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Anthracycline, Gemcitabine, and Pazopanib in Epithelioid Sarcoma: A Multi-institutional Case Series.

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Publication Date
2018-09-13
ICR Author
Jones, Robin
Author
Frezza, AM
Jones, RL
Lo Vullo, S
Asano, N
Lucibello, F
Ben-Ami, E
Ratan, R
Teterycz, P
Boye, K
Brahmi, M
Palmerini, E
Fedenko, A
Vincenzi, B
Brunello, A
Desar, IME
Benjamin, RS
Blay, JY
Broto, JM
Casali, PG
Gelderblom, H
Grignani, G
Gronchi, A
Hall, KS
Mir, O
Rutkowski, P
Wagner, AJ
Anurova, O
Collini, P
Dei Tos, AP
Flucke, U
Hornick, JL
Lobmaier, I
Philippe, T
Picci, P
Ranchere, D
Renne, SL
Sbaraglia, M
Thway, K
Wagrodzki, M
Wang, W-L
Yoshida, A
Mariani, L
Kawai, A
Stacchiotti, S
Type
Journal Article
Metadata
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Abstract
Importance: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. Objective:To report on the activity of anthracycline-based regimens, gemcitabine-based regimens, and pazopanib in patients with advanced ES. Design, Setting, and Participants: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. Exposures:All patients included in the study received anthracycline-based regimens, gemcitabine-based regimens, or pazopanib. Main Outcome and Measures: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). Results: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. Conclusions and Relevance: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.
URL
https://repository.icr.ac.uk/handle/internal/2959
Collections
  • Clinical Studies
Licenseref URL
http://www.rioxx.net/licenses/under-embargo-all-rights-reserved
Version of record
10.1001/jamaoncol.2018.0219
Subject
Humans
Sarcoma
Sulfonamides
Pyrimidines
Anthracyclines
Deoxycytidine
Antineoplastic Combined Chemotherapy Protocols
Remission Induction
Retrospective Studies
Adolescent
Adult
Aged
Middle Aged
Female
Male
Young Adult
Kaplan-Meier Estimate
Response Evaluation Criteria in Solid Tumors
Research team
Sarcoma Clinical Trials (R Jones)
Language
eng
License start date
2018-09-13
Citation
JAMA oncology, 2018, 4 (9), pp. e180219 - ?

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