Sarcomatous transformation of clival chordoma after charged-particle radiotherapy. Report of two cases

J Neurosurg. 2006 Jul;105(1):136-41. doi: 10.3171/jns.2006.105.1.136.

Abstract

Skull base chordomas containing a sarcomatous component are extremely rare. Here the authors report two new cases in which a recurrent tumor with a sarcomatous component appeared after the patient had undergone charged-particle radiotherapy. Histological examinations performed in Case 1 revealed some retention of epithelial features in the sarcomatous component, whereas no such regions were observed in Case 2. Both patients had rapidly deteriorating clinical courses and died within 6 months after diagnosis of the recurrent tumor. The authors discuss the significance of the histological subtypes of these tumors for long-term prognosis and their pathogenetic mechanisms in relation to radiotherapy. Although these sarcomatous transformations are rare in conventional chordomas, a careful histological examination and thorough follow-up imaging studies are crucial when treating patients with such lesions.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Cell Transformation, Neoplastic
  • Chordoma / pathology*
  • Chordoma / radiotherapy*
  • Cranial Fossa, Posterior*
  • Fatal Outcome
  • Female
  • Humans
  • Middle Aged
  • Sarcoma / pathology*
  • Sarcoma / therapy
  • Skull Base Neoplasms / pathology*
  • Skull Base Neoplasms / radiotherapy*