肾透明细胞癌

Introduction
The 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
Introduction
CCSK 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’ tumor
CT findings
Diagnosis
4
Differential diagnosis
1.Wilms tumor 2.Malignant rhabdoid tumor 3.Renal cell carcinoma 4.Lymphoma
Differential diagnosis
1
Wilms tumor 01.heterogeneous solid renal mass with a “claw sign” 02.tumor extension into the renBaidu Nhomakorabeal 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 age 02.extensive metastasis at the time of diagnosis 03.synchronous brain tumor
2
Differential diagnosis
normal renal parenchyma
CT findings
Solid component presented heterogeneous enhancement with a large cystic area and showed tumor vessels with high density
cystic foci
CT findings
Axial and coronal image show a large
right hypochondrial soft tissue mass,
almost totally infiltrating the right
2.
kidney with only a little residual
3
Renal cell carcinoma
makes up 1% of all pediatric renal malignancies smaller at presentation calcified in 25%
Lymphoma
multiple bilateral low-attenuation masses
2
Clinical presentation
Most of the children were taken to the hospital for abdominal mass
Clinical presentation
01 abdominal pain 02 hematuria
03 abdominal distention
unilateral solitary low-attenuation mass,
large conglomerate masses, or unilateral
nephromegaly
lymphadenopathy elsewhere
4
5
Therapy and prognosis
For suspicious renal lesions, it is crucial to ensure prompt diagnosis in order to select the appropriate surgical procedure and treatment
3
CT findings
heterogeneous enhancement with attenuation less than that of normal renal parenchyma
contain areas of low attenuation corresponding to necrosis and cysts
1 Introduction 2 Clinical presentation 3 CT findings 4 Differential diagnosis 5 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
CCSK is the second most common renal tumor in children with a mean age of diagnosis overlapping with Wilms tumor
It is known for its morphologic diversity, aggressive behaviour, tendency to recur and metastasis to bone