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.

Extraskeletal osteosarcoma: A European Musculoskeletal Oncology Society study on 266 patients

“…of patients (localised) Outcome Lee (1995) [8] 40 5-year OS 37% Ahmad (2002) [6] 60 (38) 5-year DFS 46% (localised) Choi (2014) [9] 53 (42) 3-year OS 61% (localised) Thampi (2014) [10] 256 ( Overall survival (OS) was calculated from diagnosis to death or last follow up, disease-free survival (DFS) was calculated from date of surgery to relapse/progression or last follow up by the KaplaneMeier method. The time scale was extended to the last follow up if none of these events were observed.…”

Biological roles of microRNA-140 in tumor growth, migration, and metastasis of osteosarcoma in vivo and in vitro

“…There were 26 males and 21 females among the 47 cases (age, 9 to 35 years; average age, 17.15±7.26 years). There were 19 cases with tumor diameter less than 10 cm and 28 cases more than 10 cm [15]. According to Enneking classification, there were 17 intracompartmental patients and 30 extracompartmental patients.…”

“…OS is the fifth most common malignancy in the age group of [15][16][17][18][19], and the second most common disease in adolescents after lymphoma. OS incidence in general population is 2-3 million per year, but an incidence peak between 15-19 years of age is reported at 8-11 million per year [2].…”

Tumor lysis syndrome in an extraskeletal osteosarcoma: a case report and review of the literature

“…It accounts for less than 4% of all osteosarcomas and approximately 1 to 2% of all soft tissue sarcomas (STSs) [5]. The clinical features of patients with this tumor differ significantly from the clinical features of patients with skeletal osteosarcoma, including older age, propensity for axial tumors, and female preponderance.…”

“…Such tumors may reach an enormous size before detection, because the enlarging mass may not be associated with pain. ESOS may be one of the differential diagnoses to be considered in the case of calcified masses arising in retroperitoneal space [5, 6]. …”