ABSTRACT

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.

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.

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).

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