Treatment and outcome of patients with relapsed clear cell sarcoma of the kidney (CCSK): A combined SIOP and AIEOP study.

Overall survival nomogram and relapse-related factors of clear cell sarcoma of the kidney: A study based on published patients

“…Most of them were single-center observations with small samples. There is only a large cohort of 37 out of 237 patients with relapse (5). The second aim of this study was to obtain a large sample to observe the consistency of the relapse characteristics we obtained with those reported in previous studies, and to explore the relapse-related factors.…”

“…The first purpose of our study was to obtain a large sample to explore the predictors of OS and establish an easy-to-use nomogram to fill the gap in the accurate prediction of CCSK prognosis. Clinical research of CCSK in recent years showed that the combined treatment of surgical resection + chemotherapy + radiotherapy has a 5-years RFS of 72.9-85% and a 5-years OS of 74.5-88% (4)(5)(6)(7)(8)24). It can be seen that with the optimization of diagnosis and treatment guidelines, the survival rate had a good performance, but some patients still have poor prognoses.…”

“…Rarity represents the greatest barrier to advances in the diagnosis and treatment of CCSK. There have been several follow-up clinical studies on CCSK relapse (5,(16)(17)(18)32). Most of them were single-center observations with small samples.…”

“…Clear cell sarcoma of the kidney (CCSK) is a rare pediatric malignant tumor most common in children aged 2-3 years old and accounting for only 3-5% of children's renal tumors (1)(2)(3). The clinical features and therapeutic regimen of CCSK which is similar to high-risk nephroblastoma have been systematically studied by the North American National Wilms' Tumor Study Group (NWTSG) and the European International Society of Pediatric Oncology (SIOP) (4)(5)(6)(7)(8)(9). The rarity of the tumor limits the study breadth of CCSK, so the prognosis prediction is still poorly understood.…”

“…A combined SIOP and Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) study showed that relapse occurs in about 16% of patients (5). Several follow-up clinical studies on relapse of CCSK showed that relapse patients who receive only primary treatment with resection + chemotherapy + radiotherapy exhibit a poor prognosis due to the absence of effective treatment standards for relapsed CCSK (16)(17)(18).…”