A rare paediatric renal tumour, clear cell sarcoma of kidney

Abstract

Clear cell sarcoma of kidney is a rare paediatric malignant renal neoplasm which affects children in the 2nd and 3rd year of life. It has a propensity to metastasize to bones, lungs and brain and has a tendency to recur after treatment. The renal biopsy showed classic pattern of clear cell sarcoma of kidney confirmed with immunohistochemical markers and the subsequent post neoadjuvant nephrectomy showed predominantly myxoid pattern possibly due to treatment effects. Since the tumour mimics other paediatric malignant neoplasms with small round blue cell morphology, immunohistochemical markers play a prime role in differentiating them for effective treatment with doxorubicin. Due to aggressive nature and poor prognostic features of clear cell sarcoma of the kidney, it is important to diagnose this tumour and exclude the common neoplasms occurring at this age group prior to neoadjuvant treatment. 1.Argani P, Perlman EJ, Breslow NE, et al. Clear cell sarcoma of the kidney: a review of 351 cases from the National Wilms Tumor Study Group Pathology Center. Am J Surg Pathol 2000; 24: 4–18.2.Ooms AHAG, Vujanić GM, D'Hooghe E, et al. Renal tumors of childhood-a histopathologic pattern-based diagnostic approach. Cancers 2020; 12: 729.3.Kao YC, Sung YS, Zhang L, et al. BCOR overexpression is a highly sensitive marker in round cell sarcomas with BCOR genetic abnormalities. Am J Surg Pathol 2016; 40: 1670–8.