Low-grade fibromyxoid sarcoma and sclerosing epithelioid fibrosarcoma, outcome of advanced disease: retrospective study from the Ultra-Rare Sarcoma Working Group.
Loading...
Embargo End Date
ICR Authors
Authors
Giani, C
Denu, RA
Ljevar, S
Gronchi, A
Napolitano, A
Rosenbaum, E
Salawu, A
Bajpai, J
Connolly, EA
Lee, ATJ
Trent, JC
Koseła-Paterczyk, H
Chia-Chen Li, Z
Ogura, K
Palmerini, E
Baldi, GG
Brunello, A
Campos, F
Cicala, CM
Maki, RG
Wagner, AJ
Andelkovic, V
Loong, HH
Wong, DD
Jones, RL
Tap, WD
Taverna, SM
Lazar, AJ
Demicco, EG
Hong, A
Bovee, JVMG
Dei Tos, AP
Fletcher, CDM
Baumhoer, D
Sbaraglia, M
Schaefer, IM
Miceli, R
Stacchiotti, S
Denu, RA
Ljevar, S
Gronchi, A
Napolitano, A
Rosenbaum, E
Salawu, A
Bajpai, J
Connolly, EA
Lee, ATJ
Trent, JC
Koseła-Paterczyk, H
Chia-Chen Li, Z
Ogura, K
Palmerini, E
Baldi, GG
Brunello, A
Campos, F
Cicala, CM
Maki, RG
Wagner, AJ
Andelkovic, V
Loong, HH
Wong, DD
Jones, RL
Tap, WD
Taverna, SM
Lazar, AJ
Demicco, EG
Hong, A
Bovee, JVMG
Dei Tos, AP
Fletcher, CDM
Baumhoer, D
Sbaraglia, M
Schaefer, IM
Miceli, R
Stacchiotti, S
Document Type
Journal Article
Date
2024-09-01
Date Accepted
2024-07-30
Abstract
BACKGROUND: To present findings from a retrospective study conducted by the Ultra-Rare Sarcoma Working Group on metastatic low-grade fibromyxoid sarcoma (LGFMS), sclerosing epithelioid fibrosarcoma (SEF), and hybrid (H)-LGFMS/SEF across 28 global centres. METHODS: Patients treated at participating institutions from January 2000 to September 2022 were retrospectively selected. Diagnosis was confirmed by expert pathologists. Primary endpoint was progression-free survival (PFS-1) from metastasis detection to first progression or death. PFS-2 was calculated from therapy initiation. RESULTS: A total of 101 patients were identified (32 LGFMS, 50 SEF, 19 H-LGFMS/SEF). Median (m) follow-up was 62.1 months. mPFS-1 was 28.7, 11.8, and 20.3 months for LGFMS, SEF, and H-LGFMS/SEF, respectively. mOS was 145.8, 41.9, and 113.5 months, respectively. Treatments included anthracycline-based chemotherapy, gemcitabine-based chemotherapy (G), pazopanib, trabectedin, others. mPFS-2 was: 20.1, 5.5, and 3.5 months in H-LGFMS/SEF, SEF, and LGFMS, respectively, with anthracyclines; 19.5, 7.7, and 6.9 months in LGFMS, SEF, and H-LGFMS/SEF, respectively, with pazopanib; 12.0, 9.7, and 3.1 months in H-LGFMS/SEF, LGFMS, and SEF, respectively. Occasional responses occurred with ifosfamide/oral cyclophosphamide, and prolonged stable disease with immune checkpoint inhibitors. CONCLUSIONS: In this series, the largest available, metastatic LGFMS, SEF, and H-LGFMS/SEF showed different courses. Systemic agents have modest efficacy, informing future trials of novel agents for these tumours.
Citation
ESMO Open, 2024, 9 (9), pp. 103689 -
Source Title
ESMO Open
Publisher
ELSEVIER
ISSN
2059-7029
eISSN
2059-7029