BACKGROUND:
Sacral chordoma is a rare, low to intermediate-grade tumor that poses substantial challenges in terms of timely diagnosis and adequate treatment. Few studies have examined the oncologic and functional outcomes of patients treated for sacral chordoma.

METHODS:
The clinical records of sixteen patients who had undergone sacrectomy for chordoma between 1985 and 2001 were evaluated retrospectively. All patients underwent resection by means of a sequential combined anterior and posterior approach. Patients were followed clinically at six-month intervals following recovery from the index surgical procedure. The disease onset, treatment, hospital stay, recurrence rates, survival, adjuvant therapy, functional outcome measures, and complications were evaluated.

RESULTS:
The average age at the time of diagnosis was sixty-one years. The mean tumor size was 15.2 cm in diameter, and all patients had a resection involving S1 or S2. The mean duration of follow-up was sixty-six months, and the tumor recurred in twelve of the sixteen patients. The mean time to metastasis was fifty months. Four patients were clinically disease-free at a mean follow-up of 94.5 months, while five patients died as a result of progressive local or metastatic disease at a mean follow-up of 31.4 months. Only one patient had normal bowel and bladder control postoperatively, and only three were able to walk without assistive devices. Eight patients had wound complications, and one patient had a deep-vein thrombosis. With the numbers available, neither negative margins at the time of initial tumor resection nor adjuvant radiation therapy had a significant impact on survival or local recurrence. More cephalad levels of resection were associated with significantly worse bowel (p = 0.01) and bladder (p = 0.01) control. Complications were frequent and were more common with a larger tumor size at the time of presentation (p = 0.034).

CONCLUSIONS:
The treatment of sacral chordoma is an arduous clinical undertaking that requires a multidisciplinary approach and attention to detail from the outset. Despite aggressive well-planned surgical management and adherence to strict surveillance protocols, frequent recurrence and the late onset of metastatic disease are to be expected in a substantial proportion of patients, especially those with a very large chordoma or one at a more cephalad level. Adequate surgical treatment results in substantial functional impairment and numerous complications; however, it does offer the possibility of long-term disease-free survival. We advocate an attempt at complete resection, when there is still a possibility of cure, and aggressive treatment of local recurrences.

LEVEL OF EVIDENCE:
Therapeutic Level IV. See Instructions to Authors for a complete description of levels of evidence.



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