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. 2021 May 20:9:675373.
doi: 10.3389/fped.2021.675373. eCollection 2021.

Childhood Clear Cell Sarcoma of Kidney: Incidence and Survival

Hui Gao  1 , Qi-Yuan Cheng  1 , Qian Zhao  1 , Long-Xiang Tao  2 , Cheng Zhang  3
Affiliations

Childhood Clear Cell Sarcoma of Kidney: Incidence and Survival

Hui Gao et al. Front Pediatr. .

Abstract

This study is to describe current incidence of childhood clear cell sarcoma of kidney (CCSK) and to investigate the present survival of this cancer. Surveillance, Epidemiology, and End Result (SEER) data was used to identify children with CCSK and Wilms tumor (WT) aged 0-19 years in the US. Age-adjusted incidences were estimated over the decades. Age- and sex-specific epidemiology was also presented. Propensity score matching was used to balance features of CCSK and WT cases. Log rank test was used to compare survivals and Cox regression was used to evaluate independent effects of factors. The present age-adjusted incidence of childhood CCSK was 0.205 per million, which remained stable for years and ranked third in all pediatric renal tumors. The incidence rate ratios for boy and age under 4 were 3 and 21, respectively. The current 5-year overall survival (OS) rate for CCSK was 87%, which is not evidently inferior to that for WT (90%); however the outcome of CCSK was significantly poorer if both groups were well-balanced (OS rate was 86 vs. 95%). Early year of diagnosis and distant metastasis were independent survival factors. In conclusion, occurrence of CCSK remains stable over the years, with an age-adjusted incidence of 0.205 per million. Boy and age under 4 are risk factors for tumor development. CCSK currently has a favorable outcome but its nature may be more aggressive than common kidney tumor, which in turn proves efficacy of modern treatment.

Keywords: clear cell sarcoma; epidemiology; incidence; kidney neoplasms; survival.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Age and sex-specific incidence trend of childhood CCSK (0–19 years). (A) Age and sex-specific number of childhood CCSK during 1986–2017; (B) Age and sex-specific trend plot of age-adjusted incidence of childhood CCSK during 1986–2017. (C) Group-specific age-adjusted incidence for CCSK. CCSK, clear cell sarcoma of kidney.
Figure 2
Figure 2
Survival curves of children (0–19 years) with WT and CCSK. (A) Before propensity score matching; (B) After propensity score matching. WT, Wilms tumor; CCSK, clear cell sarcoma of kidney.
See this image and copyright information in PMC

References

    1. Sotelo-Avila C, Gonzalez-Crussi F, Sadowinski S, Gooch WR, Pena R. Clear cell sarcoma of the kidney: a clinicopathologic study of 21 patients with long-term follow-up evaluation. Hum Pathol. (1985) 16:1219–30. 10.1016/S0046-8177(85)80034-4 - DOI - PubMed
    1. Miniati D, Gay AN, Parks KV, Naik-Mathuria BJ, Hicks J, Nuchtern JG, et al. Imaging accuracy and incidence of Wilms' and non-Wilms' renal tumors in children. J Pediatr Surg. (2008) 43:1301–7. 10.1016/j.jpedsurg.2008.02.077 - DOI - PubMed
    1. van den Heuvel-Eibrink MM, Grundy P, Graf N, Pritchard-Jones K, Bergeron C, Patte C, et al. Characteristics and survival of 750 children diagnosed with a renal tumor in the first seven months of life: a collaborative study by the SIOP/GPOH/SFOP, NWTSG, and UKCCSG Wilms tumor study groups. Pediatr Blood Cancer. (2008) 50:1130–4. 10.1002/pbc.21389 - DOI - PubMed
    1. Seibel NL, Chi YY, Perlman EJ, Tian J, Sun J, Anderson JR, et al. Impact of cyclophosphamide and etoposide on outcome of clear cell sarcoma of the kidney treated on the National Wilms Tumor Study-5 (NWTS-5). Pediatr Blood Cancer. (2019) 66:e27450. 10.1002/pbc.27450 - DOI - PMC - PubMed
    1. Doganis D, Zborovskaya A, Trojanowski M, Zagar T, Bouka P, Baka M, et al. Wilms tumour event-free and overall survival in Southern and Eastern Europe: Pooled analyses of clinical data from four childhood cancer registries (1999-2017). Eur J Cancer. (2019) 115:37–46. 10.1016/j.ejca.2019.04.008 - DOI - PubMed

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