Characteristics and Patterns of Metastatic Disease from Chordoma
Victoria A. Young,
Kevin M. Curtis,
H. Thomas Temple,
Frank J. Eismont,
Thomas F. DeLaney,
Francis J. Hornicek
Affiliations
Victoria A. Young
Department of Orthopaedics, University of Miami Miller School of Medicine, P.O. Box 016960 Miami, FL 33101, USA
Kevin M. Curtis
Department of Biochemistry & Molecular Biology, University of Miami Miller School of Medicine, P.O. Box 016960 Miami, FL 33101, USA
H. Thomas Temple
Department of Orthopaedics, University of Miami Miller School of Medicine, P.O. Box 016960 Miami, FL 33101, USA
Frank J. Eismont
Department of Orthopaedics, University of Miami Miller School of Medicine, P.O. Box 016960 Miami, FL 33101, USA
Thomas F. DeLaney
Department of Radiation Oncology, Massachusetts General Hospital, Harvard Medical School, Francis H. Burr Proton Therapy Center (FHBPTC), 55 Fruit Street, Boston, MA 02114, USA
Francis J. Hornicek
Department of Orthopedic Surgery, Massachusetts General Hospital, Harvard Medical School, Orthopaedic Associates, 55 Fruit Street Yawkey 3B, Boston, MA 02114, USA
Chordoma is a rare, slow-growing malignant tumor arising from notochordal remnants. A retrospective review of patient records at two major referral centers was undertaken to assess the incidence, location, and prognostic factors of metastatic disease from chordoma. 219 patients with chordoma (1962–2009) were identified. 39 patients (17.8%) developed metastatic disease, most frequently to lung (>50%). Median survival from the time of initial diagnosis was 130.4 months for patients who developed metastatic disease and 159.3 months for those who did not (P=0.05). Metastatic disease was most common in the youngest patients (P=0.07), and it was 2.5 times more frequent among patients with local recurrence (26.3%) than in those without (10.8%) (P=0.003). Patient survival with metastatic disease was highly variable, and it was dependent on both the location of the tumor primary and the site of metastasis. Metastasis to distal bone was the most rapid to develop and had the worst prognosis.