课件：肾透明细胞癌.pptx

sarcomaOf kidneycellClear45123IntroductionClinical presentationCONTENTS CT findingsDifferential diagnosisTherapy and prognosisIntroduction 1 Clear cell sarcoma of the kidney (CCSK) is an uncommon renal neoplasm of childhood. It accounts for 2–5% of all pediatric renal malignanciesIntroductionCCSK is the second most common renal tumor in children with a mean age of diagnosis overlapping with Wilms tumorIt is known for its morphologic diversity, aggressive behaviour, tendency to recur and metastasis to boneIntroductionCCSK was first described as “bone metastasizing renal tumor of childhood” since it has predilection for skeletal metastasis Bone metastases occur in 40 to 60% of patients with CCSK, unlike less than 2% of patients with Wilms’ tumorIntroductionThe peak incidence is between 3 and 5 years of age. It is very rare in infants younger than 6 months It is the most frequently misdiagnosed renal tumor in children, as it is unusual, has varied morphology, and there are no specific diagnostic markers Clinical presentation 2Most of the children were taken to the hospital for abdominal massClinical presentation abdominal pain01hematuria02abdominal distention03CT findings 3heterogeneous enhancement with attenuation less than that of normal renal parenchymacontain areas of low attenuation corresponding to necrosis and cystscystic fociCT findingsAxial and coronal image show a large right hypochondrial soft tissue mass, almost totally infiltrating the right kidney with only a little residual normal renal parenchyma2.CT findingsSolid component presented heterogeneous enhancement with a large cystic area and showed tumor vessels with high densityCT findingsRenal clear cell carcinomaDiagnosisDifferential diagnosis41.Wilms tumor 2.Malignant rhabdoid tumor3.Renal cell carcinoma 4.LymphomaDifferential diagnosis1Wilms tumor01.heterogeneous solid renal mass with a “claw sign”02.tumor extension into the renal vein and IVC in 5-10%03.most frequently metastasizes to the lungs,bil