Clinical outcomes of molecularly confirmed clear cell sarcoma from a single institution and in comparison with data from the Surveillance, Epidemiology, and End Results registry

Abstract

BACKGROUND: The authors compared disease‐specific survival (DSS) in stage‐specific subgroups of patients with clear cell sarcoma, including those with lymph node metastases (N1M0) and those with distant metastases (N0M1). METHODS: Clinical data regarding soft tissue sarcoma patients were obtained from The University of Texas M. D. Anderson Cancer Center (MDACC) (1980‐2007) and the Surveillance, Epidemiology, and End Results (SEER) registry (1988‐2004). When possible, clear cell sarcoma diagnoses were confirmed using fluorescence in situ hybridization or reverse‐transcription polymerase chain reaction. Kaplan‐Meier estimates were used to calculate DSS, and Cox multivariate analysis was performed to identify prognostic factors. RESULTS: Fifty‐two patients at MDACC and 130 SEER patients were diagnosed with clear cell sarcoma. Five‐year DSS for the MDACC and SEER cohorts were 67% and 62%, respectively. Patients with N1M0 and N0M1 disease demonstrated significant differences in 5‐year DSS: 74% versus 14% at MDACC (P = .014) and 52% versus 0% in SEER (P = .014). After adjustment, the hazards ratio (HR) for dying was 2.79 for N1M0 disease (95% confidence interval [95% CI], 1.32‐5.91) and 11.37 (95% CI, 5.19‐24.91) for N0M1 disease compared with stage II disease (P < .001). Non‐Caucasian ethnicity (HR, 3.99; 95% CI, 2.27‐6.99 [P < .001]) and truncal tumor site (HR, 2.41; 95% CI, 1.15‐5.05 [P = .02]) were also found to be predictors of decreased DSS. CONCLUSIONS: The findings of the current study suggest that patients with N1M0 clear cell sarcoma have 5‐year DSS that is more similar to that of patients with stage III than stage IV soft tissue sarcoma. Cancer 2009. © 2009 American Cancer Society.

Prognostic Factors for Survival in Patients with Clear Cell Sarcoma: An Analysis of the Surveillance, Epidemiology, and End Results (SEER) Database

“…The median age of the patients in our study was 41.28 years (range, 6–91 years). Although Kawai et al [19] reported that gender was an independent prognostic factor, previous studies found that gender was not an independent factor for prognosis of CCS [3,12,21]. The findings of these previous studies are consistent with those of the present study.…”

“…Similar to Blazer et al [21], we found that the majority of patients were diagnosed at an early or localized stage. The 5-year disease-specific survival was 62.9%, and 10-year disease-specific survival was 51.3%.…”

“…Previous studies have shown that the prognostic factors associated with CCS are tumor size [7,9–13,19], tumors site [12], treatment [7,13], and stage [13,21]. However, only tumor size and stage were prognostic factors in the present study.…”

Sarcomas in the United States: Recent trends and a call for improved staging

“…The 5-year survival was 71.4%, which falls within the range reported in the literature and represents an increase in survival over the last decade due to improved diagnostic and therapeutic measures [9]. Tumors with higher grade, increased size, local extension, and metastasis all exhibited lower survival, as expected [10, 11]. Surgical resection is the only curative treatment for sarcoma, and surgical patients had significantly higher survival [3, 12].…”

“…However, Black patients and patients from rural areas showed lower 5-year survival when compared to Whites and patients from metropolitan areas, respectively. Further risk-adjusted histology-specific studies are mandated to understand if these differences represent a disparity in access to care among minorities and under-served populations or are a result of differences in histological make-up and tumor behavior in these populations [1, 4, 11].…”