Low-grade fibromyxoid sarcoma and sclerosing epithelioid fibrosarcoma, outcome of advanced disease: Retrospective study from the Ultra-Rare Sarcoma Working Group Journal Article
| Authors: | Giani, C.; Denu, R. A.; Ljevar, S.; Gronchi, A.; Napolitano, A.; Rosenbaum, E.; Salawu, A.; Bajpai, J.; Connolly, E. A.; Lee, A. 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. V. M. G.; Dei Tos, A. P.; Fletcher, C. D. M.; Baumhoer, D.; Sbaraglia, M.; Schaefer, I. M.; Miceli, R.; Stacchiotti, S. |
| Article Title: | Low-grade fibromyxoid sarcoma and sclerosing epithelioid fibrosarcoma, outcome of advanced disease: Retrospective study from the Ultra-Rare Sarcoma Working Group |
| 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. © 2024 The Author(s) |
| Keywords: | immunohistochemistry; adult; cancer chemotherapy; controlled study; treatment response; cancer surgery; unclassified drug; major clinical study; overall survival; systemic therapy; bone metastasis; gemcitabine; cancer radiotherapy; follow up; lymph node metastasis; progression free survival; cyclophosphamide; retrospective study; cancer mortality; ifosfamide; sarcoma; liver metastasis; lung metastasis; gene rearrangement; fibrosarcoma; pazopanib; trabectedin; anthracycline; protein ewsr1; soft tissue metastasis; mucin 4; clinical outcome; peptides and proteins; overall response rate; advanced disease; systemic therapies; immune checkpoint inhibitor; low-grade fibromyxoid sarcoma; low grade fibromyxoid sarcoma; human; male; female; article; sclerosing epithelioid fibrosarcoma; rna binding protein fus; treatment interval; ultra-rare sarcomas |
| Journal Title: | ESMO Open |
| Volume: | 9 |
| Issue: | 9 |
| ISSN: | 2059-7029 |
| Publisher: | European Society for Medical Oncology |
| Date Published: | 2024-09-01 |
| Start Page: | 103689 |
| Language: | English |
| DOI: | 10.1016/j.esmoop.2024.103689 |
| PROVIDER: | scopus |
| PMCID: | PMC11416581 |
| PUBMED: | 39265219 |
| DOI/URL: | |
| Notes: | Article -- Source: Scopus |
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