Surgical Treatment of Sacral Chordoma

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Introduction

Chordoma is a rare, indolent primary bone malignancy. It comprises 1%–4% of primary bone malignancies. The incidence is 0.08 in 100,000; it has a male predominance (∼2:1) and a peak presentation between 50 and 60 years. Chordoma, in originating from the notochord, occurs along the midline spinal axis from skull base to sacrum. Sacral cases account for one-third of all chordomas. Chordoma is the most common primary bone tumor of the sacrum comprising greater than 50% of all cases. Survival is poor (72% and 48% at 5 and 10 years, respectively) and dependent primarily upon en bloc surgical resection.

Evidence for Surgical Resection

The Enneking principles of musculoskeletal oncology suggest wide en bloc resection of chordoma as the preferred surgical strategy; however, this can be difficult even in the hands of the most experienced surgeons. The surgical goal of wide, en bloc resection is achieved in only 35%–81% of cases, because of sacral region anatomy, tumor size, and preference for maintaining neurological function. In a recent multicenter study, including 167 patients with sacral chordoma, the median duration of local recurrence–free survival was 4 years, with 57 patients suffering local recurrence during the follow-up period. Both previous surgery and intralesional resection were identified as predictors of local recurrence. The median duration of overall survival with a sacral chordoma is 6 years; increasing age and motor deficit predict shorter survival. Previously intralesional resection has been associated with higher local recurrence. In a retrospective, single institution experience, 43 patients with sacral chordoma underwent surgical resection. All four patients with only intralesional resection developed local recurrence and metastases, and then died of their disease. In contrast of 27 patients undergoing wide surgical excision, only 9 experienced local recurrence during the follow-up period.

Functional outcome after sacrectomy is related to the level of the nerve roots sacrificed for midline tumors: low sacral (sacrifice of at least one S4 nerve root), midsacral (sacrifice of at least one S3 nerve root), high sacral (sacrifice of at least one S2 nerve root), total (sacrifice of at least one S1 nerve root), and hemicorporectomy (translumbar amputation). In general, patients with a sacral amputation distal to the S3 nerve root (low sacral) experience limited deficits. Patients who undergo a mid- or high sacral resection experience bowel and bladder dysfunction with severity dependent on the level of sacral amputation. Total sacrectomy results in motor deficit and loss of sphincter control and sexual ability, if both S1 nerves are sacrificed. The level of sacrectomy also correlates with operative morbidity and length of hospital stay.