Intracranial chordomas: a clinicopathological and prognostic study of 51 cases

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✓ Fifty-one patients with intracranial chordomas who were surgically treated between 1960 and 1984 were studied. Median patient age was 46 years, and 73% presented with diplopia or headache. Nineteen tumors were classified as the “chondroid” type. The extent of surgical removal was a biopsy in 11 patients and subtotal removal or greater in 40. Thirty-nine patients received postoperative radiation therapy. At the time of analysis, 17 patients were alive, and the estimated 5- and 10-year survival rates were 51% and 35%, respectively, for the group of 51 patients. Univariate analysis showed that: 1) patients undergoing resection lived longer (the 5-year survival rate was 36% for the 11 biopsy patients compared with 55% for the 40 patients who had resection; 2) patients who underwent postoperative radiotherapy tended to have longer disease-free survival times; and 3) overall and disease-free survival data were the same for patients with chondroid tumors and those with typical chordomas. Multivariate analysis showed that: 1) age was the factor most strongly associated with longer overall and disease-free survival; 2) diplopia was associated with longer survival; and 3) tumoral mitotic activity tended to be associated with shorter disease-free survival. One tumor metastasized to the cervical cord, and two tumors underwent anaplastic transformation. These data suggest that the prognosis in patients with chordomas is unfavorable, young age is the single factor most strongly associated with longer survival, surgical resection is beneficial, and postoperative radiotherapy may prolong disease-free survival.

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Address reprint requests to: Terrence L. Cascino, M.D., Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, Minnesota 55905.

© AANS, except where prohibited by US copyright law.

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Figures

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    Photomicrographs of typical (upper) and chondroid (lower) chordomas. Upper Left: Typical chordoma specimen exhibiting extensive cellular vacuolization and mucoid microcyst formation. H & E, × 190. Upper Right: Abundant cytoplasmic mucus in physaliphorous cells (arrows) in a typical chordoma. H & E, × 300. Lower Left: Chondroid tumor specimen showing chordoma cells. H & E, × 300. Lower Right: Various proportions of hyaline cartilage in a chondroid chordoma. H & E, × 125.

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    Graph showing overall survival and disease-free survival for 51 patients with cranial chordoma, and expected survival of age- and sex-matched persons from the general population. Vertical hatch marks indicate length of follow-up monitoring for patients still alive at the time of analysis. n = number of cases in each group.

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    Graph showing overall survival according to age of patient. Vertical hatch marks indicate length of follow-up monitoring for patients still alive at the time of analysis. n = number of cases in each group.

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    Graph showing overall survival according to age of patient and the symptom diplopia. Vertical hatch marks indicate length of follow-up monitoring for patients still alive at the time of analysis, n = number of cases in each group.

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    Graph showing overall survival for younger and older patients with or without surgical resection. Vertical hatch marks indicate length of follow-up monitoring for patients still alive at the time of analysis, n = number of cases in each group.

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    Graph showing disease-free survival for younger and older patients with or without postoperative radiotherapy. Vertical hatch marks indicate length of follow-up monitoring for patients still alive at the time of analysis. n = number of cases in each group.

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    Graph showing overall survival according to both patient age and presence of chondroid features. Vertical hatch marks indicate length of follow-up monitoring for patients still alive at the time of analysis, n = number of cases in each group.

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