TY - JOUR
T1 - International Multicenter Retrospective Study From the Ultra-rare Sarcoma Working Group on Low-grade Fibromyxoid Sarcoma, Sclerosing Epithelioid Fibrosarcoma, and Hybrid Forms
T2 - Outcome of Primary Localized Disease
AU - Giani, Claudia
AU - Salawu, Abdulazeez
AU - Ljevar, Silva
AU - Denu, Ryan A
AU - Napolitano, Andrea
AU - Palmerini, Emanuela
AU - Connolly, Elizabeth A
AU - Ogura, Koichi
AU - Wong, Daniel D
AU - Scanferla, Roberto
AU - Rosenbaum, Evan
AU - Bajpai, Jyoti
AU - Li, Zola Chia-Chen
AU - Bae, Susie
AU - D'Ambrosio, Lorenzo
AU - Bialick, Steve
AU - Wagner, Andrew J
AU - Lee, Alexander T J
AU - Koseła-Paterczyk, Hanna
AU - Baldi, Giacomo G
AU - Brunello, Antonella
AU - Lee, Yeh Chen
AU - Loong, Herbert H
AU - Boikos, Sosipatros
AU - Campos, Fernando
AU - Cicala, Carlo M
AU - Maki, Robert G
AU - Hindi, Nadia
AU - Figura, Costanza
AU - Almohsen, Shahd S
AU - Patel, Sheyaskumar
AU - Jones, Robin L
AU - Ibrahim, Toni
AU - Karim, Rooshdiya
AU - Kawai, Akira
AU - Carey-Smith, Richard
AU - Boyle, Richard
AU - Taverna, Silvia M
AU - Lazar, Alexander J
AU - Demicco, Elizabeth G
AU - Bovee, Judith V M G
AU - Dei Tos, Angelo P
AU - Fletcher, Christopher
AU - Baumhoer, Daniel
AU - Sbaraglia, Marta
AU - Schaefer, Inga-Marie
AU - Miceli, Rosalba
AU - Gronchi, Alessandro
AU - Stacchiotti, Silvia
PY - 2024/10/28
Y1 - 2024/10/28
N2 - 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.
AB - 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.
UR - https://edmgr.ovid.com/ajsp/accounts/ifauth.htm
U2 - 10.1097/PAS.0000000000002330
DO - 10.1097/PAS.0000000000002330
M3 - Article
C2 - 39466087
SN - 0147-5185
JO - The American Journal of Surgical Pathology
JF - The American Journal of Surgical Pathology
ER -