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Chordoma: incidence and survival patterns in the United States, 1973–1995

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Abstract

Background: Chordoma, a rare tumor arising from notochordal remnants, has been described to date only by single-institution case series or small population-based surveys.

Methods: We used data from the Surveillance, Epidemiology, and End Results (SEER) program of the National Cancer Institute, 1973–1995, to calculate age-adjusted incidence and survival rates for 400 cases of microscopically confirmed chordoma and to derive information regarding case distribution and risk of second cancer.

Results: The age-adjusted chordoma incidence rate (IR) of 0.08 per 100,000 was age-dependent, more common in males (IR 0.10) than females (IR 0.06) and rare among patients aged <40 years and blacks. Within the axial skeleton 32% of cases were cranial, 32.8% spinal and 29.2% sacral. Young age (<26 years; p = 0.0001) and female sex (p = 0.037) were associated with greater likelihood of cranial presentation. There was no overall increased risk for second primary cancers after chordoma. Median survival was 6.29 years; 5- and 10-year relative survival rates were 67.6% and 39.9%, respectively. Comparison with other bone sarcomas revealed racial disparities in incidence for the two developmental tumors, chordoma and Ewing's sarcoma.

Conclusions: This study provides new data regarding incidence and survival patterns of chordoma in the US. Additional epidemiologic studies are required to elucidate the genetic and environmental determinants underlying this rare, distinctive neoplasm.

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References

  1. Salisbury JR (1993) The pathology of the human notochord. J Pathol 171: 253-255.

    Google Scholar 

  2. Ho KL (1985) Ecchordosis physaliphora and chordoma: a comparative ultrastructural study. Clin Neuropathol 4: 77-86.

    Google Scholar 

  3. Paavolainen P, Teppo L (1976) Chordoma in Finland. Acta Orthop Scand 47: 46-51.

    Google Scholar 

  4. Eriksson B, Gunterberg B, Kindblom LG (1981) Chordoma. A clinicopathologic and prognostic study of a Swedish national series. Acta Orthop Scand 52: 49-58.

    Google Scholar 

  5. O'Neill P, Bell BA, Miller JD, Jacobson I, Guthrie W (1985) Fifty years of experience with chordomas in southeast Scotland. Neurosurgery 16: 166-170.

    Google Scholar 

  6. Price CH, Jeffree GM (1977) Incidence of bone sarcoma in SW England, 1946-74, in relation to age, sex, tumour site and histology. Br J Cancer 36: 511-522.

    Google Scholar 

  7. Dreghorn CR, Newman RJ, Hardy GJ, Dickson RA (1990) Primary tumors of the axial skeleton. Experience of the Leeds Regional Bone Tumor Registry. Spine 15: 137-140.

    Google Scholar 

  8. Dorfman HD, Czerniak B (1995) Bone cancers. Cancer 75: 203-210.

    Google Scholar 

  9. World Health Organization (1990) International Classification of Diseases for Oncology, 2nd edn. Geneva: World Health Organization.

    Google Scholar 

  10. Breslow NE, Day NE (1987) Statistical Methods in Cancer Research. Lyon: International Agency for Research on Cancer.

    Google Scholar 

  11. Dahlin DC, Unni KK (1986) Chordoma. In: Bone Tumors, 4th edn. Springfield, IL: Charles C. Thomas, pp. 379-391.

    Google Scholar 

  12. Heffelfinger MJ, Dahlin DC, MacCarty CS, Beabout JW (1973) Chordomas and cartilaginous tumors at the skull base. Cancer 32: 410-420.

    Google Scholar 

  13. Kamrin RP, Potanos JN, Pool JL (1964) An evaluation of the diagnosis and treatment of chordoma. J Neurol Neurosurg Psychiatry 27: 157-165.

    Google Scholar 

  14. Higinbotham NL, Phillips RF, Farr HW, Hustu HO (1967) Chordoma. Thirty-five-year study at Memorial Hospital. Cancer 20: 1841-1850.

    Google Scholar 

  15. Krayenbühl H, Yasargil MG (1975) Cranial chordomas. Progr Neurol Surg 6: 380-434.

    Google Scholar 

  16. Firooznia H, Pinto RS, Lin JP, Baruch HH, Zausner J (1976) Chordoma: radiologic evaluation of 20 cases. Am J Roentgenol 127: 797-805.

    Google Scholar 

  17. Scuotto A, Albanese V, Tomasello F (1980) Clival chordomas in children. Acta Neurol (Napoli) 2: 121-127.

    Google Scholar 

  18. Rich TA, Schiller A, Suit HD, Mankin HJ (1985) Clinical and pathologic review of 48 cases of chordoma. Cancer 56: 182-187.

    Google Scholar 

  19. Krengli M, Liebsch NJ, Hug EB, Orecchia R (1998) Review of current protocols for protontherapy in USA. Tumori 84: 209-216.

    Google Scholar 

  20. Hall WA, Clark HB (1995) Sacrococcygeal chordoma metastatic to the brain with review of the literature. J Neurooncol 25: 155-159.

    Google Scholar 

  21. Ishida T, Dorfman HD (1994) Chondroid chordoma versus low-grade chondrosarcoma of the base of the skull: can immunohis-tochemistry resolve the controversy? J Neurooncol 18: 199-206.

    Google Scholar 

  22. Saito A, Hasegawa T, Shimoda T, et al. (1998) Dedifferentiated chordoma: a case report. Jpn J Clin Oncol 28: 766-771.

    Google Scholar 

  23. Meis JM, Raymond AK, Evans HL, Charles RE, Giraldo AA (1987) "Dedifferentiated'' chordoma. A clinicopathologic and immunohistochemical study of three cases. Am J Surg Pathol 11: 516-525.

    Google Scholar 

  24. Miettinen M, Lehto VP, Virtanen I (1984) Malignant fibrous histiocytoma within a recurrent chordoma. A light microscopic, electron microscopic, and immunohistochemical study. Am J Clin Pathol 82: 738-743.

    Google Scholar 

  25. Choi YJ, Kim TS (1994) Malignant fibrous histiocytoma in chordoma ― immunohistochemical evidence of transformation from chordoma to malignant fibrous histiocytoma. Yonsei Med J 35: 239-243.

    Google Scholar 

  26. Chambers PW, Schwinn CP (1979) Chordoma. A clinicopathologic study of metastasis. Am J Clin Pathol 72: 765-776.

    Google Scholar 

  27. Halpern J, Kopolovic J, Catane R (1984) Malignant fibrous histiocytoma developing in irradiated sacral chordoma. Cancer 53: 2661-2662.

    Google Scholar 

  28. Belza MG, Urich H (1986) Chordoma and malignant fibrous histiocytoma. Evidence for transformation. Cancer 58: 1082-1087.

    Google Scholar 

  29. Hruban RH, May M, Marcove RC, Huvos AG (1990) Lumbo-sacral chordoma with high-grade malignant cartilaginous and spindle cell components. Am J Surg Pathol 14: 384-389.

    Google Scholar 

  30. Nolte K (1979) Malignant intracranial chordoma and sarcoma of the clivus in infancy. Pediatr Radiol 8: 1-6.

    Google Scholar 

  31. Sibley RK, Day DL, Dehner LP, Trueworthy RC (1987) Metas-tasizing chordoma in early childhood: a pathological and immu-nohistochemical study with review of the literature. Pediatr Pathol 7: 287-301.

    Google Scholar 

  32. Matsumoto J, Towbin RB, Ball WSJ (1989) Cranial chordomas in infancy and childhood. A report of two cases and review of the literature. Pediatr Radiol 20: 28-32.

    Google Scholar 

  33. Kaneko Y, Sato Y, Iwaki T, et al. (1991) Chordoma in early childhood: a clinicopathological study. Neurosurgery 29: 442-446.

    Google Scholar 

  34. Coffin CM, Swanson PE, Wick MR, Dehner LP (1993) Chordoma in childhood and adolescence. A clinicopathologic analysis of 12 cases [See comments]. Arch Pathol Lab Med 117: 927-933.

    Google Scholar 

  35. Borba LAB, al-Mefty O, Mrak RE, Suen J (1996) Cranial chordomas in children and adolescents [See comments]. J Neuro-surg 84: 584-591.

    Google Scholar 

  36. Austin-Seymour M, Munzenrider J, Goitein M, et al. (1989) Fractionated proton radiation therapy of chordoma and low-grade chondrosarcoma of the base of the skull. J Neurosurg 70: 13-17.

    Google Scholar 

  37. O'Connell JX, Renard LG, Liebsch NJ, et al. (1994) Base of skull chordoma. A correlative study of histologic and clinical features of 62 cases. Cancer 74: 2261-2267.

    Google Scholar 

  38. Gay E, Sekhar LN, Rubinstein E, et al. (1995) Chordomas and chondrosarcomas of the cranial base: results and follow-up of 60 patients. Neurosurgery 36: 887-896.

    Google Scholar 

  39. Munzenrider JE, Liebsch NJ (1999) Proton therapy for tumors of the skull base. Strahlenther Onkol 175(Suppl. 2): 57-63.

    Google Scholar 

  40. Castro JR, Linstadt DE, Bahary JP, et al. (1994) Experience in charged particle irradiation of tumors of the skull base: 1977±1992 [See comments]. Int J Radiat Oncol Biol Phys 29: 647-655.

    Google Scholar 

  41. Fagundes MA, Hug EB, Liebsch NJ, et al. (1995) Radiation therapy for chordomas of the base of skull and cervical spine: patterns of failure and outcome after relapse. Int J Radiat Oncol Biol Phys 33: 5790-584.

    Google Scholar 

  42. Hug EB, Loredo LN, Slater JD, et al. (1999) Proton radiation therapy for chordomas and chondrosarcomas of the skull base. J Neurosurg 91: 432-439.

    Google Scholar 

  43. Halperin EC (1997) Why is female sex an independent predictor of shortened overall survival after proton/photon radiation therapy for skull base chordomas? Int J Radiat Oncol Biol Phys 38: 225-230.

    Google Scholar 

  44. Dahlin DC, Unni K (1986) Bone Tumors: General Aspects and Data on 8,542 Cases, 4th edn. Springfield, IL: Charles C. Thomas.

    Google Scholar 

  45. Finley JL, Silverman JF, Dabbs DJ, et al. (1986) Chordoma: diagnosis by fine-needle aspiration biopsy with histologic, immun-ocytochemical, and ultrastructural conformation. Diagn Cytopathol 2: 330-337.

    Google Scholar 

  46. Walaas L, Kindblom LG (1991) Fine-needle aspiration biopsy in the preoperative diagnosis of chordoma: a study of 17 cases with application of electron microscopic, histochemical, and immuno-cytochemical examination. Hum Pathol 22: 22-28.

    Google Scholar 

  47. Mapstone TB, Kaufman B, Ratcheson RA (1983) Intradural chordoma without bone involvement: nuclear magnetic resonance (NMR) appearance. Case report. J Neurosurg 59: 535-537.

    Google Scholar 

  48. Rosenthal DI, Scott JA, Mankin HJ, Wismer GL, Brady TJ (1985) Sacrococcygeal chordoma: magnetic resonance imaging and computed tomography. Am J Roentgenol 145: 143-147.

    Google Scholar 

  49. Miettinen M, Lehto VP, Dahl D, Virtanen I (1983) Differential diagnosis of chordoma, chondroid, and ependymal tumors as aided by anti-intermediate filament antibodies. Am J Pathol 112: 160-169.

    Google Scholar 

  50. Miettinen M (1984) Chordoma. Antibodies to epithelial membrane antigen and carcinoembryonic antigen in differential diagnosis. Arch Pathol Lab Med 108: 891-892.

    Google Scholar 

  51. Brooks JJ, LiVolsi VA, Trojanowski JQ (1987) Does chondroid chordoma exist? Acta Neuropathol (Berl) 72: 229-235.

    Google Scholar 

  52. Persons DL, Bridge JA, Neff JR (1991) Cytogenetic analysis of two sacral chordomas. Cancer Genet Cytogenet 56: 197-201.

    Google Scholar 

  53. Gibas Z, Miettinen M, Sandberg AA (1992) Chromosomal abnormalities in two chordomas. Cancer Genet Cytogenet 58: 169-173.

    Google Scholar 

  54. DeBoer JM, Neff JR, Bridge JA (1992) Cytogenetics of sacral chordoma. Cancer Genet Cytogenet 64: 95-96.

    Google Scholar 

  55. Bridge JA, Pickering D, Neff JR (1994) Cytogenetic and molecular cytogenetic analysis of sacral chordoma. Cancer Genet Cytogenet 75: 23-25.

    Google Scholar 

  56. Mertens F, Kreicbergs A, Rydholm A, et al. (1994) Clonal chromosome aberrations in three sacral chordomas. Cancer Genet Cytogenet 73: 147-151.

    Google Scholar 

  57. Butler MG, Dahir GA, Hedges LK, et al. (1995) Cytogenetic, telomere, and telomerase studies in five surgically managed lumbosacral chordomas. Cancer Genet Cytogenet 85: 51-57.

    Google Scholar 

  58. Buonamici L, Roncaroli F, Fioravanti A, et al. (1999) Cytogenetic investigation of chordomas of the skull. Cancer Genet Cytogenet 112: 49-52.

    Google Scholar 

  59. Dalprá L, Malgara R, Miozzo M, et al. (1999) First cytogenetic study of a recurrent familial chordoma of the clivus. Int J Cancer 81: 24-30.

    Google Scholar 

  60. Foote RF, Ablin G, Hall W (1958) Chordoma in siblings. Calif Med 88: 386.

    Google Scholar 

  61. Enin IP (1964) [Chordoma of the nasopharynx in two members of the same family]. Vestn Oto-rino-laring 26: 88-90.

    Google Scholar 

  62. Kerr WA, Allen KL, Haynes DR, Sellars SL (1975) Letter: Familial nasopharyngeal chordoma. S Afr Med J 49: 1584.

    Google Scholar 

  63. Parry DM, Patronas N, Glenn GM, Duffey D, Oldfield E, Tucker MA (1997) Autosomal dominant transmission of chordoma in three successive generations. Am J Hum Genet 61: 77-77.

    Google Scholar 

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Authors and Affiliations

  1. Genetic Epidemiology Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA

    Mary L. McMaster, Alisa M. Goldstein, Christina M. Bromley, Naoko Ishibe & Dilys M. Parry

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  1. Mary L. McMaster
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McMaster, M.L., Goldstein, A.M., Bromley, C.M. et al. Chordoma: incidence and survival patterns in the United States, 1973–1995. Cancer Causes Control 12, 1–11 (2001). https://doi.org/10.1023/A:1008947301735

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