International Multicenter Retrospective Study From the Ultra-rare Sarcoma Working Group on Low-grade Fibromyxoid Sarcoma, Sclerosing Epithelioid Fibrosarcoma, and Hybrid Forms

The aim of the study was to report the outcome of primary localized low-grade fibromyxoid sarcoma (LGFMS), sclerosing epithelioid fibrosarcoma (SEF), and hybrid LGFMS/SEF (H-LGFMS/SEF). Patients with primary localized LGFMS, SEF, or H-LGFMS/SEF, surgically treated with curative intent from January 2000 to September 2022, were enrolled from 14 countries and 27 institutions. Pathologic inclusion criteria were predefined by expert pathologists. The primary endpoint was overall survival (OS). Secondary endpoints were crude cumulative incidence (CCI) of local recurrence (LR), CCI of distant metastases (DM), and post-metastases OS (p-OS). Two hundred ninety-four patients (239 LGFMS, 32 SEF, and 23 H-LGFMS/SEF) were identified. At a median(m-) follow-up (FU) of 57.1 months, 12/294 patients died. The 5- and 10-year OS were 99.0% and 95.9% in LGFMS, 86.2% and 67.0% in SEF, and 84.8% and 84.8% in H-LGFMS/SEF, respectively. Predictors of worse OS included pathology, age at surgery, systemic therapy, and radiotherapy. LR developed in 13/294 (4.4%) patients. The observed m-time to LR was 10.7 months. The 5- and 10-yr CCI-LR were 4.7% in LGFMS and 6.6% in SEF, respectively. There were no LR events in H-LGFMS/SEF. The sole predictor of higher risk of LR was histology. DM developed in 23/294 (7.8%) patients. The observed m-time to DM was 28.2 months. The 5- and 10-yr CCI-DM were 1.3% and 2.7% in LGMFS, 29.9% and 57.7% in SEF, 48.9% and 48.9% in H-LGFMS/SEF, respectively. Predictors of higher risk of DM were histology, systemic therapy, and radiotherapy. Primary localized LGFMS treated with complete surgical resection has an excellent prognosis, while about 50% of H-LGFMS/SEF and SEF develop DM within 5 to 10 years. Very long-term FU is needed to understand absolute cure rates.

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Journal
The American Journal of Surgical Pathology
Published
2024-10-28
DOI
https://doi.org/10.1097/pas.0000000000002330
Citations
4
Primary Topic
Sarcoma Diagnosis and Treatment
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article
Field-Weighted Citation Impact
1.68
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article

International Multicenter Retrospective Study From the Ultra-rare Sarcoma Working Group on Low-grade Fibromyxoid Sarcoma, Sclerosing Epithelioid Fibrosarcoma, and Hybrid Forms

Andrea Napolitano, Andrew J. Wagner, Shahd S. Almohsen, Alexander T.J. Lee et al.
4 citations
The American Journal of Surgical Pathology
Sarcoma Diagnosis and Treatment
1.68
article

International Multicenter Retrospective Study From the Ultra-rare Sarcoma Working Group on Low-grade Fibromyxoid Sarcoma, Sclerosing Epithelioid Fibrosarcoma, and Hybrid Forms

Andrea Napolitano, Andrew J. Wagner, Shahd S. Almohsen, Alexander T.J. Lee, Nadia Hindi, Daniel Wong, Carlo María Cicala, Sosipatros A. Boikos, Daniel Baumhoer, Silva Ljevar, Angelo Paolo Dei Tos, Hanna Koseła-Paterczyk, Herbert H. Loong, Akira Kawai, Abdulazeez Salawu, Elizabeth A. Connolly, Richard Carey‐Smith, Elizabeth G. Demicco, Rosalba Miceli, Ryan A. Denu, Roberto Scanferla, Evan Rosenbaum, Robin L. Jones, Toni Ibrahim, Alexander J. Lazar, Christopher D.�M. Fletcher, Lorenzo D’Ambrosio, Steve Bialick, Silvia Stacchiotti, Stefano Taverna, Koichi Ogura, Claudia Giani, Giacomo Giulio Baldi, Alessandro Gronchi, Robert G. Maki, Marta Sbaraglia, Yeh Chen Lee, Susie Bae, Emanuela Palmerini, Antonella Brunello, Judith V.M.G. Bovée, Inga‐Marie Schaefer, Jyoti Bajpai, Costanza Figura, Zhi Li, Richard Boyle, Fernando Augusto Batista Campos, S. Patel, Rooshdiya Z. Karim
article en
4 citations

Abstract

The aim of the study was to report the outcome of primary localized low-grade fibromyxoid sarcoma (LGFMS), sclerosing epithelioid fibrosarcoma (SEF), and hybrid LGFMS/SEF (H-LGFMS/SEF). Patients with primary localized LGFMS, SEF, or H-LGFMS/SEF, surgically treated with curative intent from January 2000 to September 2022, were enrolled from 14 countries and 27 institutions. Pathologic inclusion criteria were predefined by expert pathologists. The primary endpoint was overall survival (OS). Secondary endpoints were crude cumulative incidence (CCI) of local recurrence (LR), CCI of distant metastases (DM), and post-metastases OS (p-OS). Two hundred ninety-four patients (239 LGFMS, 32 SEF, and 23 H-LGFMS/SEF) were identified. At a median(m-) follow-up (FU) of 57.1 months, 12/294 patients died. The 5- and 10-year OS were 99.0% and 95.9% in LGFMS, 86.2% and 67.0% in SEF, and 84.8% and 84.8% in H-LGFMS/SEF, respectively. Predictors of worse OS included pathology, age at surgery, systemic therapy, and radiotherapy. LR developed in 13/294 (4.4%) patients. The observed m-time to LR was 10.7 months. The 5- and 10-yr CCI-LR were 4.7% in LGFMS and 6.6% in SEF, respectively. There were no LR events in H-LGFMS/SEF. The sole predictor of higher risk of LR was histology. DM developed in 23/294 (7.8%) patients. The observed m-time to DM was 28.2 months. The 5- and 10-yr CCI-DM were 1.3% and 2.7% in LGMFS, 29.9% and 57.7% in SEF, 48.9% and 48.9% in H-LGFMS/SEF, respectively. Predictors of higher risk of DM were histology, systemic therapy, and radiotherapy. Primary localized LGFMS treated with complete surgical resection has an excellent prognosis, while about 50% of H-LGFMS/SEF and SEF develop DM within 5 to 10 years. Very long-term FU is needed to understand absolute cure rates.

The American Journal of Surgical PathologyVol. 49(1)
Hebron University (PS), Brigham and Women's Hospital (US), MedStar Washington Hospital Center (US), Mount Sinai Hospital (CA), The University of Sydney (AU), Memorial Sloan Kettering Cancer Center (US), The University of Texas MD Anderson Cancer Center (US), Harvard University (US), University of Miami (US), National Taiwan University (TW), Chinese University of Hong Kong (HK), University of Toronto (CA), Homi Bhabha National Institute (IN), Royal Prince Alfred Hospital (AU), Leiden University Medical Center (NL), Tata Memorial Hospital (IN), Peter MacCallum Cancer Centre (AU), Sir Charles Gairdner Hospital (AU), University Hospital of Basel (CH), Princess Margaret Cancer Centre (CA), AC Camargo Hospital (BR), UNSW Sydney (AU), Institute of Cancer Research (CA), Chris O’Brien Lifehouse (AU), Prince of Wales Hospital (AU), Istituto Oncologico Veneto (IT), Prince of Wales Hospital (CN), Azienda Ospedaliero-Universitaria Careggi (IT), Dana-Farber Cancer Institute (US), Istituto Ortopedico Rizzoli (IT), Hospital Universitario Fundación Jiménez Díaz (ES), The Christie NHS Foundation Trust (GB), The Maria Sklodowska-Curie National Research Institute of Oncology (PL), Istituti di Ricovero e Cura a Carattere Scientifico (IT), Fondazione IRCCS Istituto Nazionale dei Tumori (IT), Sylvester Comprehensive Cancer Center (US), Hospital of Prato (IT), Vall d'Hebron Institute of Oncology (ES), University of Turin (IT), University of Pennsylvania (US), The University of Notre Dame Australia (AU)
Reduced inequalities
Openalex Percentile: Top 15%
Sarcoma Diagnosis and Treatment
1.68
DOIOpen Access PDFOpenAlex
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