Multicentric Chordoma

Excerpt

Chordoma is a rare neoplasm of the neuraxis, long believed to arise from notochord remnants. Its distribution has principally thought to favor the sacrococcygeal region, followed by the skull base and then elsewhere discretely along the spine. Although recent data may challenge this pattern, even more infrequently chordomas are seen arising simultaneously at multiple points along the neuraxis [1]. Very few cases of multicentric chordoma have been reported. A more diffuse pattern complicates treatment strategies for this slowly growing, locally recurrent neoplasm that has historically not responded well to chemotherapy. Recent efforts have sought to better explain the inciting variables that lead to this disease and further efforts will hopefully clarify the cause for the exceedingly rare, diffuse presentation, as well as new molecularly based treatment strategies. …