Current Status of the Treatment for Sacral Chordomas and its Future Trends

Abstract

Chordomas are rare slow-growing, low to intermediate-grade malignant neoplasms (less than 5% of all primary bone neoplasms); however they sometimes metastasise to distant organs. The 10-year overall survival rate is relatively low. The sacrococcygeal region is one of the most common sites. Intensive surgery is the first line for the treatment, since chordomas are resistant to chemotherapy. In turn, radiotherapy presents marginal effect. It has been reported that surgical margins govern local recurrence and survival. However, sacral chordomas represent a therapeutic challenge as a large number of sacral chordomas are often very large at the time of diagnosis and may comprise adjacent vital organs. Sacrec- tomy includes four surgical approaches; i.e., a combined sequential anterior and posterior approach, a combined simulta- neous anterior and posterior approach, a posterior approach alone and combined extended ilioinguinal and posterior ap- proaches. Because of its large invasion, various postoperative complications were considered. Bladder, bowel and sexual dysfunction and wound complications were major and important complications. The efficacy of adjuvant therapy for the treatment of sacral chordoma is under debate. Chemotherapy has not significant benefit to local control as well as overall survival in patients with chordomas. Standard dose of radiotherapy is not effective for chordomas, either. Some authors described that Hadron-based therapy has several advantages compared with conventional photon-based therapy. Recently some authors described efficacy of new molecular-targeting agents. The infrequency of this disease has prevented to es- tablish the optimal treatment. In this review, we summarized accumulating knowledge of the treatment for sacral chordo- mas and discuss future trends.