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

advanced disease low-grade fibromyxoid sarcoma sclerosing epithelioid fibrosarcoma systemic therapies ultra-rare sarcomas

Journal

ESMO open
ISSN: 2059-7029
Titre abrégé: ESMO Open
Pays: England
ID NLM: 101690685

Informations de publication

Date de publication:
11 Sep 2024
Historique:
received: 23 05 2024
revised: 28 07 2024
accepted: 30 07 2024
medline: 13 9 2024
pubmed: 13 9 2024
entrez: 12 9 2024
Statut: aheadofprint

Résumé

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. 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. 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. 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.

Sections du résumé

BACKGROUND 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 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 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 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.

Identifiants

DOI: 10.1016/j.esmoop.2024.103689 PMID: 39265219
pubmed: 39265219
pii: S2059-7029(24)01458-3
doi: 10.1016/j.esmoop.2024.103689
pii:
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

103689

Informations de copyright

Copyright © 2024 The Author(s). Published by Elsevier Ltd.. All rights reserved.

Auteurs

C Giani (C)

Department of Medical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.

R A Denu (RA)

Division of Cancer Medicine, The University of Texas MD Anderson Cancer Center, Houston, USA.

S Ljevar (S)

Department of Clinical Epidemiology and Trial Organisation, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.

A Gronchi (A)

Department of Sarcoma Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.

A Napolitano (A)

Department of Medical Oncology, The Royal Marsden NHS, London, UK.

E Rosenbaum (E)

Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, USA.

A Salawu (A)

Division of Medical Oncology and Hematology, Mount Sinai Hospital and Princess Margaret Cancer Centre, Toronto, Canada.

J Bajpai (J)

Department of Medical Oncology, Tata Memorial Centre, Homibhabha National University, Mumbai, India.

E A Connolly (EA)

Central Clinical School, Faculty of Medicine and Health Science, The University of Sydney, Sydney, Australia.

A T J Lee (ATJ)

Department of Medical Oncology, The Christie NHS Foundation Trust, Manchester, UK.

J C Trent (JC)

Department of Medical Oncology, Sylvester Comprehensive Cancer Center, University of Miami, Miami, USA.

H Koseła-Paterczyk (H)

Department of Medical Oncology, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, Poland.

Z Chia-Chen Li (Z)

Department of Medical Oncology, National Taiwan University Cancer Center, Taipei, Taiwan.

K Ogura (K)

Department of Musculoskeletal Oncology, National Cancer Center Hospital, Tokyo, Japan.

E Palmerini (E)

Department of Medical Oncology, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy.

G G Baldi (GG)

Department of Medical Oncology, Hospital of Prato, Azienda USL Toscana Centro, Prato, Italy.

A Brunello (A)

Department of Oncology, Medical Oncology 1 Unit, Istituto Oncologico Veneto IOV - IRCCS, Padua, Italy.

F Campos (F)

Department of Medical Oncology, A.C.Camargo Cancer Center, São Paulo, Brazil.

C M Cicala (CM)

Department of Medical Oncology, Vall d'Hebron University Hospital, Vall d'Hebron Institute of Oncology (VHIO), Barcelona, Spain.

R G Maki (RG)

Department of Medical Oncology, University of Pennsylvania Perelman School of Medicine, Philadelphia, USA.

A J Wagner (AJ)

Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, USA.

V Andelkovic (V)

Department of Medical Oncology, Princess Alexandra Hospital (PAH), Woolloongabba, Australia.

H H Loong (HH)

Department of Clinical Oncology, The Chinese University of Hong Kong, Prince of Wales Hospital, Hong Kong SAR, China.

D D Wong (DD)

Department of Anatomical Pathology, PathWest, Sir Charles Gairdner Hospital, Perth, Australia.

R L Jones (RL)

Department of Medical Oncology, The Royal Marsden NHS, London, UK.

W D Tap (WD)

Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, USA.

S M Taverna (SM)

Technology Transfer Office, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.

A J Lazar (AJ)

Department of Pathology and Genomic Medicine, The University of Texas MD Anderson Cancer Center, Houston, USA.

E G Demicco (EG)

Department of Pathology and Laboratory Medicine, Mount Sinai Hospital and Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Canada.

A Hong (A)

Central Clinical School, Faculty of Medicine and Health Science, The University of Sydney, Sydney, Australia.

J V M G Bovee (JVMG)

Department of Pathology, Leiden University Medical Center (LUMC), Leiden, The Netherlands.

A P Dei Tos (AP)

Department of Pathology, Azienda Ospedale-Università Padova, Padova, Italy.

C D M Fletcher (CDM)

Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, USA.

D Baumhoer (D)

Department of Pathology, University Hospital Basel, Basel, Switzerland.

M Sbaraglia (M)

Department of Pathology, Azienda Ospedale-Università Padova, Padova, Italy.

I M Schaefer (IM)

Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, USA.

R Miceli (R)

Department of Clinical Epidemiology and Trial Organisation, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.

S Stacchiotti (S)

Department of Medical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy. Electronic address: silvia.stacchiotti@istitutotumori.mi.it.

Classifications MeSH