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SHR Neuro Krebs Kardio Lipid

Scheipl, S; Igrec, J; Leithner, A; Smolle, M; Haybäck, J; Liegl, B.
[Chordoma: is there a molecular basis for diagnosis and treatment?]
Pathologe. 2020; 41(2):153-162 [OPEN ACCESS]
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Autor/innen der Med Uni Graz:
Haybäck Johannes
Igrec Jasminka
Leithner Andreas
Liegl-Atzwanger Bernadette
Scheipl Susanne
Smolle Maria
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Abstract:
Chordomas are malignant bone tumours with a reported annual incidence of 0.08 per 100,000 cases. They show a notochordal differentiation and are characterised by their nuclear expression of brachyury (TBXT). Chordomas are localised in the axial skeleton, where they occur from the clivus to the sacrococcygeal region. They are slow growing, locally destructive tumours, and are often not diagnosed until they have reached an advanced stage. Putative precursor-lesions are benign notochordal cell lesions, which are microscopically small and intraosseous. Different histological chordoma subtypes exist, which differ in their prognosis. To date, there are no known recurrent genetic drivers for this disease. Brachyury seems to play a key role in the pathogenesis of chordoma, though the detailed mechanism still needs to be elucidated. Surgical en bloc resection with negative margins is the only curative treatment for this disease. High-dose irradiation, particularly with protons and carbon ions, is a therapeutic alternative in cases of inoperable tumours. Currently, there is no approved medical treatment for chordoma. Clinical trials exploring additional therapeutic modalities are ongoing.

Find related publications in this database (Keywords)
TBXT
INI1
Classic chordoma
Chondroid chordoma
Poorly differentiated chordoma
Dedifferentiated chordoma
Heavy ion irradiation
En-bloc resection
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