Portal de Periódicos da CAPES

Extraskeletal Osteosarcoma

2014; Lippincott Williams & Wilkins; Volume: 39; Issue: 1 Linguagem: Inglês

10.1097/coc.0000000000000005

1537-453X

Terence T. Sio, Charles C. Vu, Schoeb Sohawon, Paul Van Houtte, Juliette Thariat, Paul J. Novotny, Robert C. Miller, Gil Bar‐Sela,

Cardiac tumors and thrombi

To report characteristics, treatment, and outcomes for an international cohort of patients with extraskeletal osteosarcoma (ESOS).Through the Rare Cancer Network, retrospective data on patients with ESOS were collected. Patient characteristics, multimodality treatment information, and survival status were analyzed.Thirty-seven patients in 4 health care institutions were identified. Thirty-one (86%) patients had grade 3 or 4 tumors. Most patients (27 [73%]) had stage III disease. Fourteen (38%) received neoadjuvant chemotherapy or chemoradiation. Of 28 (85%) who underwent surgery, 21 (75%) had free margins achieved and 15 (41%) subsequently received adjuvant chemotherapy. At median follow-up of 45 months, 20 (55%) patients were alive, 13 (43%) of whom were disease free. Univariate analysis showed that poor overall survival was related to stage IV (P<0.001), no surgery (P 10 cm (P=0.002), and age (P=0.002). In multivariate analysis, primary size >10 cm (P=0.005) was prognostic for overall survival. For patients without metastases, univariate analysis showed disease-free survival (DFS) related to primary size >10 cm (P=0.003), surgery (P=0.004), local recurrence (P=0.003), and age (P 10 cm (P=0.01) and older age (P<0.001) were significant for worse outcome.Multimodality treatment remains standard for localized ESOS, with indications for neoadjuvant therapy less clear. Larger tumor size and older age were prognostic of poorer DFS.