肾透明细胞癌ppt课件.pptx

cellcellsarcomasarcomaC ClearlearOf kidneyOf kidneyCONTENTS 1Introduction2Clinical presentation3CT findings4Differential diagnosis5Therapy and prognosis1Introduction u Clear cell sarcoma of the kidney(CCSK)is an uncommon renal neoplasm of childhood.It accounts for 25%of all pediatric renal malignanciesIntroductionIt is known for its morphologic diversity,aggressive behaviour,tendency to recur and metastasis to boneCCSK is the second most common renal tumor in children with a mean age of diagnosis overlapping with Wilms tumorIntroductionBone metastases occur in 40 to 60%of patients with CCSK,unlike less than 2%of patients with Wilms tumorCCSK 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 2Clinical presentation uMost of the children were taken to the hospital for abdominal mass01abdominal pain03hematuria02abdominal distentionClinical presentation 3CT findings uheterogeneous enhancement with attenuation less than that of normal renal parenchymaucontain areas of low attenuation corresponding to necrosis and cystsucystic 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 densityDiagnosisCT findingsRenal clear Renal clear Renal clear Renal clear cell carcinomacell carcinomacell carcinomacell carcinoma4Differential diagnosis1.Wilms tumor 2.Malignant rhabdoid tumor3.Renal cell carcinoma 4.LymphomaWilms 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,bilateral in 4-13%Malignant rhabdoid tumor(MRT)01.diagnosed between 0-3 years of age02.extensive metastasis at the time of diagnosis 03.synchronous brain tumor21Differential diagnosisLymphoma multiple bilateral low-attenuation massesunilateral solitary low-attenuation mass,large conglomerate masses,or unilateral nephromegalylymphadenopathy elsewhere Renal cell carcinoma makes up 1%of all pediatric renal malignancies smaller at presentation calcified in 25%43Differential diagnosis5Therapy and prognosisuFor suspicious renal lesions,it is crucial to ensure prompt diagnosis in order to select the appropriate surgical procedure and treatment Therapy and prognosisTherapyTherapyTherapyTherapyuradical nephrectomyuchemotherapyuradiotherapyuWhile prognosis traditionally has been poor,newer treatment regimens show relapse-free u cancer-specific survival rate of 100%for revised stage 1 CCSK and overall survival rate for patients with CCSK of 83%prognosisprognosisprognosisprognosisTherapy and prognosisuTo conclude CCSK is a frequently misdiagnosed tumor due to tremendous morphologic diversity where in the primary contribution of IHC is to exclude other diagnostic possibilities.The accurate and timely diagnosis is also critical because of its aggressive behaviourTHANK YOU资料可以编辑修改使用学习愉快！课件仅供参考哦，实际情况要实际分析哈！感谢您的观看感谢您的观看