Prognostic factors of cutaneous soft tissue sarcomas in children: a SEER population-based study

Abstract

This study aims to analyze the clinicopathological characteristics and survival outcomes of cutaneous soft tissue sarcomas (CSTS) in children. We selected pediatric cases of CSTS diagnosed between 2000 and 2019 from the Surveillance, Epidemiology, and End Results (SEER) database. Survival rates were calculated using Kaplan-Meier methods. We performed univariate analyses with the log-rank test and multivariate survival analyses using Cox proportional-hazards models to determine factors affecting overall survival (OS). Additionally, we constructed a predictive nomogram based on the outcomes of the Cox regression. A total of 148 pediatric patients with CSTS were reviewed. The median age at diagnosis was 13 years (range: 0–18 years). Prognostically, tumors located on the extremities showed better outcomes compared to those on the head, neck, or trunk. Among the histological types, angiosarcoma had the lowest five-year survival rate at 51.3%, which was substantially lower compared to fibrous histiocytoma and leiomyosarcoma. Cox regression analysis highlighted surgical intervention as the only significant independent prognostic factor for OS, with an increased risk of mortality observed in patients not undergoing surgery. Additionally, patients with distant-stage disease exhibited significantly lower survival rates than those with localized conditions. Pediatric CSTS represents a diverse and infrequent group of tumors, predominantly fibrous histiocytoma and leiomyosarcoma. Surgery was identified as the crucial determinant of survival, underscoring its role in effectively managing these patients.