• The Journal of the Korean bone and joint tumor society
• /
• v.4 no.2
• /
• pp.94-98
• /
• 1998

Clear cell sarcoma of the kidney (CCSK) is a rare malignant tumor of the renal origin in childhood, distinguished from Wilms tumor by its pathologic and clinical features. Bone metastasis is one of the characteristic clinical features. The common site of metastasis of the clear cell Sarcoma of the kidney is axial skeleton including skull, spines, ribs and femur. A cases of clear cell Sarcoma of the kidney presented to us, which solely metastasized to the hand bones without metastasizing to any other tissues including axial skeleton. We report this case with review of literatures.

• Advances in pediatric surgery
• /
• v.7 no.2
• /
• pp.162-165
• /
• 2001

Clear Cell Sarcoma of the Kidney(CCSK) is a rare malignant childhood tumor with frequent metastasis to the bone. We report a case of right sided in a 5 month-old girl. A radical nephrectomy was performed. It was clinical stage III with renal capsular invasion and lymph node metastasis by the classification of NWTS-5. Histologic examination revealed the classic pattern of CCSK. Postoperative adjuvant chemotherapy with doxorubicin and radiotheraphy were applied.

• Korean Journal of Radiology
• /
• v.22 no.7
• /
• pp.1185-1193
• /
• 2021

Objective: Clear cell sarcoma of the kidney (CCSK) is the second-most common but extremely rare primary renal malignancy in children after Wilms' tumor. The aims of this study were to evaluate the imaging features that could distinguish between CCSK and Wilms' tumor and to assess the features with diagnostic value for identifying CCSK. Materials and Methods: We reviewed the initial contrast-enhanced abdominal-pelvic CT scans of children with CCSK and Wilms' tumor between 2010 to 2019. Fifty-eight children (32 males and 26 females; age, 0.3-10 years), 7 with CCSK, and 51 with Wilms' tumor, were included. The maximum tumor diameter, presence of engorged perinephric vessels, maximum density of the tumor (Tmax) of the enhancing solid portion, paraspinal muscle, contralateral renal vein density, and density ratios (Tmax/muscle and Tmax/vein) were analyzed on the renal parenchymal phase of contrast-enhanced CT. Fisher's exact tests and Mann-Whitney U tests were conducted to analyze the categorical and continuous variables, respectively. Logistic regression and receiver operating characteristic curve analyses were also performed. Results: The age, sex, and tumor diameter did not differ between the two groups. Engorged perinephric vessels were more common in patients in the CCSK group (71% [5/7] vs. 16% [8/51], p = 0.005). Tmax (median, 148.0 vs. 111.0 Hounsfield unit, p = 0.004), Tmax/muscle (median, 2.64 vs. 1.67, p = 0.002), and Tmax/vein (median, 0.94 vs. 0.59, p = 0.002) were higher in the CCSK compared to the Wilms' group. Multiple logistic regression revealed that engorged vessels (odds ratio 13.615; 95% confidence interval [CI], 1.770-104.730) and Tmax/muscle (odds ratio 5.881; 95% CI, 1.337-25.871) were significant predictors of CCSK. The cutoff values of Tmax/muscle (86% sensitivity, 77% specificity) and Tmax/vein (71% sensitivity, 86% specificity) for the diagnosis of CCSK were 1.97 and 0.76, respectively. Conclusion: Perinephric vessel engorgement and greater tumor enhancement (Tmax/muscle > 1.97 or Tmax/vein > 0.76) are helpful for differentiating between CCSK and Wilms' tumor in children aged below 10 years.