Chordoma

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Histopathology of a chordoma with tumor cells arranged like strands in front of a mucoid matrix. Hematoxylin-eosin stain . Magnification 200x
Chordoma on the coccyx in the axial computed tomography image.
Chordoma on the coccyx in the sagittal computed tomography image.

Chordomas are slowly and destructively growing tumors of the spine that metastasize in about 10% of cases .

Chordomas are counted among the bone tumors , although they do not come from bone tissue, but from remnants of the chorda dorsalis (Notochordoa) at the ends of the spine . This also explains their main locations, namely the base of the skull and tailbone .

Epidemiology

Chordomas make up about 1% of all bone tumors and usually only appear after the age of 30. Men and women are roughly equally affected.

Symptoms

Symptoms arise particularly from pressure on nerves from the tumor, which can generally lead to pain and nerve failure. Chordomas in the area of ​​the skull base lead to headache and neck pain, diplopia and other cranial nerve failures.

therapy

Therapy consists of surgical removal. Since this is only completely successful in 30% of cases, radiation therapy usually follows in order to further reduce the recurrence rate. Nevertheless, in two thirds of the cases there is a local recurrence of the tumor. Chordomas are resistant to chemotherapy. Studies suggest the effectiveness of adjuvant therapy with imatinib and lapatinib .

Gene analyzes on eleven patients who were no longer helped by standard therapy showed that DNA repair was impaired in three patients. A PARP inhibitor approved for other types of cancer with a lack of homologous recombination stopped tumor growth for ten months in an experimental treatment - until a new resistance mutation canceled the drug's effect.

forecast

The 5-year survival rate is 63–78%, the 10-year survival rate 16–32%.

literature

  • Heuck, Wörtler, Vestring: Radiology of bone and joint diseases: primary and secondary bone tumors . Thieme Verlag, 1997, ISBN 3-13-107071-4

Individual evidence

  1. ↑ a b c d e Jason K. Wasserman, Denis Gravel, Bibianna Purgina: Chordoma of the Head and Neck: A Review . In: Head and Neck Pathology . October 4, 2017, ISSN  1936-0568 , doi : 10.1007 / s12105-017-0860-8 , PMID 28980142 .
  2. ↑ Phase II study on lapatinib in advanced EGFR-positive chordoma, Annals of Oncology 00: 1–6, 2013, doi: 10.1093 / annonc / mdt117 .
  3. ↑ Stefan Gröschel, Daniel Hübschmann, Francesco Raimondi, et al. (2019) Defective homologous recombination DNA repair as therapeutic target in advanced chordoma. Nature Communications, https://doi.org/10.1038/s41467-019-09633-9

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