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Multicenter Study
. 2024 Sep;9(9):103689.
doi: 10.1016/j.esmoop.2024.103689. Epub 2024 Sep 11.

Low-grade fibromyxoid sarcoma and sclerosing epithelioid fibrosarcoma, outcome of advanced disease: retrospective study from the Ultra-Rare Sarcoma Working Group

C Giani  1 , R A Denu  2 , S Ljevar  3 , A Gronchi  4 , A Napolitano  5 , E Rosenbaum  6 , A Salawu  7 , J Bajpai  8 , E A Connolly  9 , A T J Lee  10 , J C Trent  11 , H Koseła-Paterczyk  12 , Z Chia-Chen Li  13 , K Ogura  14 , E Palmerini  15 , G G Baldi  16 , A Brunello  17 , F Campos  18 , C M Cicala  19 , R G Maki  20 , A J Wagner  21 , V Andelkovic  22 , H H Loong  23 , D D Wong  24 , R L Jones  5 , W D Tap  6 , S M Taverna  25 , A J Lazar  26 , E G Demicco  27 , A Hong  9 , J V M G Bovee  28 , A P Dei Tos  29 , C D M Fletcher  30 , D Baumhoer  31 , M Sbaraglia  29 , I M Schaefer  30 , R Miceli  3 , S Stacchiotti  32
Affiliations
Multicenter Study

Low-grade fibromyxoid sarcoma and sclerosing epithelioid fibrosarcoma, outcome of advanced disease: retrospective study from the Ultra-Rare Sarcoma Working Group

C Giani et al. ESMO Open. 2024 Sep.

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.

Keywords: advanced disease; low-grade fibromyxoid sarcoma; sclerosing epithelioid fibrosarcoma; systemic therapies; ultra-rare sarcomas.

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Figures

Figure 1
Figure 1
Progression free survival (PFS) curves of advanced low-grade fibromyxoid sarcoma (LGFMS), sclerosing epithelioid fibrosarcoma (SEF), and hybrid (H)-LGFMS/SEF, treated with different systemic regimens. Panel A shows PFS curves of patients treated with anthracycline-based regimens (LGFMS = 18, SEF = 30, H-LGFMS/SEF = 9); Panel B shows PFS curves of patients treated with gemcitabine-based regimens (LGFMS = 7, SEF = 19, H-LGFMS/SEF = 4); Panel C shows PFS curves of patients treated with pazopanib (LGFMS = 11, SEF = 21, H-LGFMS/SEF = 4): Panel D shows PFS curves patients treated with trabectedin (LGFMS = 8, SEF = 8, H-LGFMS/SEF = 3). Patients could appear in more than one category if they received more than one regimen.
Figure 2
Figure 2
Overall survival (OS) curves of advanced low-grade fibromyxoid sarcoma (LGFMS), sclerosing epithelioid fibrosarcoma (SEF), and hybrid (H)-LGFMS/SEF, treated with different systemic regimens. Panel A shows OS curves of patients treated with anthracycline-based regimens (LGFMS = 18, SEF = 30, H-LGFMS/SEF = 9); Panel B shows OS curves of patients treated with gemcitabine-based regimens (LGFMS = 7, SEF = 19, H-LGFMS/SEF = 4); Panel C shows OS curves of patients treated with pazopanib (LGFMS = 11, SEF = 21, H-LGFMS/SEF = 4): Panel D shows OS curves patients treated with trabectedin (LGFMS = 8, SEF = 8, H-LGFMS/SEF = 3). Patients could appear in more than one category if they received more than one regimen.
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References

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