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. 2025 Jun 9;17(6):e85606.
doi: 10.7759/cureus.85606. eCollection 2025 Jun.

Clear Cell Sarcoma Incidence and Survival: A Surveillance, Epidemiology, and End Results (SEER) Database Analysis

Harvey Y Wang  1 , Laura M Cogua  1 , Connor J Tupper  1   2 , Peter T Silberstein  3
Affiliations

Clear Cell Sarcoma Incidence and Survival: A Surveillance, Epidemiology, and End Results (SEER) Database Analysis

Harvey Y Wang et al. Cureus. .

Abstract

Background Clear cell sarcoma (CCS) is a rare soft tissue cancer that predominantly affects young to middle-aged adults. Current literature lacks recent and accurate estimates of patient outcomes due to the disease's low incidence and the small sample sizes in existing studies. This study aims to examine the incidence and survival of patients with CCS. Methods Patients from the Surveillance, Epidemiology, and End Results (SEER) database diagnosed with CCS between 2000 and 2019 were selected. Additional variables collected included age, sex, race, stage, metastases, tumor size, treatment status for surgery, radiation, and chemotherapy, median household income, and population size. Descriptive statistics, population-based incidence, chi-square tests, and Cox regression analyses were performed. Results A total of 268 patients were included. The population-adjusted incidence ranged from 0.012/100,000 to 0.027/100,000. The total percent change over the study period was 16.751%, and the annual percent change was 0.561%. The survival rates at one, three, and five years were 78.4%, 62.0%, and 57.1%, respectively. Cox regression results showed that Black patients (p = 0.007), tumor size greater than 4.0 cm (p = 0.033), and the presence of metastases (p = 0.040) were all associated with shorter survival. Significance The findings showed that CCS incidence has remained unchanged in recent years and that prognosis is poor. Black patients were found to have shorter survival durations. Contrary to prior findings, staging and tumor size were only significantly associated with survival in univariate analyses. Limitations include a small sample size and the constraints of variables available in the SEER database. Nonetheless, future research will benefit from assessing whether race is an independent risk factor for CCS survival and from exploring ways to improve prognosis.

Keywords: clear cell sarcoma; incidence; malignant melanoma; prognosis; seer database.

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Conflict of interest statement

Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Biomedical IRB issued approval 2004658-01. Animal subjects: All authors have confirmed that this study did not involve animal subjects or tissue. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.

Figures

Figure 1
Figure 1. Average incidence of clear cell sarcoma between 2000 and 2020.
See this image and copyright information in PMC

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