[Novel molecular aspects of chordomas].
Chordomas are rare and slowly growing malignant bone tumors which mostly occur in adults. These bone tumors are characterized by epithelial and mesenchymal aspects. It is suggested that they arise from remnants of the notochord because they are found along the axial skeleton (e.g. clival, spinal and sacrococcygeal locations). It appears that cytogenetic aberrations are not randomly found in this tumor group. Loss of chromosomal material (e.g. 1p, 3p, 10q, 13q and 14q) is more frequently found than gain of material (e.g. 7q, especially 7q33). Several studies demonstrated brachyury expression (T; 6q27) as a possible candidate gene in the oncogenesis of chordomas (e.g. knock down in the chordoma cell line U-CH1). So far therapy consists of complete resection and irradiation, e.g. with carbon ions. Targeting therapy is not yet established in routine protocols but phase II studies with tyrosine kinase inhibitors have shown partial response of tumors and, in some studies stabilization of the disease has been described.
Institut für Pathologie und Zytologie, Dr. Horst Schmidt Klinik GmbH, Ludwig-Erhard-Str. 100, 65199, Wiesbaden, Deutschland, scheil-bertram@pathologie-wiesbaden.de.
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D000328:Adult; D001859:Bone Neoplasms / Q000235:genetics* / Q000473:pathology* / Q000628:therapy; D001842:Bone and Bones / Q000473:pathology / Q000601:surgery; D045744:Cell Line, Tumor; D002471:Cell Transformation, Neoplastic / Q000235:genetics / Q000473:pathology; D002817:Chordoma / Q000235:genetics* / Q000473:pathology* / Q000628:therapy; D002869:Chromosome Aberrations; D005326:Fetal Proteins / Q000235:genetics; D056726:Genetic Association Studies; D006801:Humans; D011379:Prognosis; D020825:T-Box Domain Proteins / Q000235:genetics; D000098908:Brachyury Protein
D005326:Fetal Proteins; D020825:T-Box Domain Proteins; D000098908:Brachyury Protein