Abstract
BackgroundSynovial sarcoma is a high-grade, malignant soft tissue sarcoma (STS) considered as relatively chemosensitive tumor, with particular sensitivity to ifosfamide. The aim of this prospective study was to analyze outcomes of patients (pts) with localized synovial sarcoma treated in a single institution with uniform neo- and adjuvant combined therapy protocol. MethodsOne hundred and seventy one pts (96 women and 75 men) with localized synovial sarcoma were treated at our institution between 1997 and 2014. Chemotherapy consisted of 4 cycles of ifosfamide 12g/m2 (2 cycles given preoperatively) and two cycles of doxorubicin-based regimen 75mg/m2. Most pts received neoadjuvant hypofractionated radiation therapy followed by immediate surgery. At a median follow-up of 114 months (range, 3 to 244 months) 69 deaths were recorded. ResultsMedian age at diagnosis was 33 years (range 17-69). The most common localization was lower limb (n=121), followed by upper limb (n=32). Tumors larger than 5cm in size were found in 70% of pts (median 8cm; range 2 – 30cm). Seventy-seven cases (45%) had primary diagnosed tumor. Sixty-four (37%) pts were referred to our institute after unplanned tumor excision and 30 (18%) pts because of clinical local recurrence. At the time of analysis, 36 (21%) pts had local recurrence (only 8% of pts with primary tumor) and 76 (44%) pts developed metastases. Median disease-free survival was 82 months. The 5-year overall survival (OS), local recurrence-free survival (LRFS) and distant recurrence-free survival (DRFS) rates were 74%, 80% and 60%, respectively. By univariate analysis, unplanned tumor excision (p=0.0025) and positive margins (p=0.048) were related to higher risk of local recurrence. In multivariable Cox’s regression, age >35 years (p=0.045), male sex (p=0.001), stage IIIB (p<0.01) and histology other than monophasic (p=0.033) were associated with worse OS. ConclusionsIn adult patients with localized synovial sarcoma, a long-term survival and local control can be achieved with intensive combined therapy. Our results confirms the importance of planned surgery and clear surgical margins for local control. We have demonstrated that age, sex, disease stage, and histology are independent prognostic factors of overall survival. Legal entity responsible for the studyMaria Sklodowska-Curie Institute - Oncology Center, Warsaw, Poland. FundingHas not received any funding. DisclosureH.M. Kosela Paterczyk: Honoraria (institution): Novartis; Honoraria (self): MSD; Honoraria (self): BMS. P. Rutkowski: Honoraria (self), Advisory / Consultancy, Speaker Bureau / Expert testimony: MSD; Honoraria (self), Advisory / Consultancy, Speaker Bureau / Expert testimony: Novartis; Honoraria (self), Advisory / Consultancy, Speaker Bureau / Expert testimony, Research grant / Funding (institution): BMS; Honoraria (self): Roche; Honoraria (self), Speaker Bureau / Expert testimony: Pfizer; Honoraria (self), Speaker Bureau / Expert testimony: Eli Lilly; Advisory / Consultancy: Blueprint Medicines. All other authors have declared no conflicts of interest.
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