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Sie zeigen eine notochordale Differenzierung mit einer nukle\u00e4ren Expression von Brachyury (<jats:italic>TBXT<\/jats:italic>). Hauptlokalisation ist das axiale Skelett vom Clivus bis zum Sakrum und dem Os coccygis. Chordome wachsen langsam und lange nur lokal destruierend. Sie werden daher oft erst sp\u00e4t diagnostiziert. Putative Vorl\u00e4uferl\u00e4sionen sind Chordareste und der benigne notochordale Zelltumor. Dabei handelt es sich um zumeist mikroskopisch kleine, intraoss\u00e4re L\u00e4sionen. Bei Chordomen werden unterschiedliche histologische Subtypen differenziert, welche sich prognostisch unterscheiden. Wiederkehrende genetische Ver\u00e4nderungen als Tumorausl\u00f6ser sind nicht bekannt. Brachyury scheint eine noch nicht im Detail gekl\u00e4rte Schl\u00fcsselfunktion in der Entstehung von Chordomen zu besitzen. Die chirurgische En-bloc-Resektion mit negativen Resektionsr\u00e4ndern ist die einzige kurative Behandlungsoption. Die Hochdosisbestrahlung, speziell mit Protonen und Carbonionen, ist eine therapeutische Alternative in inoperablen F\u00e4llen. Medikament\u00f6se Therapien sind f\u00fcr Chordome derzeit nicht zugelassen. Erste klinische Studien zu neuen Therapiemodalit\u00e4ten laufen.<\/jats:p>","DOI":"10.1007\/s00292-020-00761-4","type":"journal-article","created":{"date-parts":[[2020,2,25]],"date-time":"2020-02-25T14:02:48Z","timestamp":1582639368000},"page":"153-162","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Chordome: Gibt es eine molekulargenetische Grundlage f\u00fcr Diagnostik und Therapie?","Chordoma: is there a\u00a0molecular basis for diagnosis and treatment?"],"prefix":"10.1007","volume":"41","author":[{"given":"Susanne","family":"Scheipl","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jasminka","family":"Igrec","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andreas","family":"Leithner","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria","family":"Smolle","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Johannes","family":"Hayb\u00e4ck","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bernadette","family":"Liegl","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2020,2,25]]},"reference":[{"key":"761_CR1","doi-asserted-by":"crossref","first-page":"972","DOI":"10.1016\/j.ijrobp.2017.12.263","volume":"100","author":"N Aibe","year":"2018","unstructured":"Aibe\u00a0N, Demizu\u00a0Y, Sulaiman\u00a0NS et\u00a0al (2018) Outcomes of patients with primary sacral chordoma treated with definitive proton beam therapy. 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