Annals of Diagnostic Pathology
Volume 11, Issue 4 , Pages 262-273 , August 2007

Dedifferentiated chordoma of the thoracic spine with rhabdomyosarcomatous differentiation. Report of a case and review of the literature

Case presented in part at the IV International Postgraduate Course in Soft Tissue Tumor Pathology. Bolzano, Italy, 5-7 June 1997 (abstr in Pathologica 1998;90:200-201).

  • Michele Bisceglia, MD

      Affiliations

    • Department of Pathology, Division of Anatomic Pathology, IRCCS “Casa Sollievo della Sofferenza” Hospital, I-71013 San Giovanni Rotondo (FG), Italy
    • Corresponding Author InformationCorresponding author. Tel.: +39 0882 410366; fax: +39 0882 410411.
  • ,
  • Vincenzo Antonio D'Angelo, MD

      Affiliations

    • Department of Neurosciences, IRCCS “Casa Sollievo della Sofferenza” Hospital, I-71013 San Giovanni Rotondo (FG), Italy
  • ,
  • Giuseppe Guglielmi, MD

      Affiliations

    • Department of Radiology, IRCCS “Casa Sollievo della Sofferenza” Hospital, I-71013 San Giovanni Rotondo (FG), Italy
  • ,
  • David Ben Dor, MD

      Affiliations

    • Department of Pathology, The Barzilai Medical Center, Ashkelon IL-78306, Israel
  • ,
  • Gianandrea Pasquinelli, MD

      Affiliations

    • Department of Clinical Pathology, Policlinico “S Orsola” Hospital, I-40138 Bologna, Italy

References 

  1. Huvos AG. In: Bone tumors. Diagnosis, treatment, and prognosis. 2nd ed. Philadelphia (Pa): WB Saunders; 1991;p. 599–624
  2. Unni KK. In: Dahlin's bone tumors: general aspects and data on 11,087 Cases. 5th ed.. Philadelphia (Pa): Lippincott-Raven; 1996;p. 291–305
  3. Dorfman HD, Czerniak B. In: Bone tumors. St Louis (Mo): Mosby; 1998;p. 1139–1152
  4. Campanacci M. Bone and soft tissues tumors. Bologna: Aulo Gaggi; Berlin: Springer-Verlag, 1990, p. 689-705.
  5. Topsakal C, Bulut S, Erol FS, et al. Chordoma of the thoracic spine—case report. Neurol Med Chir. 2002;42:175–180
  6. Heffelfinger MJ, Dahlin DC, MacCarty CS, et al. Chordomas and cartilaginous tumors at the skull base. Cancer. 1973;32:410–420
  7. Chambers PW, Schwinn CP. Chordoma. A clinicopathologic study of metastasis. Am J Clin Pathol. 1979;72:765–776
  8. Volpe R, Mazabraud A. A clinicopathologic review of 25 cases of chordoma (a pleomorphic and metastasizing neoplasm). Am J Surg Pathol. 1983;7:161–170
  9. Meis JM, Raymond AK, Evans HL, et al. “Dedifferentiated” chordoma. A clinicopathologic and immunohistochemical study of three cases. Am J Surg Pathol. 1987;11:516–525
  10. Fox JE, Batsakis JG, Owano LR. Unusual manifestations of chordoma. A report of two cases. J Bone Joint Surg Am. 1968;50:1618–1628
  11. Nanda A, Hirsh LF, Antoiniades K. Malignant fibrous histiocytoma in a recurrent thoracic chordoma: case report and literature review. Neurosurgery. 1991;28:588–592
  12. Boriani S, Chevalley F, Weinstein JN, et al. Chordoma of the spine above the sacrum. Treatment and outcome in 21 cases. Spine. 1996;21:1569–1577
  13. McMaster ML, Goldstein AM, Bromley CM, et al. Chordoma: incidence and survival patterns in the United States, 1973-1995. Cancer Causes Control. 2001;12:1–11
  14. Coffin CM, Swanson PE, Wick MR, et al. Chordoma in childhood and adolescence. A clinicopathologic analysis of 12 cases. Arch Pathol Lab Med. 1993;117:927–933
  15. Utne JR, Pugh Dg. The roentgenologic aspects of chordoma. Am J Roentgenol Radium Ther Nucl Med. 1955;74:593–608
  16. Buraczewski J, Rudowski W. Chordoma of the thoracic spine appearing as a mediastinal tumor; report of two cases. J Thorac Surg. 1957;34:75–84
  17. Caputo PJ. Chordoma of the thoracic spine; case report of a rare entity. Northwest Med. 1959;58:553–554
  18. Higinbotham NL, Phillips RF, Farr HW, et al. Chordoma. Thirty-five-year study at Memorial Hospital. Cancer. 1967;20:1841–1850
  19. Gregorius FK, Batzdorf U. Removal of thoracic chordoma by staged laminectomy and thoracotomy: case report. Am Surg. 1979;45:535–537
  20. Sundaresan N, Galicich JH, Chu FC, et al. Spinal chordomas. J Neurosurg. 1979;50:312–319
  21. Stratt B, Steiner RM. The radiologic findings in posterior mediastinal chordoma. Skeletal Radiol. 1980;5:171–173
  22. Cotler HB, Cotler JM, Cohn HE, et al. Intrathoracic chordoma presenting as a posterior superior mediastinal tumor. Spine. 1983;8:781–786
  23. Meyer JE, Lepke RA, Lindfors KK, et al. Chordomas: their CT appearance in the cervical, thoracic and lumbar spine. Radiology. 1984;153:693–696
  24. Schwarz SS, Fisher WS, Pulliam MW. Thoracic chordoma in a patient with paraparesis and ivory vertebral body. Neurosurgery. 1985;16:100–102
  25. Rich TA, Schiller A, Suit HD, et al. Clinical and pathologic review of 48 cases of chordoma. Cancer. 1985;56:182–187
  26. Bellotti C, Ettorre F, Oliveri G, et al. Dorsal vertebral chordoma with intrathoracic invasion. Surgical treatment of a case. Neurochirurgie. 1986;32:86–89
  27. Amendola BE, Amendola MA, Oliver E, et al. Chordoma: role of radiation therapy. Radiology. 1986;158:839–843
  28. Brooks M, Kleefield J, O'Reilly GV, et al. Thoracic chordoma with unusual radiographic features. Comput Radiol. 1987;11:85–90
  29. Kaulbach C, Heller M, Loning T. Thoracic chordoma. Rontgenblatter. 1990;43:295–297
  30. Winants D, Bertal A, Hennequin L, et al. Imaging of cervical and thoracic chordoma. Apropos of 2 cases. J Radiol. 1992;73:169–174
  31. Walsh TM, Mayer PJ. Chordoma of the thoracic spine presenting as a second primary malignant lesion. A case report. Spine. 1992;17:1524–1528
  32. Bjornsson J, Wold LE, Ebersold MJ, et al. Chordoma of the mobile spine. A clinicopathologic analysis of 40 patients. Cancer. 1993;71:735–740
  33. Meador T, Wivell W. Thoracic spine mass in a 34-year-old man. Acad Radiol. 1997;4:157–158
  34. Cury JD, Peterson RJ, Lacy GD, et al. Tracheal deviation from an atypical mediastinal mass. Chest. 1997;111:503–505
  35. Murphy JM, Wallis F, Toland J, et al. CT and MRI appearances of a thoracic chordoma. Eur Radiol. 1998;8:1677–1679
  36. Bergh P, Kindblom LG, Gunterberg B, et al. Prognostic factors in chordoma of the sacrum and mobile spine: a study of 39 patients. Cancer. 2000;88:2122–2134
  37. Soyer J, Le Carrou T, Wager M, et al. Difficulty in treating spinal chordoma of the thoracic vertebrae. Neurochirurgie. 2001;47:69–71
  38. Crapanzano JP, Ali SZ, Ginsberg MS, et al. Chordoma: a cytologic study with histologic and radiologic correlation. Cancer. 2001;93:40–51
  39. Husain F. Chordoma of the thoracic spine. Report of a case. J Bone Joint Surg Br. 1960;42-B:560–564
  40. Ahrendt MN, Wesselhoeft CW. Chordoma presenting as a posterior mediastinal mass in a pediatric patient. J Pediatr Surg. 1992;27:1515–1518
  41. Cesinaro AM, Maiorana A, Collina G, et al. Extra-axial chordoma. Report of a case with immunohistochemical study. Pathologica. 1993;85:755–760
  42. Taki S, Kakuda K, Kakuma K, et al. Posterior mediastinal chordoma: MR imaging findings. AJR Am J Roentgenol. 1996;166:26–27
  43. Catalano O, Sandomenico F, Nunziata A. Giant mediastinal chordoma. Description of a case. Radiol Med (Torino). 2000;99:390–391
  44. Mahassini N, Bernoussi Z, Toubi F, et al. Chordoma: atypical mediastinal mass. Report of a case. Rev Pneumol Clin. 2001;57:427–430
  45. Huang SM, Chen CC, Chiu PC, et al. Unusual presentation of posterior mediastinal chordoma in a 2-year-old boy. J Pediatr Hematol Oncol. 2003;25:743–746
  46. Suster S, Moran CA. Chordomas of the mediastinum: clinicopathologic, immunohistochemical, and ultrastructural study of six cases presenting as posterior mediastinal masses. Hum Pathol. 1995;26:1354–1362
  47. Roddie M, Adam A, Lambert H, et al. Case report 516: lumbar vertebral chordoma causing sclerosis of affected vertebra (3rd lumbar vertebra). Skeletal Radiol. 1989;17:611–613
  48. Erdem E, Angtuaco EC, Van Hemert R, et al. Comprehensive review of intracranial chordoma. Radiographics. 2003;23:995–1009
  49. D'Haen B, De Jaegere T, Goffin J, et al. Chordoma of the lower cervical spine. Clin Neurol Neurosurg. 1995;97:245–248
  50. Ng WK, Tang V. Crush preparation findings of “sarcomatoid” chordoma of the sacrum: report of a case with histologic, immunohistochemical, and ultrastructural correlation. Diagn Cytopathol. 2001;25:406–410
  51. Fan F, Templeton K, Damjanov I. Epithelioid cellular chordoma of the sacrum: a potential diagnostic problem. Ann Diagn Pathol. 2005;9:139–412
  52. Losi L, Tomasich G. Dedifferentiated (sarcomatoid) chordoma in the base of the skull. Report of a case. Pathologica. 1996;88:514–518
  53. Morimitsu Y, Aoki T, Yokoyama K, et al. Sarcomatoid chordoma: chordoma with a massive malignant spindle-cell component. Skeletal Radiol. 2000;29:721–725
  54. Ghadially FN. In: Diagnostic electron microscopy of tumours. 2nd ed.. London: Butterworths; 1985;p. 308–313
  55. Erlandson RA. In: Diagnostic transmission electron microscopy of tumors. New York (NY): Raven Press; 1994;p. 324–327
  56. Dickersin GR. In: Diagnostic electron microscopy. A text/Atlas. 2nd ed.. New York (NY): Springer-Verlag; 2000;p. 604–612
  57. Wojno KJ, Hruban RH, Garin-Chesa P, et al. Chondroid chordomas and low-grade chondrosarcomas of the craniospinal axis. An immunohistochemical analysis of 17 cases. Am J Surg Pathol. 1992;16:1144–1152
  58. Dahlin DC, Beabout JW. Dedifferentiation of low-grade chondrosarcomas. Cancer. 1971;28(2):461–466
  59. McCarthy EF, Dorfman HD. Chondrosarcoma of bone with dedifferentiation: a study of eighteen cases. Hum Pathol. 1982;13:36–40
  60. Debernardi L. Cordoma sarcomatoso del sacro—contributo alla conoscenza istologica e clinica dei tumori di origine cordale. Arch Sci Med (Torino). 1913;37:454–456
  61. Davison C, Weil A. Malignant chordoma of the lumbar region. Arch Neurol Psychyatry. 1928;19:415–423
  62. Mabrey RE. Chordoma. A study of 150 cases. Am J Cancer. 1935;25:501–517
  63. Poppen JL, King AB. Chordoma: experience with thirteen cases. J Neurosurg. 1952;9:139–163
  64. Knechtges TC. Sacrococcygeal chordoma with sarcomatous features (spindle cell metaplasia). Am J Clin Pathol. 1970;53:612–616
  65. Makek M, Leu HJ. Malignant fibrous histiocytoma arising in a recurrent chordoma. Case report and electron microscopic findings. Virchows Arch A Pathol Anat Histol. 1982;397:241–250
  66. Miettinen M, Lehto VP, Virtanen I. Malignant fibrous histiocytoma within a recurrent chordoma. A light microscopic, electron microscopic, and immunohistochemical study. Am J Clin Pathol. 1984;82:738–743
  67. Halpern J, Kopolovic J, Catane R. Malignant fibrous histiocytoma developing in irradiated sacral chordoma. Cancer. 1984;53:2661–2662
  68. Kishikawa H, Tanaka K. Chordoma—report of an autopsy case with fibrosarcoma. Acta Pathol Jpn. 1974;24:299–308
  69. Belza MG, Urich H. Chordoma and malignant fibrous histiocytoma. Evidence for transformation. Cancer. 1986;58:1082–1087
  70. Rone R, Ramzy I, Duncan D. Anaplastic sacrococcygeal chordoma. Fine needle aspiration cytologic findings and embryologic considerations. Acta Cytol. 1986;30:183–188
  71. Miettinen M, Karaharju E, Jarvinen H. Chordoma with a massive spindle-cell sarcomatous transformation. A light- and electron-microscopic and immunohistological study. Am J Surg Pathol. 1987;11:563–570
  72. Smith J, Reuter V, Demas B. Case report 576. Anaplastic sacrococcygeal chordoma (dedifferentiated chordoma). Skeletal Radiol. 1989;18:561–564
  73. Hruban RH, May M, Marcove RC, et al. Lumbo-sacral chordoma with high-grade malignant cartilaginous and spindle cell components. Am J Surg Pathol. 1990;14:384–389
  74. Tomlinson FH, Scheithauer BW, Forsythe PA, et al. Sarcomatous transformation in cranial chordoma. Neurosurgery. 1992;31:13–18
  75. Fukuda T, Aihara T, Ban S, et al. Sacrococcygeal chordoma with a malignant spindle cell component. A report of two autopsy cases with a review of the literature. Acta Pathol Jpn. 1992;42:448–453
  76. Fleming GF, Heimann PS, Stephens JK, et al. Dedifferentiated chordoma. Response to aggressive chemotherapy in two cases. Cancer. 1993;72:714–718
  77. Choi YJ, Kim TS. Malignant fibrous histiocytoma in chordoma-immunohistochemical evidence of transformation from chordoma to malignant fibrous histiocytoma. Yonsei Med J. 1994;35:239–243
  78. Ikeda H, Honjo J, Sakurai H, et al. Dedifferentiated chordoma arising in irradiated sacral chordoma. Radiat Med. 1997;15:109–111
  79. Woenckhaus J, Benedix P, Holotiuk O, et al. Sacrococcygeal chordoma with malignant spindle cell components. Case report and review of the literature. Pathologe. 1997;18:395–400
  80. Saito A, Hasegawa T, Shimoda T, et al. Dedifferentiated chordoma: a case report. Jpn J Clin Oncol. 1998;28:766–771
  81. Layfield LJ, Liu K, Dodd LG, et al. “Dedifferentiated” chordoma: a case report of the cytomorphologic findings on fine-needle aspiration. Diagn Cytopathol. 1998;19:378–381
  82. Munshi HG, Merajver SD, Valdez R, et al. Metastatic dedifferentiated chordoma with elevated beta-hCG: a case report. Am J Clin Oncol. 2002;25:274–276
  83. Sahasrabudhe NS, Jadhav MV, Holla VV. Dedifferentiated chordoma—a case report. Indian J Pathol Microbiol. 2002;45:353–354
  84. Sefiani S, Amarti A, Maher M, et al. Dedifferentiated chordoma of the skull base. A case report. Neurochirurgie. 2002;48:436–439
  85. Gil Z, Fliss DM, Voskoboinik N, et al. Cytogenetic analysis of three variants of clival chordoma. Cancer Genet Cytogenet. 2004;154:124–130
  86. Rosenberg AE, Brown GA, Bhan AK, et al. Chondroid chordoma—a variant of chordoma. A morphologic and immunohistochemical study. Am J Clin Pathol. 1994;101:36–41
  87. Brooks JJ, LiVolsi VA, Trojanowski JQ. Does chondroid chordoma exist?. Acta Neuropathol (Berl). 1987;72:229–235
  88. Walker WP, Landas SK, Bromley CM, et al. Immunohistochemical distinction of classic and chondroid chordomas. Mod Pathol. 1991;4:661–666
  89. Rutherfoord GS, Davies AG. Chordomas—ultrastructure and immunohistochemistry: a report based on the examination of six cases. Histopathology. 1987;775–787
  90. Meis JM, Giraldo AA. Chordoma. An immunohistochemical study of 20 cases. Arch Pathol Lab Med. 1988;112:553–556
  91. Brooks JJ, Trojanowski JQ, LiVolsi VA. Chondroid chordoma: a low-grade chondrosarcoma and its differential diagnosis. Curr Top Pathol. 1989;80:165–181
  92. Reith JD, Bauer TW, Fischler DF, et al. Dedifferentiated chondrosarcoma with rhabdomyosarcomatous differentiation. Am J Surg Pathol. 1996;20:293–298
  93. Shanks JH, Banerjee SS, Eyden BP. Focal rhabdomyosarcomatous differentiation in primary liposarcoma. J Clin Pathol. 1996;49:770–772
  94. Eusebi V, Damiani S, Pasquinelli G, et al. Small cell neuroendocrine carcinoma with skeletal muscle differentiation: report of three cases. Am J Surg Pathol. 2000;24:223–230

PII: S1092-9134(06)00126-2

doi: 10.1016/j.anndiagpath.2006.09.002

Annals of Diagnostic Pathology
Volume 11, Issue 4 , Pages 262-273 , August 2007