title: Clivus chordoma: is it enough to image the primary site?
abstract: Chordomas are rare malignant tumors arising from embryonic remnants of the primitive notochord, around which the skull base and vertebral column develop. They are locally aggressive but metastasize rarely. To our knowledge, this is the first reported case of synchronous intraosseous chordomas. A 32-year-old man presented with intermittent double vision secondary to a right-side abducent nerve palsy. Imaging revealed a clivus chordoma and an asymptomatic synchronous second primary chordoma in the fifth lumbar vertebra. Both chordomas were surgically excised: the clivus using the endonasal, endoscopic route and the L5 vertebra by total vertebral excision and replacement with a titanium prosthesis. The patient made an uneventful and complete recovery. We have modified our departmental practice as we believe that all patients diagnosed with chordoma should have magnetic resonance imaging of their entire spinal tract to exclude a second primary chordoma.
authors.affiliation: Department of Ear, Nose, and Throat Diseases, Head and Neck Surgery, International Neuroscience Institute, Hannover, Germany.
authors.forename: Shahzada K
authors.initials: SK
authors.lastname: Ahmed
authors.forename: Hideyuki
authors.initials: H
authors.lastname: Murata
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Draf
references.citation: Gan No Rinsho. 1983 Sep;29(11):1375-8
references.pmid: 6632220
references.citation: Arch Dermatol. 1997 Dec;133(12):1579-80, 1582-3
references.pmid: 9420546
references.citation: Cancer Causes Control. 2001 Jan;12(1):1-11
references.pmid: 11227920
references.citation: J Comput Assist Tomogr. 1983 Apr;7(2):286-9
references.pmid: 6833561
references.citation: Acta Neurochir (Wien). 2003 Jul;145(7):599-603; discussion 603
references.pmid: 12910405
references.citation: Cancer. 1952 Nov;5(6):1170-8
references.pmid: 12998023
references.citation: Zh Vopr Neirokhir Im N N Burdenko. 1981 Nov-Dec;(6):52-4
references.pmid: 7336839
references.citation: Clin Oncol (R Coll Radiol). 1996;8(2):123-4
references.pmid: 8859613
references.citation: Br J Neurosurg. 2006 Apr;20(2):99-103
references.pmid: 16753627
references.citation: J Cancer Res Clin Oncol. 2006 Aug;132(8):529-35
references.pmid: 16788847
references.citation: Am J Clin Pathol. 1979 Nov;72(5):765-76
references.pmid: 389031