BACKGROUND. Chordomas are rare neoplasms that arise from the notochord remnant. They develop in the sacrococcygeal (50%) or cervical (15%) region and are generally regarded as a locally aggressive tumor with a slow progressive growth rate and a metastatic incidence ranging from 3 to 48%. Skin involvement by chordoma is rare, but can occur by direct extension, by local recurrence and by metastases. OBJECTIVE. To illustrate by a case report the clinical presentation and management of this disease. METHODS. We present a case of sacral chordoma with metastases over a 10-year period to the lungs, the soft tissue of the chest wall, the triceps tendon, and distant cutaneous metastases to the back and the nose. RESULTS. The cutaneous metastases were treated by excision. CONCLUSION. Chordoma is a slow growing tumor of the notochord remnant that may metastasize to the skin. Physicians and pathologists should be aware of this entity.
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