RT Journal Article T1 Anthracycline, Gemcitabine, and Pazopanib in Epithelioid Sarcoma: A Multi-institutional Case Series. A1 Frezza, Anna Maria A1 Jones, Robin L A1 Lo-Vullo, Salvatore A1 Asano, Naofumi A1 Lucibello, Francesca A1 Ben-Ami, Eytan A1 Ratan, Ravin A1 Teterycz, Pawel A1 Boye, Kjetil A1 Brahmi, Mehdi A1 Palmerini, Emanuela A1 Fedenko, Alexander A1 Vincenzi, Bruno A1 Brunello, Antonella A1 Desar, Ingrid M E A1 Benjamin, Robert S A1 Blay, Jean Yves A1 Broto, Javier Martin A1 Casali, Paolo G A1 Gelderblom, Hans A1 Grignani, Giovanni A1 Gronchi, Alessandro A1 Hall, Kirsten Sundby A1 Mir, Olivier A1 Rutkowski, Piotr A1 Wagner, Andrew J A1 Anurova, Olga A1 Collini, Paola A1 Dei-Tos, Angelo P A1 Flucke, Uta A1 Hornick, Jason L A1 Lobmaier, Ingvild A1 Philippe, Terrier A1 Picci, Piero A1 Ranchere, Dominique A1 Renne, Salvatore L A1 Sbaraglia, Marta A1 Thway, Khin A1 Wagrodzki, Michal A1 Wang, Wei-Lien A1 Yoshida, Akihiko A1 Mariani, Luigi A1 Kawai, Akira A1 Stacchiotti, Silvia K1 Anthracyclines K1 Antineoplastic Combined Chemotherapy Protocols K1 Deoxycytidine K1 Pyrimidines K1 Remission Induction K1 Gemcitabine AB Epithelioid sarcoma (ES) is an exceedingly rare malignant neoplasm with distinctive pathologic, molecular, and clinical features as well as the potential to respond to new targeted drugs. Little is known on the activity of anthracycline-based regimens, gemcitabine-based regimens, and pazopanib in this disease. To report on the activity of anthracycline-based regimens, gemcitabine-based regimens, and pazopanib in patients with advanced ES. Seventeen sarcoma reference centers in Europe, the United States, and Japan contributed data to this retrospective analysis of patients with locally advanced/metastatic ES diagnosed between 1990 and 2016. Local pathological review was performed in all cases to confirm diagnosis according to most recent criteria. All patients included in the study received anthracycline-based regimens, gemcitabine-based regimens, or pazopanib. Response was assessed by RECIST. Progression-free survival (PFS) and overall survival (OS) were computed by Kaplan-Meier method. Classic and proximal subtypes were defined based on morphology (according to 2013 World Health Organization guidelines). Overall, 115 patients were included, 80 (70%) were men and 35 (30%) were women, with a median age of 32 years (range, 15-77 years). Of the 115 patients with ES, 85 were treated with anthracycline-based regimens, 41 with gemcitabine-based regimens, and 18 with pazopanib. Twenty-four received more than 1 treatment. Median follow-up was 34 months. Response rate for anthracycline-based regimens was 22%, with a median PFS of 6 months. One complete response (CR) was reported. A trend toward a higher response rate was noticed in morphological proximal type (26%) vs classic type (19%) and in proximal vs distal primary site (26% vs 18%). The response rate for gemcitabine-based regimens was 27%, with 2 CR and a median PFS of 4 months. In this group, a trend toward a higher response rate was reported in classic vs proximal morphological type (30% vs 22%) and in distal vs proximal primary site (40% vs 14%). In the pazopanib group, no objective responses were seen, and median PFS was 3 months. This is the largest retrospective series of systemic therapy in ES. We confirm a moderate activity of anthracycline-based and gemcitabine-based regimens in ES, with a similar response rate and PFS in both groups. The value of pazopanib was low. These data may serve as a benchmark for trials of novel agents in ES. PB American Medical Association YR 2018 FD 2018-04-12 LK http://hdl.handle.net/10668/12507 UL http://hdl.handle.net/10668/12507 LA en NO Frezza AM, Jones RL, Lo Vullo S, Asano N, Lucibello F, Ben-Ami E, et al. Anthracycline, Gemcitabine, and Pazopanib in Epithelioid Sarcoma: A Multi-institutional Case Series. JAMA Oncol. 2018 Sep 1;4(9):e180219. DS RISalud RD Apr 11, 2025