Epidemiological trends of synovial sarcoma by primary tumor sites in the US from 2000 to 2020

• Published in: Cancer epidemiology 2024-10, Vol.92, p.102627, Article 102627
• Main Authors: Patel, Riddhi R., Delclos, George L., DeSantis, Stacia M., Cannell, Michael B., Lupo, Philip J., Lin, Patrick P., Araujo, Dejka M.
• Format: Article
• Language: English
• Subjects: Abdomen; Age; Cancer therapies; Epidemiology; Fatalities; Incidence; Mortality; Neck; Pelvis; Prevalence; Primary tumor site; Sarcoma; Survival; Synovial sarcoma; Thorax; Trends; Tumors
• ISSN: 1877-7821; 1877-783X; 1877-783X
• DOI: 10.1016/j.canep.2024.102627

Synovial sarcoma (SS) is a rare soft-tissue cancer. Existing literature encompasses Surveillance, Epidemiology, and End Results (SEER) data-based research on SS explaining the incidence-prevalence in general, by subtypes, and by age at diagnosis. Therefore, this study aimed to fill in the gap of knowledge about measures of disease occurrence and burden of SS by tumor site using the SEER database. In this cross-sectional study, primary SS patients were selected from SEER 17 Registries, Nov. 2021 (2000–2020) using ICD-O-3 codes 9040, 9041, 9042, and 9043. Patients with additional cancers were excluded. The primary tumor site was categorized into (1) head/neck, (2) internal thorax, (3) abdomen/pelvis, (4) upper extremity, and (5) lower extremity using ICD-10CM codes. Five outcomes were analyzed: age-adjusted incidence rate, 5-year limited-duration prevalence rate, incidence-based mortality, case-fatality rate, and overall survival. From 2000–2020, the overall age-adjusted incidence rate was 0.15 per 100,000; the 5-year limited duration prevalence rate was 0.56 per 100,000; and the incidence-based mortality rate was 0.06 per 100,000 people. The case-fatality and 5-year OS rates were 39.2 % and 62.9 %, respectively. Lower extremity had the highest incidence of 0.07 (estimated 1166 cases), prevalence of 0.36 (estimated 224 cases), and mortality rate of 0.025 (estimated 429 deaths) per 100,000. The other four locations had much closer rates with each other. Intrathoracic SS had the highest case-fatality rate of 71.5 % (148/207) and lowest 5-year OS of 26.0 % (95 % CI: 19.6 %, 32.9 %) than other sites. Based on the measures of disease frequency, the most common primary tumor site is the lower extremity, followed by the upper extremity, abdomen/pelvis, internal thorax, and head/neck. The least favorable primary location is the internal thorax. Those with a primary location of the upper extremity have the longest overall survival, followed by the head/neck, lower extremity, abdomen/pelvis, and internal thorax. •The incidence rate of synovial sarcoma has been stable over the past two decades.•The incidence and mortality rates were higher in the lower extremity patients.•The case-fatality rate was 39.2 % and 5-year overall survival rate was 62.9 %.•Patients with upper extremity tumors had the longest overall survival.•Intra-thoracic patients had the highest case fatality and shortest overall survival.