Chordomas are slow-growing, malignant tumors of bone that are thought to be derived from the primitive notochord and occur almost exclusively in the axial skeleton. The so-called extra-axial chordoma has been shown to demonstrate identical features to the classic chordoma, except that it is found outside the axial skeleton. Only six cases of extra-axial chordoma have been reported in the literature to date. In this report, we present another case of extra-axial chordoma for the first time originating from the lung parenchyma. A 79-year-old man presented a 7.3-cm-sized cavitary lung mass. Pathologic examination, including immunohistochemical studies, revealed that the mass was a chordoma. We report an extra-axial chordoma for the first time presenting as a lung mass.

1.
Fechner RE, Mills SE: Tumors of the bones and joints; in Rosai J, Sobin L (eds): Atlas of Tumor Pathology. Washington, Armed Forces Institute of Pathology, 1993, pp 239–243.
2.
Mindell ER: Current concept review: chordoma. J Bone Joint Surg 1981;63:501–505.
3.
Dahlin DC, MacCarty CS: Chordoma: a study of fifty-nine cases. Cancer 1952;5:1170–1178.
4.
Povysil C, Matejovsky Z: A comparative ultrastructural study of chondrosarcoma, chordoid sarcoma, chordoma and chordoma periphericum. Pathol Res Pract 1985;179:546–559.
5.
Miettinen M, Gannon FH, Lackman R: Chordoma-like soft tissue sarcoma in the leg: a light and electron microscopic and immunohistochemical study. Ultrastruct Pathol 1992;16:577–586.
6.
Cesinaro AM, Maiorana A, Collina G, Fano RA: Extra-axial chordoma. Report of a case with immunohistochemical study. Pathologica 1993;85:755–760.
7.
Carstens PHB: Chordoid tumor: a light, electron microscopic, and immunohisto-chemical study. Ultrastruct Pathol 1995;19:291–295.
8.
Nielsen GP, Mangham DC, Grimer RJ, Rosenberg AE: Chordoma periphericum. Am J Surg Pathol 2001;25:263–267.
9.
Salisbury JR: The pathology of the human notochord. J Pathol 1993;171:253–255.
10.
DiFrancesco LM, Davanzo Castillo CA, Temple WJ: Extra-axial chordoma. Arch Pathol Lab Med 2006;130:1871–1874.
11.
Selvaraj A, Wood AJ: Superior mediastinal chordoma presenting as a bilobed paravertebral mass. Eur J Cardiothorac Surg 2003;23:248–250.
12.
Crapanzano JP, Ali SZ, Ginsberg MS, Zakowski MF: Chordoma: a cytologic study with histologic and radiologic correlation. Cancer 200;93:40–51.
13.
Folpe AL, Agoff SN, Willis J, Weiss SW: Parachordoma is immunohistochemically and cytogenetically distinct from axial chordoma and extraskeletal myxoid chondrosarcoma. Am J Surg Pathol 1999;23:1059–1067.
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