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Skull Base 2007; 17(5): 331-340
DOI: 10.1055/s-2007-986438
CASE REPORT

© Thieme Medical Publishers

Clinical Course and Autopsy Findings of a Patient with Clival Chordoma Who Underwent Multiple Surgeries and Radiation during a 10-Year Period

Masashi Tamaki1 , Masaru Aoyagi1 , Toshihiko Kuroiwa2 , Masaaki Yamamoto4 , Seiji Kishimoto3 , Kikuo Ohno1
  • 1Department of Neurosurgery, Graduate School, Tokyo Medical and Dental University, Yushima, Bunkyo-ku, Tokyo, Japan
  • 2Department of Pathology, Graduate School, Tokyo Medical and Dental University, Yushima, Bunkyo-ku, Tokyo, Japan
  • 3Department of Head and Neck Surgery, Graduate School, Tokyo Medical and Dental University, Yushima, Bunkyo-ku, Tokyo, Japan
  • 4Katsuta Hospital Mito Gamma House, Ibaraki, Japan
Further Information

Publication History

Publication Date:
07 September 2007 (online)

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ABSTRACT

The management of clival chordoma remains problematic. We present the case of a 48-year-old woman with clival chordoma who underwent multiple surgeries and radiation therapy, including gamma knife stereotactic radiosurgery (GK-SRS), during a 10-year clinical course. The tumor was initially removed by gross total resection via the trans-sphenoidal approach, followed by external linac radiation therapy. The tumor recurred at the clivus 5 years after the initial operation. After repeated trans-sphenoidal removal of recurrent tumors, she twice underwent GK-SRS for a tumor remnant adjacent to the brainstem. Although this part of the tumor was controlled by GK-SRS, there was further tumor extension toward the sphenoid and maxillary sinuses. Ultimately, lower cranial nerve dysfunction developed due to tumor extension into the lower part of the clivus and the patient died of respiratory failure. Autopsy revealed the tumor to extend from the lower clivus to the bilateral middle fossae. The lower part of the tumor extended to the nasal cavity and to the posterior wall of the pharynx, resulting in compression of the upper pharyngeal region. The tumor around the jugular foramen compressed the lower cranial nerves bilaterally. Tumor cells did not, however, invade the intradural space microscopically. Although chordoma is not biologically malignant, this tumor can show massive extension with destruction of bony structures and extracranial invasion of connective tissues. Therefore, the optimal treatment strategy is to remove the tumor mass as extensively as possible, including normal bony structures and connective tissues surrounding the tumor, using skull base surgical techniques.

KEYWORDS

Chordoma - autopsy - skull base surgery - gamma knife radiosurgery - proton radiation therapy

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Masashi TamakiM.D. 

Department of Neurosurgery, Graduate School, Tokyo Medical and Dental University

1-5-45 Yushima, Bunkyo-ku, Tokyo 113-8519, Japan

Email: mtamaki.nsrg@tmd.ac.jp

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