Anthracycline, gemcitabine moderately active in epithelioid sarcoma
Both anthracycline- and gemcitabine-based treatment regimens appeared moderately active in epithelioid sarcoma, according to a brief report published in JAMA Oncology.
“The prognosis in epithelioid sarcoma is serious, especially for proximal type, with a 5-year OS rate of 50%,” Anna Maria Frezza, MD, medical oncologist at Fondazione IRCCS Istituto Nazionale Tumori in Italy, and colleagues wrote. “The current knowledge on the activity of commonly used drugs for sarcoma in epithelioid sarcoma is based on limited retrospective studies. This is particularly relevant today, when new target agents potentially active in this disease are under evaluation.”
The researchers performed a retrospective analysis of 115 patients (median age, 32 years) diagnosed with locally advanced or metastatic epithelioid sarcoma between 1990 and 2016. The patients received treatment at 17 sarcoma reference centers in Europe, the United States and Japan. All patients underwent anthracycline-based (n = 85) or gemcitabine-based (n = 41) regimens, or pazopanib (Votrient, Novartis; n = 18). Twenty-four patients underwent more than one treatment.
Median follow-up was 34 years.
Anthracycline-based regimens conferred a response rate of 22% and a median PFS of 6 months. Only one patient experienced a complete response.
In this treatment group, research observed higher response rates among those with morphological proximal type disease compared with classic type (26% vs. 19%), and proximal site compared with distal primary site (26% vs. 18%).
Gemcitabine conferred a similar response rate to anthracycline regimens — at 27% — with a median PFS of 4 months. Two patients treated with gemcitabine-based regimens experienced complete responses.
Among patients treated with gemcitabine, researchers observed a trend toward a higher response rate among those with classic type compared with proximal morphological type disease (30% vs. 22%), as well as for distal compared with proximal primary sites (40% vs. 14%).
No objective responses occurred among patients treated with pazopanib. These patients had a median PFS of 3 months.
Frezza and colleagues acknowledged that the retrospective nature and small sample size limited the study.
“Although the number of patients was low, we observed signs of a differential activity of anthracycline-based and gemcitabine-based regimens between the two endothelial sarcoma variants,” the researchers wrote. “A further subtype-adapted grading system based on pathologic features and its correlation with treatment response would be interesting to explore. We also hope that this report will provide a benchmark for future trials on medical agents in this disease.” – by Andy Polhamus
Disclosures: Frezza reports research funding to her institution from Eli Lilly, Epizyme, GlaxoSmithKline, Novartis and Pfizer. Please see the study for all other authors’ relevant financial disclosures.