Sacral chordoma is a low-grade malignancy, but its outcomes are poor due to recurrence, metastases and complications, making long-term survival rare. A man in his forties with intermittent low back pain for 2 years developed urinary retention. Digital rectal examination revealed a large mass in the anterior sacrum. The chordoma was diagnosed via biopsy. Surgery was performed via a combined anterior–posterior approach, preserving the S1 nerve roots and resecting the tumor with the rectum. Pathological findings revealed a chordoma with a few necrotic areas. Pulmonary metastasis developed without recurrence 18 years later, necessitating partial resection performed thoracoscopically. Low-grade histology identical to that of the primary tumor was confirmed. Radiotherapy was recommended, but the patient declined and was instead followed up. Five years after pulmonary metastasis was detected, the patient presented to our hospital with swelling and pain in the left chest wall. Tumor transplantation from a thoracoscopic biopsy site was suspected, and palliative radiotherapy consisting of 50 Gray/20 fractions was administered. The patient died 13 years after irradiation and 36 years after primary surgery, i.e., he lived a normal lifespan. To achieve long-term survival in patients with sacral chordoma by reducing recurrence and complications, we reviewed the literature on resection methods, improved resection margins, and nerve preservation.
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