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International Multicenter Retrospective Study From the Ultra-rare Sarcoma Working Group on Low-grade Fibromyxoid Sarcoma, Sclerosing Epithelioid Fibrosarcoma, and Hybrid Forms: Outcome of Primary Localized Disease

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Title: International Multicenter Retrospective Study From the Ultra-rare Sarcoma Working Group on Low-grade Fibromyxoid Sarcoma, Sclerosing Epithelioid Fibrosarcoma, and Hybrid Forms: Outcome of Primary Localized Disease
Authors: Giani, Claudia, Salawu, Abdulazeez, Ljevar, Silva, Denu, Ryan A, Napolitano, Andrea, Palmerini, Emanuela, Connolly, Elizabeth A, Ogura, Koichi, Wong, Daniel D, Scanferla, Roberto, Rosenbaum, Evan, Bajpai, Jyoti, Li, Zola Chia-Chen, Bae, Susie, D'Ambrosio, Lorenzo, Bialick, Steve, Wagner, Andrew J, Lee, Alexander T J, Koseła-Paterczyk, Hanna, Baldi, Giacomo G, Brunello, Antonella, Lee, Yeh Chen, Loong, Herbert H, Boikos, Sosipatros, Campos, Fernando, Cicala, Carlo M, Maki, Robert G, Hindi, Nadia, Figura, Costanza, Almohsen, Shahd S, Patel, Sheyaskumar, Jones, Robin L, Ibrahim, Toni, Karim, Rooshdiya, Kawai, Akira, Carey-Smith, Richard, Boyle, Richard, Taverna, Silvia M, Lazar, Alexander J, Demicco, Elizabeth G, Bovee, Judith V M G, Dei Tos, Angelo P, Fletcher, Christopher, Baumhoer, Daniel, Sbaraglia, Marta, Schaefer, Inga-Marie, Miceli, Rosalba, Gronchi, Alessandro, Stacchiotti, Silvia
Contributors: Institut Català de la Salut, [Giani C] Department of Medical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy. [Salawu A] Division of Medical Oncology and Hematology, Mount Sinai Hospital and Princess Margaret Cancer Centre, Toronto, Canada. [Ljevar S] Unit of Biostatistics for Clinical Research, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy. [Denu RA] Division of Cancer Medicine, The University of Texas, MD Anderson Cancer Center, Houston, TX. [Napolitano A] Department of Medical Oncology, The Royal Marsden NHS and Institute of Cancer Research, London. [Palmerini E] Osteoncology, Bone and Soft Tissue Sarcomas, and Innovative Therapies Unit, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy. [Cicala CM] Servei d’Oncologia Mèdica, Vall d’Hebron Hospital Universitari, Barcelona, Spain. Vall d’Hebron Institute of Oncology (VHIO), Barcelona, Spain, Vall d'Hebron Barcelona Hospital Campus
Source: Am J Surg Pathol
Scientia
Scientia. Dipòsit d'Informació Digital del Departament de Salut
instname
Publisher Information: Ovid Technologies (Wolters Kluwer Health), 2024.
Publication Year: 2024
Subject Terms: Male, Adult, Time Factors, Adolescent, Fibrosarcoma, Tumors de parts toves - Cirurgia, Soft Tissue Neoplasms, Young Adult, Risk Factors, DISEASES::Neoplasms::Neoplasms by Site::Soft Tissue Neoplasms, Humans, Other subheadings::Other subheadings::Other subheadings::/surgery, Child, Retrospective Studies, Aged, Aged, 80 and over, Otros calificadores::Otros calificadores::Otros calificadores::/cirugía, ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT::Diagnosis::Prognosis::Treatment Outcome, Sarcoma, DISEASES::Neoplasms::Neoplasms by Histologic Type::Neoplasms, Connective and Soft Tissue::Neoplasms, Connective Tissue::Neoplasms, Fibrous Tissue::Fibrosarcoma, Original Articles, Middle Aged, TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS::diagnóstico::pronóstico::resultado del tratamiento, Treatment Outcome, Child, Preschool, Avaluació de resultats (Assistència sanitària), Female, ENFERMEDADES::neoplasias::neoplasias por localización::neoplasias de los tejidos blandos, Neoplasm Grading, Neoplasm Recurrence, Local, low-grade fibromyxoid sarcoma, sclerosing epithelioid fibrosarcoma, hybrid forms, localized disease, prognostic factors, survival, ENFERMEDADES::neoplasias::neoplasias por tipo histológico::neoplasias de tejido conjuntivo y de tejidos blandos::neoplasias de tejido conjuntivo::neoplasias de tejido fibroso::fibrosarcoma
Description: 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.
Document Type: Article
Other literature type
File Description: application/pdf
Language: English
ISSN: 1532-0979
0147-5185
DOI: 10.1097/pas.0000000000002330
Access URL: https://pubmed.ncbi.nlm.nih.gov/39466087
https://hdl.handle.net/11351/12680
https://hdl.handle.net/11577/3538162
https://doi.org/10.1097/PAS.0000000000002330
Rights: CC BY NC ND
URL: http://creativecommons.org/licenses/by-nc-nd/4.0/ http://creativecommons.org/licenses/by-nc-nd/4.0/
URL: http://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (http://creativecommons.org/licenses/by-nc-nd/4.0/) (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc-nd/4.0/ (http://creativecommons.org/licenses/by-nc-nd/4.0/)
Accession Number: edsair.doi.dedup.....92f306169a077aef78ee43cbdfd73044
Database: OpenAIRE
Description
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.
ISSN:15320979
01475185
DOI:10.1097/pas.0000000000002330