sarcomas. British Journal of Cancer. doi:https://doi.org/10.1038/s41416-024-02868-4.

Hosseini, H., Heydari, S., Kiavash Hushmandi, Salman Daneshi and Rasoul Raesi (2025). Bone tumors: a systematic review of prevalence, risk determinants, and survival patterns. BMC Cancer, 25(1). doi:https://doi.org/10.1186/s12885-025-13720-0.

Pullan, J.E., and Lotfollahzadeh, S. (2024) Primary Bone Cancer Available at: https://www.ncbi.nlm.nih.gov/books/NBK560830/ (Accessed: 9^th April 2026)

Sieff, C. (2025) DBA Syndrome Available at: https://www.ncbi.nlm.nih.gov/books/NBK7047/ (Accessed: 22^nd April 2026)

Thakkar, J.B., Deshpande, S.V. and Janu, A. (2019) Enchondroma Vs Low grade Chondrosarcoma : Differentiating features and role of imaging in management. Available at:https://epos.myesr.org/poster/esr/ecr2019/C-2823/Background (Accessed: 23^rd April 2026)

Tumor Surgery (2026) Adamantinoma: A Rare Bone Tumor of the Tibia Explained. Available at: https://www.tumorsurgery.org/2025/12/16/adamantinoma-rare-bone-tumor-tibia/ (Accessed: 4^th April 2026)

van Langevelde, K. and Robin Smithuis, R. (2023) Cartilage tumors with special attention to Atypical Cartilaginous Tumors. Available at: https://radiologyassistant.nl/musculoskeletal/bone-tumors/chondrotumors-1 (Accessed: 23^rd April 2026)

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Wansleben, S., Peres, J., Hare, S., Goding, C.R. and Prince, S. (2014). T-box transcription factors in cancer biology. Biochimica et Biophysica Acta (BBA) – Reviews on Cancer, [online] 1846(2), pp.380–391. doi:https://doi.org/10.1016/j.bbcan.2014.08.004.

‌WHO Editorial Board. WHO classification of bone tumours. In WHO Classification of Tumours Editorial Board. Soft tissue and bone tumours. Bovee J, Flanagan AM, Lazar AJ, Nielsen GP and Yoshida A (eds) pp 338. International Agency for Research on Cancer (2020)

Updated July 2026 Next Review July 2028