肾透明细胞癌Renal clear cell carcinoma.pptxVIP

cell sarcoma Clear Of kidney CONTENTS Introduction Clinical presentation CT findings Differential diagnosis Therapy and prognosis 1 Introduction Clear cell sarcoma of the kidney (CCSK) is an uncommon renal neoplasm of childhood. It accounts for 2–5% of all pediatric renal malignancies Introduction It is known for its morphologic diversity, aggressive behaviour, tendency to recur and metastasis to bone CCSK is the second most common renal tumor in children with a mean age of diagnosis overlapping with Wilms tumor Introduction Bone metastases occur in 40 to 60% of patients with CCSK, unlike less than 2% of patients with Wilms’ tumor CCSK was first described as “bone metastasizing renal tumor of childhood” since it has predilection for skeletal metastasis The peak incidence is between 3 and 5 years of age. It is very rare in infants younger than 6 months Introduction It is the most frequently misdiagnosed renal tumor in children, as it is unusual, has varied morphology, and there are no specific diagnostic markers 2 Clinical presentation Most of the children were taken to the hospital for abdominal mass 01 abdominal pain 03 hematuria 02 abdominal distention Clinical presentation 3 CT findings heterogeneous enhancement with attenuation less than that of normal renal parenchyma contain areas of low attenuation corresponding to necrosis and cysts cystic foci CT findings Axial and coronal image show a large right hypochondrial soft tissue mass, almost totally infiltrating the right kidney with only a little residual normal renal parenchyma 2. CT findings Solid component presented heterogeneous enhancement with a large cystic area and showed tumor vessels with high density Diagnosis CT findings Renal clear cell carcinoma 4 Differential diagnosis 1.Wilms tumor 2.Malignant rhabdoid tumor 3.Renal cell carcinoma 4.Lymphoma Wilms tumor 01.heterogeneous solid renal mass with a “claw sign” 02.tumor extension into the renal vein and IVC in 5-10% 03.most frequently m