Frezza AM, et al. - Researchers sought to report whether anthracycline-based regimens, gemcitabine-based regimens, and pazopanib are active in advanced epithelioid sarcoma (ES). Findings of this largest retrospective series of systemic therapy in ES suggest that anthracycline-based and gemcitabine-based regimens have a moderate activity in ES, with a similar response rate and progression-free survival (PFS). However, pazopanib seemed to have limited activity.

Methods

• For this retrospective analysis, data of patients with locally advanced/metastatic ES diagnosed between 1990 and 2016 was assessed from 17 sarcoma reference centers in Europe, the United States, and Japan.
• Researchers performed local pathological review in all cases to confirm diagnosis according to most recent criteria.
• Anthracycline-based regimens, gemcitabine-based regimens, or pazopanib was administered to all patients.
• RECIST was used to assess response.
• Kaplan-Meier method was used to determine PFS and overall survival (OS).
• Based on morphology, they determined classic and proximal subtypes (according to 2013 World Health Organization guidelines).

Results

• Overall, researchers included 115 patients [80 (70%) men and 35 (30%) women; median age 32 years (range, 15-77 years)].
• Anthracycline-based regimens was provided to 85, gemcitabine-based regimens to 41, and pazopanib to 18 with pazopanib.
• More than 1 treatment was received by 24 patients.
• Median follow-up was 34 months.
• With a median PFS of 6 months, anthracycline-based regimens led to response rate of 22%.
• Complete response (CR) was reported in one patient.
• They noted a trend toward a higher response rate in morphological proximal type (26%) vs classic type (19%) and in proximal vs distal primary site (26% vs 18%).
• Gemcitabine-based regimens led to the response rate of 27%, with 2 CR and a median PFS of 4 months.
• In this group, they identified a trend toward a higher response rate in classic vs proximal morphological type (30% vs 22%) and in distal vs proximal primary site (40% vs 14%).
• The pazopanib group displayed no objective responses, and median PFS of 3 months.