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Sarcoma. 2015;2015:517657. doi: 10.1155/2015/517657. Epub 2015 Dec 30.

Characteristics and Patterns of Metastatic Disease from Chordoma.

Author information

1
Department of Orthopaedics, University of Miami Miller School of Medicine, P.O. Box 016960 Miami, FL 33101, USA; University of Miami Tissue Bank, 1951 NW 7th Avenue, Suite 200, Miami, FL 33136, USA.
2
Department of Biochemistry & Molecular Biology, University of Miami Miller School of Medicine, P.O. Box 016960 Miami, FL 33101, USA; Geriatric Research, Education, and Clinical Center and Research Service, Bruce W. Carter Veterans Affairs Medical Center, Miami, FL 33125, USA.
3
Department of Orthopaedics, University of Miami Miller School of Medicine, P.O. Box 016960 Miami, FL 33101, USA.
4
Department of Radiation Oncology, Massachusetts General Hospital, Harvard Medical School, Francis H. Burr Proton Therapy Center (FHBPTC), 55 Fruit Street, Boston, MA 02114, USA.
5
Department of Orthopedic Surgery, Massachusetts General Hospital, Harvard Medical School, Orthopaedic Associates, 55 Fruit Street Yawkey 3B, Boston, MA 02114, USA.

Abstract

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.

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