Review of current treatment of sacral chordoma

Abstract

Chordoma is a relatively rare, locally aggressive tumor which is known to arise from embryonic remnants of the notochord and to occur exclusively along the spinal axis, with a predilection for the sacrum. Although chordoma typically presents as a single lesion, a few cases of metastasis have been reported and the prognosis of such patients may be poor. Chordomas are slowly growing tumors with insidious onset of symptoms, making early diagnosis difficult. Recent improvements in imaging have provided valuable information for early diagnosis. The optimal treatment for sacral chordoma is en bloc sacral resection with wide surgical margins. Improvement in surgical techniques has widened the opportunities to provide effective treatment. However, the effects of adjuvant treatment options are still both unclear and controversial. Substantial progress has been made in the study of molecular-targeted therapy. The authors review the current surgical and adjuvant treatment modalities, including molecular-targeted therapy, available for management of sacral chordoma.

Figure 1

(a) and (b): Preoperative CT image of a 54‐year‐old man shows a sacral chordoma, with lytic bone destruction of the osseous pelvis. (c) and (d): Preoperative T₁‐weighted MR image shows the tumor affecting the entire sacrum with a soft tissue mass covered anteriorly by the presacral fascia.

Figure 2

(a): Anteroposterior radiographs and (b) three‐dimensional model obtained after implantation of the improved Galveston L‐rod. (c): Three‐dimensional model of patient's pelvis with the special prosthesis, and (d) postoperative radiograph showing well‐incorporated grafts at the pelvic sites and unchanged stable position of the prosthesis.