Chordoma : Prognosis

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Chordoma - Prognosis: Chordoma is a slow-growing but locally aggressive tumor with a poor prognosis, depending upon the tumor location, size, and resectability. The natural history is frequently characterized by multiple local recurrences followed by fatal outcome. Death is usually due to local effects of the tumor, such as blockage of the genitourinary tract or gastrointestinal tract, rather than due to distant metastases.

Recurrences occur in the form of multiple nodules at the site of previous resection and can develop a decade or so after initial therapy. Metastases occur via hematogenous route in 30-40% of cases to sites such as lungs (most common), skin and subcutaneous tissues, liver, lymph nodes, and cerebrospinal fluid. Metastases to skin can mimic sweat gland tumors.

Sacral chordomas can often be completely removed with negative margins and have the best outcomes. The 5-yr and 10-yr survival rates range from 60-95% and 40-60% respectively.

Skull base and spine tumors are often difficult to resect completely and recur in 60-75% of cases. The 5-yr and 10-yr survival rates for skull base chordomas are 63-78% and 16-32% respectively.

De-differentiated chordomas have the worst prognosis and are usually rapidly fatal within a few months of diagnosis.