title: Clinical Features and Outcomes Differ between Skeletal and Extraskeletal Osteosarcoma.
abstract: Background. Extraskeletal osteosarcoma (ESOS) is a rare subtype of osteosarcoma. We investigated patient characteristics, overall survival, and prognostic factors in ESOS. Methods. We identified cases of high-grade osteosarcoma with known tissue of origin in the Surveillance, Epidemiology, and End Results database from 1973 to 2009. Demographics were compared using univariate tests. Overall survival was compared with log-rank tests and multivariate analysis using Cox proportional hazards methods. Results. 256/4,173 (6%) patients with high-grade osteosarcoma had ESOS. Patients with ESOS were older, were more likely to have an axial tumor and regional lymph node involvement, and were female. Multivariate analysis showed ESOS to be favorable after controlling for stage, age, tumor site, gender, and year of diagnosis [hazard ratio 0.75 (95% CI 0.62 to 0.90); p = 0.002]. There was an interaction between age and tissue of origin such that older patients with ESOS had superior outcomes compared to older patients with skeletal osteosarcoma. Adverse prognostic factors in ESOS included metastatic disease, larger tumor size, older age, and axial tumor site. Conclusion. Patients with ESOS have distinct clinical features but similar prognostic factors compared to skeletal osteosarcoma. Older patients with ESOS have superior outcomes compared to older patients with skeletal osteosarcoma.
authors.affiliation: Department of Pediatrics, UCSF School of Medicine and UCSF Benioff Children's Hospital, 505 Parnassus Avenue M649, P.O. Box 0106, San Francisco, CA 94143, USA.
authors.forename: Sheila
authors.initials: S
authors.lastname: Thampi
authors.affiliation: Department of Pediatrics, UCSF School of Medicine and UCSF Benioff Children's Hospital, 505 Parnassus Avenue M649, P.O. Box 0106, San Francisco, CA 94143, USA.
authors.forename: Katherine K
authors.initials: KK
authors.lastname: Matthay
authors.affiliation: Department of Medicine and Epidemiology and Biostatistics, UCSF School of Medicine 505 Parnassus Avenue, San Francisco, CA 94143, USA.
authors.forename: W John
authors.initials: WJ
authors.lastname: Boscardin
authors.affiliation: Department of Pediatrics, UCSF School of Medicine and UCSF Benioff Children's Hospital, 505 Parnassus Avenue M649, P.O. Box 0106, San Francisco, CA 94143, USA.
authors.forename: Robert
authors.initials: R
authors.lastname: Goldsby
authors.affiliation: Department of Pediatrics, UCSF School of Medicine and UCSF Benioff Children's Hospital, 505 Parnassus Avenue M649, P.O. Box 0106, San Francisco, CA 94143, USA.
authors.forename: Steven G
authors.initials: SG
authors.lastname: DuBois