Abstract
Twelve patients with histologically confirmed clivus chordoma were treated at the Johns Hopkins Hospital between 1971 and 1989. Eight of the patients were men and four were women. The mean age at first operation was 51 years (range, 10 to 80). The most common presenting symptoms were headache, diplopia, dysphagia and dysarthria, and facial sensory changes. Computed tomography, with and without contrast enhancement, proved adequate for tumor identification and localization. Magnetic resonance imaging and angiography were occasionally employed to localize the tumors further and to define tumor vascular supply and proximity to vascular structures. Twenty-two resections were performed in 11 patients, and another patient underwent biopsy only. Seven were also treated with radiation therapy. Tumors recurred in eight patients, six of whom underwent further operations. The mean time to first recurrence was 22 months (range 8 to 36 months). Six of the patients are still alive, with a mean follow-up of 31 months (range, 3 to 89 months) from first surgical resection. The mean survival time from first treatment was 31 months (range, 4 to 62 months) among those patients who died. There was no operative mortality. The 5-year cumulative survival rate was 20%. Six patients with long follow-up have had fair to good results, being free of recurrences for at least a year. However, none of the patients returned to their premorbid baseline of activities. Five of the patients had tumors with the histologic diagnosis of chondroid chordoma. Three of these patients are still alive. The mean age at first treatment was 44 (compared with 62 for typical chordomas). The mean time from symptoms to diagnosis was 29 months (typical chordomas, 18 months). The mean length of survival and time to tumor recurrence were not significantly different between chondroid and typical chordomas.
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Selected References
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- Austin-Seymour M., Munzenrider J., Goitein M., Verhey L., Urie M., Gentry R., Birnbaum S., Ruotolo D., McManus P., Skates S. Fractionated proton radiation therapy of chordoma and low-grade chondrosarcoma of the base of the skull. J Neurosurg. 1989 Jan;70(1):13–17. doi: 10.3171/jns.1989.70.1.0013. [DOI] [PubMed] [Google Scholar]
- Bagan S. M., Hollenhorst R. W. Ocular manifestations of intracranial chordomas. Trans Am Ophthalmol Soc. 1980;78:148–155. [PMC free article] [PubMed] [Google Scholar]
- CONGDON C. C. Benign and malignant chordomas; a clinico-anatomical study of twenty-two cases. Am J Pathol. 1952 Sep-Oct;28(5):793–821. [PMC free article] [PubMed] [Google Scholar]
- Chetiyawardana A. D. Chordoma: results of treatment. Clin Radiol. 1984 Mar;35(2):159–161. doi: 10.1016/s0009-9260(84)80024-0. [DOI] [PubMed] [Google Scholar]
- DAHLIN D. C., MACCARTY C. S. Chordoma. Cancer. 1952 Nov;5(6):1170–1178. doi: 10.1002/1097-0142(195211)5:6<1170::aid-cncr2820050613>3.0.co;2-c. [DOI] [PubMed] [Google Scholar]
- Eriksson B., Gunterberg B., Kindblom L. G. Chordoma. A clinicopathologic and prognostic study of a Swedish national series. Acta Orthop Scand. 1981 Feb;52(1):49–58. doi: 10.3109/17453678108991758. [DOI] [PubMed] [Google Scholar]
- Falconer M. A., Bailey I. C., Duchen L. W. Surgical treatment of chordoma and chondroma of the skull base. J Neurosurg. 1968 Sep;29(3):261–275. doi: 10.3171/jns.1968.29.3.0261. [DOI] [PubMed] [Google Scholar]
- Fuller D. B., Bloom J. G. Radiotherapy for chordoma. Int J Radiat Oncol Biol Phys. 1988 Aug;15(2):331–339. doi: 10.1016/s0360-3016(98)90012-8. [DOI] [PubMed] [Google Scholar]
- Heffelfinger M. J., Dahlin D. C., MacCarty C. S., Beabout J. W. Chordomas and cartilaginous tumors at the skull base. Cancer. 1973 Aug;32(2):410–420. doi: 10.1002/1097-0142(197308)32:2<410::aid-cncr2820320219>3.0.co;2-s. [DOI] [PubMed] [Google Scholar]
- KAMRIN R. P., POTANOS J. N., POOL J. L. AN EVALUATION OF THE DIAGNOSIS AND TREATMENT OF CHORDOMA. J Neurol Neurosurg Psychiatry. 1964 Apr;27:157–165. doi: 10.1136/jnnp.27.2.157. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Kendall B. E. Cranial chordomas. Br J Radiol. 1977 Oct;50(598):687–698. doi: 10.1259/0007-1285-50-598-687. [DOI] [PubMed] [Google Scholar]
- POPPEN J. L., KING A. B. Chordoma: experience with thirteen cases. J Neurosurg. 1952 Mar;9(2):139–163. doi: 10.3171/jns.1952.9.2.0139. [DOI] [PubMed] [Google Scholar]
- Peña C. E., Horvat B. L., Fisher E. R. The ultrastructure of chordoma. Am J Clin Pathol. 1970 Apr;53(4):544–551. doi: 10.1093/ajcp/53.4.544. [DOI] [PubMed] [Google Scholar]
- Raffel C., Wright D. C., Gutin P. H., Wilson C. B. Cranial chordomas: clinical presentation and results of operative and radiation therapy in twenty-six patients. Neurosurgery. 1985 Nov;17(5):703–710. doi: 10.1227/00006123-198511000-00002. [DOI] [PubMed] [Google Scholar]
- Reddy E. K., Mansfield C. M., Hartman G. V. Chordoma. Int J Radiat Oncol Biol Phys. 1981 Dec;7(12):1709–1711. doi: 10.1016/0360-3016(81)90197-8. [DOI] [PubMed] [Google Scholar]
- Rich T. A., Schiller A., Suit H. D., Mankin H. J. Clinical and pathologic review of 48 cases of chordoma. Cancer. 1985 Jul 1;56(1):182–187. doi: 10.1002/1097-0142(19850701)56:1<182::aid-cncr2820560131>3.0.co;2-j. [DOI] [PubMed] [Google Scholar]
- Schechter M. M., Liebeskind A. L., Azar-Kia B. Intracranial chordomas. Neuroradiology. 1974 Oct;8(2):67–82. doi: 10.1007/BF00345038. [DOI] [PubMed] [Google Scholar]
- Suit H. D., Goitein M., Munzenrider J., Verhey L., Davis K. R., Koehler A., Linggood R., Ojemann R. G. Definitive radiation therapy for chordoma and chondrosarcoma of base of skull and cervical spine. J Neurosurg. 1982 Mar;56(3):377–385. doi: 10.3171/jns.1982.56.3.0377. [DOI] [PubMed] [Google Scholar]
