A year-old male infant presented with a 15 days history of abdominal distension and constipation. There was no history of vomiting, loss of weight or haematuria. On examination, the patient had pallor and a systolic blood pressure of 84 mmHg. The abdomen was distended and a firm, non-tender swelling was found occupying the right hypochondrial and lumbar regions. Blood and urine investigations including 24 hours vanillyl mandelic acid and chest radiograph were within normal limits. Ultrasonography showed a 12.3 x 8.7 cm round heterogeneous mass arising from the superolateral aspect of the right kidney.
At laparotomy, the tumour was removed en mass along with the right ureter. The tumour was pushing the liver medially and was superficially adherent to the right dome of diaphragm, under surface of the right lobe of liver and the inferior vena cava. There were two lymphnodes over the renal hilum which were excised. Post-operatively, he was started on etoposide and ifosfamide. The histopathology was initially given as malignant round cell tumour of uncertain histogenesis. Later, on further sectioning, it was confirmed as clear cell sarcoma of kidney (Figure 1, 2). At follow-up after six months, the child was doing well. A bone scan performed at this time was normal.
