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Low-grade fibromyxoid sarcoma and sclerosing epithelioid fibrosarcoma, outcome of advanced disease: retrospective study from the ultra-rare sarcoma working group
Giani, C. Denu, R. A. Ljevar, S. Gronchi, A. Napolitano, A. Rosenbaum, E. Salawu, A. Bajpai, J. Connolly, E. A. Lee, Alexander T J
Giani, C.
Denu, R. A.
Ljevar, S.
Gronchi, A.
Napolitano, A.
Rosenbaum, E.
Salawu, A.
Bajpai, J.
Connolly, E. A.
Lee, Alexander T J
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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.
Authors
Giani, C.
Denu, R. A.
Ljevar, S.
Gronchi, A.
Napolitano, A.
Rosenbaum, E.
Salawu, A.
Bajpai, J.
Connolly, E. A.
Lee, Alexander T J
Trent, J. C.
Koseła-Paterczyk, H.
Chia-Chen Li, Z.
Ogura, K.
Palmerini, E.
Baldi, G. G.
Brunello, A.
Campos, F.
Cicala, C. M.
Maki, R. G.
Wagner, A. J.
Andelkovic, V.
Loong, H. H.
Wong, D. D.
Jones, R. L.
Tap, W. D.
Taverna, S. M.
Lazar, A. J.
Demicco, E. G.
Hong, A.
Bovee, J.
Dei Tos, A. P.
Fletcher, C. D. M.
Baumhoer, D.
Sbaraglia, M.
Schaefer, I. M.
Miceli, R.
Stacchiotti, S.
Denu, R. A.
Ljevar, S.
Gronchi, A.
Napolitano, A.
Rosenbaum, E.
Salawu, A.
Bajpai, J.
Connolly, E. A.
Lee, Alexander T J
Trent, J. C.
Koseła-Paterczyk, H.
Chia-Chen Li, Z.
Ogura, K.
Palmerini, E.
Baldi, G. G.
Brunello, A.
Campos, F.
Cicala, C. M.
Maki, R. G.
Wagner, A. J.
Andelkovic, V.
Loong, H. H.
Wong, D. D.
Jones, R. L.
Tap, W. D.
Taverna, S. M.
Lazar, A. J.
Demicco, E. G.
Hong, A.
Bovee, J.
Dei Tos, A. P.
Fletcher, C. D. M.
Baumhoer, D.
Sbaraglia, M.
Schaefer, I. M.
Miceli, R.
Stacchiotti, S.
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Date
2024
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Giani C, Denu RA, Ljevar S, Gronchi A, Napolitano A, Rosenbaum E, et al. Low-grade fibromyxoid sarcoma and sclerosing epithelioid fibrosarcoma, outcome of advanced disease: retrospective study from the Ultra-Rare Sarcoma Working Group. ESMO open. 2024 Sep;9(9):103689. PubMed PMID: 39265219. Pubmed Central PMCID: PMC11416581. Epub 2024/09/13. eng.