Chordoma metastatic to skin: A report of two cases and a brief review of the literature

Chordomas are rare, locally aggressive tumors of notochordal origin usually arising in the spine or base of the skull. Skin involvement is rare and typically occurs via direct extension of the primary tumor to the skin. Although there are increasing reports of the skin being involved as a distant metastatic site in patients with chordoma, this remains an exceedingly rare occurrence. We present two cases of patients diagnosed with metastasis of chordoma to the skin that represented distant metastasis. In the first case, a patient with a primary thoracic/lumbar chordoma presented with isolated metastasis to the skin of the left lower jaw 9 years after initial diagnosis of the chordoma. To our knowledge, this is the first reported case of a chordoma of this site to develop distant skin metastasis. In the second case, a patient with a primary sacral chordoma presented with metastasis to the skin of the right side of his chin 6 years after initial diagnosis of the chordoma, following previous metastatic spread to the liver and lung. Finally, we briefly review the literature on chordoma metastasis to the skin and highlight salient features to raise awareness of this uncommon occurrence.