Sacral chordoma: retrospective review of II surgically treated cases

Purpose of the study Chordoma is a malignant neoplasm believed to arise from notochord remnants. It accounts for approximately 3 to 4 p. 100 of primary bone tumors and is localized along the axial skeleton, 50 p. 100 being sacrococcygeal. Clinica l, radiographical and histological findings have been well established sinc e the first description by Ribbert in 1894. Sacral chordomas are however di fficult to manage and remain a challenge for surgeons and radiotherapists a like. The purpose of this study was to evaluate the long-term results of su rgical treatment and patterns of failure in patients treated for chordoma o f the sacrum in our department. Materials and methods This retrospective study included 11 cases of sacral chordomas treated from 1973 to 1998. Patient age ranged from 36 to 77 years (mean 59 years). Six patients were female and five male. The initial treatment was surgery in al l cases including intralesional removal in two cases, marginal resection in seven and complete en bloc resection rn two. Results Median follow-up was 6 years (1 month to 14 years). Tumoral recurrences wer e observed in nine cases 5 months to 8 years after treatment. In two cases, recurrence was observed 8 years after radical sacrectomy. Treatment of rec urrences was partial surgical removal with radiotherapy (40 to 70 Grays). T hree patients developed metastases in lungs, liver and bone, respectively. Seven patients died, two from metastatic disease. The 5-year overall surviv al was 64 p. 100 but only 18 p. 100 of the patients survived 10 years. Aver age disease-free survival was 18 p. 100 at 5 years and 0 p. 100 at 10 years . Discussion Chordoma is a slow-growing tumor allowing survival for several years despit e recurrent disease. However, only 10 to 20 p. 100 of the patients survive free of disease at 5 years. Recurrences are frequent (45 to 80 p. 100) and often multiple. Chordoma inevitably recurs and eventually leads to death af ter intralesional removal or marginal resection. Radical surgery should be attempted whenever technically feasible. When performed early, particularly for smaller lesions, it offers the best chance for cure. However, tumoral recurrence can occur postoperatively despite a macroscopically complete res ection. Because radiation therapy seems to be more successful in controllin g microscopic disease, it should be considered as a pre- or postoperative a djuvant to a macroscopically complete resection.