The molecular aspects of chordoma

GÜLLÜOĞLU Ş., Turksoy O., Kuskucu A., Ture U., Bayrak O. F.

NEUROSURGICAL REVIEW, cilt.39, sa.2, ss.185-196, 2016 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Derleme
  • Cilt numarası: 39 Sayı: 2
  • Basım Tarihi: 2016
  • Doi Numarası: 10.1007/s10143-015-0663-x
  • Dergi Adı: NEUROSURGICAL REVIEW
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Sayfa Sayıları: ss.185-196
  • Anahtar Kelimeler: Chordoma, Gene expression, Molecular pathways, Molecular pathology, GROWTH-FACTOR RECEPTOR, SKULL BASE CHORDOMAS, TRANSCRIPTION FACTOR BRACHYURY, C-MET, FAMILIAL CHORDOMA, IMMUNOHISTOCHEMICAL ANALYSIS, DIFFERENTIAL EXPRESSION, CLIVAL CHORDOMAS, SPINAL CHORDOMA, PDGFR-ALPHA
  • Marmara Üniversitesi Adresli: Hayır

Özet

Chordomas are one of the rarest bone tumors, and they originate from remnants of embryonic notochord along the spine, more frequently at the skull base and sacrum. Although they are relatively slow growing and low grade, chordomas are highly recurrent, aggressive, locally invasive, and prone to metastasize to the lungs, bone, and the liver. Chordomas highly and generally show a dual epithelial-mesenchymal differentiation. These tumors resist chemotherapy and radiotherapy; therefore, radical surgery and high-dose radiation are the most used treatments, although there is no standard way to treat the disease. The molecular biology process behind the initiation and progression of a chordoma needs to be revealed for a better understanding of the disease and to develop more effective therapies. Efforts to discover the mysteries of these molecular aspects have delineated several molecular and genetic alterations in this tumor. Here, we review and describe the emerging insights into the molecular landscape of chordomas.