Faculty, Staff and Student Publications

Publication Date

1-1-2025

Journal

The American Journal of Surgical Pathology

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.

Keywords

Humans, Female, Male, Fibrosarcoma, Retrospective Studies, Middle Aged, Adult, Adolescent, Young Adult, Aged, Neoplasm Grading, Neoplasm Recurrence, Local, Child, Soft Tissue Neoplasms, Treatment Outcome, Child, Preschool, Aged, 80 and over, Risk Factors, Time Factors, low-grade fibromyxoid sarcoma, sclerosing epithelioid fibrosarcoma, hybrid forms, localized disease, prognostic factors, survival

DOI

10.1097/PAS.0000000000002330

PMID

39466087

PMCID

PMC11634151

PubMedCentral® Posted Date

10-28-2024

PubMedCentral® Full Text Version

Post-print

Published Open-Access

yes

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