Objectives:
The purpose of this study is to assess the clinical characteristics and treatment modalities of 60 patients diagnosed with extremity soft tissue sarcomas of uncertain differentiation.
Methods:
Clinical findings, treatments, outcomes and treatment failures in a case series of 59 patients, who were diagnosed with localized or metastatic extremity soft tissue sarcomas of uncertain differentiation between 2002 and 2015, were examined retrospectively.
Results:
Out of 59 cases, 40 had synovial sarcoma, 7 had ASPS, 6 had extra-skeletal Ewing’s sarcoma, 3 had clear cell sarcoma, 2 had epithelioid sarcoma and 1 had extra-skeletal myxoid chondrosarcoma. Metastases were developed in 18 patients, whereas 42 patients had localized tumors at diagnosis. While 5-year overall survival rate was 36% in all patients, it was 46% in patients with localized tumor and 18% in patients who had metastasis at diagnosis. 23 patients died in 40.7 months on average (12-130). The mean diameter of the mass in all patients was 108±87 mm. The mean follow-up duration was 49.4±41.5 months (12-176).
Conclusions:
Uncertainly differentiated soft tissue sarcomas are a group of diseases that are similar to benign tumors and some are very rare and have a very poor long-term prognosis. Wide surgical margin and early diagnosis are especially important.
Keywords: Sarcoma of uncertain differentiation, synovial sarcoma of the extremities, soft tissue sarcoma of the extremities, treatment outcome, prognosis