PMC6243304_01
Contrast-enhanced CT scan of a 16-year-old male patient with renal Ewing's sarcoma/primitive neuroectodermal tumour. (a) Axial section showing a large heterogeneous soft tissue mass (black arrow) arising from the left kidney having areas of necrosis within and extending into the renal vein (white arrow). (b) Axial section reveals the tumour thrombus extending into the inferior vena cava and right hepatic vein (black arrow). (c) Coronal reconstruction image demonstrating the left renal mass with tumour thrombus extending along the inferior vena cava (black arrow).
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fig1
Contrast-enhanced CT scan of the abdomen showed a large lobulated heterogeneously enhancing mass of size 10.5 x 7 x 5 cm arising from the left kidney with infiltration of adjacent pararenal fat and the retroperitoneum (Figure 1a). Superiorly, the thrombus extended into the intrahepatic portion of the IVC and the right hepatic vein, causing the Budd-Chiari syndrome (Figure 1b,c).
Histopathology of a 16-year-old male patient with renal Ewing's sarcoma/primitive neuroectodermal tumour. (a) Haematoxylin and eosin stain showing malignant small, round cell tumour morphology. Renal parenchyma is not identified. (b) Some of the tumour cells show periodic acid-Schiff-positive diastase-resistant cytoplasmic granules (arrow). On immunohistochemistry, these cells show (c) strong membranous immunopositivity for cluster of differentiation 99 (box shows a high power view of the same image) and (d) focal immunopositivity for synaptophysin.
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fig2
Biopsy of the mass demonstrated monomorphic small, round cells arranged in a sheet-like pattern with round nuclei, scanty eosinophilic cytoplasm and indistinct cell outline (Figure 2a,b). Immunohistochemistry revealed positivity for cluster of differentiation 99 (MK2), vimentin and synaptophysin (Figure 2c,d).