1. ¹Department of Pathology, Koshigaya Hospital, Dokkyo University School of Medicine, Minami-Koshigaya, Koshigaya, Saitama, Japan
2. ²Department of Pathology, Dokkyo University School of Medicine, Mibu, Tochigi, Japan
3. ³Department of Anatomic and Diagnostic Pathology, Dokkyo University School of Medicine, Mibu, Tochigi, Japan
4. ⁴Dokkyo University School of Medicine, Mibu, Tochigi, Japan

Correspondence: Dr T Yamaguchi, MD, PhD, Department of Clinical Pathology, Sapporo Medical University School of Medicine, S1W16, Chuo-ku, Sapporo 060-8543, Japan. E-mail: takehiko@sapmed.ac.jp

Received 26 July 2004; Revised 27 December 2004; Accepted 27 December 2004; Published online 1 April 2005.

Abstract

Chordomas are rare malignant bone tumors primarily involving both ends of the axial skeleton that present as destructive bone lesions with a large soft tissue mass. Chordomas were previously believed to arise from notochordal remnants. However, recent studies suggest the possibility that chordomas arise from benign notochordal cell tumors. We present two cases of coccygeal incipient chordoma that strengthen the new hypothesis. The first case was an 83-year-old man who died of prostatic adenocarcinoma. The second case was a 79-year-old man who died of hepatocellular carcinoma. The coccygeal tumors were composed of intraosseous and extraosseous infiltrative lesions. The intraosseous lesions consisted of both benign notochordal cell tumor and incipient chordoma. The extraosseous lesions were consistent with incipient chordoma. In addition, two other small benign notochordal cell tumors were found at a different level in case 1. It is conceivable that pre-existing intraosseous benign notochordal cell tumors transform into incipient chordoma and then extend through the cortex into the surrounding soft tissue. The incidence of incipient chordoma appears much higher than expected because chordomas are rare tumors with an incidence of one case per 1 000 000 persons per year. We suspect that unknown factors transform incipient chordoma into classic chordoma.