Alveolar Soft Part Sarcoma: Clinicopathologic Analysis, Predictive Nomogram, and the Role of Adjuvant Radiation for Survival in a Retrospective Population-based Study.

Abstract

Objectives: Alveolar soft part sarcoma (ASPS) is a rare sarcoma affecting the deep soft tissues of the extremities in young adults and the head/trunk in children. This population-based study investigates demographics and factors influencing survival outcomes in ASPS.

Methods: The data for this study were collected from the SEER databases from 2000 to 2020.

Results: A total of 277 cases with ASPS were extracted from the database with a median age of 25 years and slight female predilection (52.3%). ASPS has a higher incidence in black (24.2%) and Hispanic (23.1%) races. Tumor location varied by age group: the head and neck were most commonly affected in younger patients (36.2%), while the lower extremities and hip region were predominant in older patients (68.8%). In most cases, the tumor size was >4 cm. When known, and majority were distant metastases (50.7%), and lung being the most common metastatic. The 5-year CSS with distant metastases was 33.0% (95% CI: 24.7-44.0). The highest 5-year CSS was observed with surgery with adjuvant radiation 86.7% (95% CI: 78.0-96.4). Multivariate analysis revealed that male sex was associated with worse survival (hazard ratio [HR] = 2.22), while surgery with adjuvant radiation was significantly associated with improved survival (HR = 0.36).

Conclusions: This study found that the male sex and larger tumors predict poorer ASPS outcomes, while surgery with adjuvant radiation improves survival. Future prospective clinical trials should focus on genomic mutation analysis for a personalized therapeutic approach.