Clear cell sarcoma of the kidney
Updates to Article Attributes
Clear cell sarcoma (CCS) of the kidney are a rare mesenchymal tumour that account for ~5% of primary renal neoplasms in paediatric population 1.
Epidemiology
CCS is the second most common primary malignant renal neoplasm after Wilms tumour, with an annual incidence of 20 cases in the United States 2,3. The mean age of diagnosis is 3 years 2, although it is very rare before the age of 6 months 3,4. Males are noticed to be affected more with a M:F = 2:1 1.
Clinical presentation
CCS usually presents with a palpable abdominal mass; with hematuria and bone pain being less common.
Pathology
CCS appears as a large solid mass, usually unilateral and unifocal, replacing most of the kidney or centred in the medulla 1. The cut surface appears gelatinous due to abundance of extracellular mucopolysaccharides. Hemorrhage and necrosis are frequent finding (70%) 2.
Histologically, nine subtypes have been described 1. Most of these, however, will show some of the classic features of the tumour microscopically, consisting of cords or nests of cells surrounded by delicate branching cellular fibrovascular septa akin to chicken wire.
Although the margins of the tumour appear well defined grossly, they demonstrate infiltration in to adjacent renal parenchyma with entrapment of the tubules on high power field microscopy. The entrapped tubules may become extensively dilated, giving the apparent cysts seen on gross tumour inspection 2.
Radiographic features
CT
These tumours usually enhance heterogeneously and to a lesser extent than the adjacent kidenykidney, with non enhancing foci representing hemorrhage and necrosis 5. They often cross the midline. Calcification is uncommon 5.
MRI
CCS usually appears low to intermediate on T1 weighted images and high signal on T2WI with cystic areas.
Treatment and prognosis
CCS has worse prognosis compared to Wilms tumours, especially in those younger than 12 months of age 3. The mainstay treatment is radical nephrectomy with lymph node dissection. Relapse rate is high (20-40% of patients) 4.
Differential diagnosis
The imaging appearances for CCS are non-specific and can be very similar to other primary renal neoplasms, especially Wilms tumour which is much more common. Finding of bone metastasis could suggest CCS rather than Wilms tumour 1.
-<p><strong>Clear cell sarcoma (CCS</strong>)<strong> of the kidney</strong> are a rare mesenchymal tumour that account for ~5% of primary renal neoplasms in paediatric population <sup>1</sup>. </p><h4>Epidemiology</h4><p>CCS is the second most common primary malignant renal neoplasm after <a href="/articles/wilms-tumour">Wilms tumour</a>, with an annual incidence of 20 cases in the United States <sup>2,3</sup>. The mean age of diagnosis is 3 years <sup>2</sup>, although it is very rare before the age of 6 months <sup>3,4</sup>. Males are noticed to be affected more with a M:F = 2:1 <sup>1</sup>. </p><h4>Clinical presentation</h4><p>CCS usually presents with a palpable abdominal mass; with hematuria and bone pain being less common. </p><h4>Pathology</h4><p>CCS appears as a large solid mass, usually unilateral and unifocal, replacing most of the kidney or centred in the medulla <sup>1</sup>. The cut surface appears gelatinous due to abundance of extracellular mucopolysaccharides. Hemorrhage and necrosis are frequent finding (70%) <sup>2</sup>. </p><p>Histologically, nine subtypes have been described <sup>1</sup>. Most of these, however, will show some of the classic features of the tumour microscopically, consisting of cords or nests of cells surrounded by delicate branching cellular fibrovascular septa akin to chicken wire. </p><p>Although the margins of the tumour appear well defined grossly, they demonstrate infiltration in to adjacent renal parenchyma with entrapment of the tubules on high power field microscopy. The entrapped tubules may become extensively dilated, giving the apparent cysts seen on gross tumour inspection <sup>2</sup>. </p><h4>Radiographic features</h4><h5>CT</h5><p>These tumours usually enhance heterogeneously and to a lesser extent than the adjacent kideny, with non enhancing foci representing hemorrhage and necrosis <sup>5</sup>. They often cross the midline. Calcification is uncommon <sup>5</sup>. </p><h5>MRI</h5><p>CCS usually appears low to intermediate on T1 weighted images and high signal on T2WI with cystic areas. </p><h4>Treatment and prognosis</h4><p>CCS has worse prognosis compared to Wilms tumours, especially in those younger than 12 months of age <sup>3</sup>. The mainstay treatment is radical nephrectomy with lymph node dissection. Relapse rate is high (20-40% of patients) <sup>4</sup>. </p>- +<p><strong>Clear cell sarcoma (CCS</strong>)<strong> of the kidney</strong> are a rare mesenchymal tumour that account for ~5% of primary renal neoplasms in paediatric population <sup>1</sup>. </p><h4>Epidemiology</h4><p>CCS is the second most common primary malignant renal neoplasm after <a href="/articles/wilms-tumour">Wilms tumour</a>, with an annual incidence of 20 cases in the United States <sup>2,3</sup>. The mean age of diagnosis is 3 years <sup>2</sup>, although it is very rare before the age of 6 months <sup>3,4</sup>. Males are noticed to be affected more with a M:F = 2:1 <sup>1</sup>. </p><h4>Clinical presentation</h4><p>CCS usually presents with a palpable abdominal mass; with hematuria and bone pain being less common. </p><h4>Pathology</h4><p>CCS appears as a large solid mass, usually unilateral and unifocal, replacing most of the kidney or centred in the medulla <sup>1</sup>. The cut surface appears gelatinous due to abundance of extracellular mucopolysaccharides. Hemorrhage and necrosis are frequent finding (70%) <sup>2</sup>. </p><p>Histologically, nine subtypes have been described <sup>1</sup>. Most of these, however, will show some of the classic features of the tumour microscopically, consisting of cords or nests of cells surrounded by delicate branching cellular fibrovascular septa akin to chicken wire. </p><p>Although the margins of the tumour appear well defined grossly, they demonstrate infiltration in to adjacent renal parenchyma with entrapment of the tubules on high power field microscopy. The entrapped tubules may become extensively dilated, giving the apparent cysts seen on gross tumour inspection <sup>2</sup>. </p><h4>Radiographic features</h4><h5>CT</h5><p>These tumours usually enhance heterogeneously and to a lesser extent than the adjacent kidney, with non enhancing foci representing hemorrhage and necrosis <sup>5</sup>. They often cross the midline. Calcification is uncommon <sup>5</sup>. </p><h5>MRI</h5><p>CCS usually appears low to intermediate on T1 weighted images and high signal on T2WI with cystic areas. </p><h4>Treatment and prognosis</h4><p>CCS has worse prognosis compared to <a title="Wilms tumours" href="/articles/wilms-tumour">Wilms tumours</a>, especially in those younger than 12 months of age <sup>3</sup>. The mainstay treatment is radical nephrectomy with lymph node dissection. Relapse rate is high (20-40% of patients) <sup>4</sup>. </p><h4>Differential diagnosis </h4><p>The imaging appearances for CCS are non-specific and can be very similar to other primary renal neoplasms, especially <a title="Wilms tumour" href="/articles/wilms-tumour">Wilms tumour</a> which is much more common. Finding of bone metastasis could suggest CCS rather than Wilms tumour <sup>1</sup>. </p>
Unable to process the form. Check for errors and try again.