Comments: CCSK is perhaps the most frequently misdiagnosed renal tumor in children. It is resistant to conventional Wilms tumor therapy but responds to regimens containing doxorubicin; hence, accurate diagnosis is critical. It was previously called bone-metastasizing renal tumor of childhood.
Comments: Tumor consists of monotonous sheets of cells with pale cytoplasm and indistinct cytoplasmic borders. The cytoplasm may not be “clear” and should not be solely relied upon to make the diagnosis. For an excellent review article on CCSK refer to: Am J Surg Pathol 2000 Jan; 24(1)4-18.