MMK Shing, KW Chik, H Lai, CK Li, P Yuen, K Liu, KH Lee, CK Yeung

Since the opening of the Prince of Wales Hospital in May, 1984, there were twenty-nine children who were diagnosed to have renal tumours (23 Wilms' tumours, 3 clear cell sarcoma, 3 mesoblastic nephroma). The clinical features, treatment and outcomes of Wilms' tumour were analysed retrospectively.

The twenty-three patients with Wilms' tumour were all Chinese patients except 1 Caucasian boy who went back to United Kingdom after initial treatment, hence he was excluded from further analysis. The male to female ratio of the remaining twenty-two patients was 1.2:1. The mean age at presentation was 2.7 years (0.1 to 9.9 years). The clinical presentation were accidental discovery of abdominal mass by parents or clinicians, abdominal pain, gross haematuria, vomiting and diarrhoea. One patient who had bilateral Wilms' tumour was found to have corrected transposition of the great arteries and polycythaemia (haemoglobin 19.3 gm/dl). None of the patients had hypertension. Ten patients had a family history of malignancy. The kidneys involved by Wilms' tumour were twelve in the left kidneys, nine in the right kidneys and one in both kidneys. One patient had tumour thrombus in the renal vein, and another patient had tumour thrombus in renal vein and inferior vena cava. There was no metastasis to lung, liver and bone. Immediate nephrectomy was performed in sixteen patients. Tumour debulking operations were performed in two patients because of huge tumours. Four patients had biopsy of the tumours, followed by chemotherapy to shrink do n the tumours, and then nephrectomy. The histological diagnosis were favourable Wilms' tumour (n= 19), Wilms' tumour with focal anaplastic changes (n= 1), and unknown (n=2). The staging of disease (National Wilms' tumour Study IV) were Stage I (n= 12), Stage 11(6), Stage III (n=3) and Stage V (n= 1). Seven patients had tumour bed radiotherapy after nephrectomy. Twenty-one patients had complete response to treatment, while 1 patient had progressive disease. Two patients had pulmonary relapses. Three patients died because of pulmonary relapse (n=1), anthracycline induced cardiomyopathy (n=1) and progressive disease (n=1). The overall survival in this group of patients was 86.4% with a median follow up of 5 years (range: 6 months to 18 years and 2 months).

Conclusion With multi-disciplinary approach, there is high chance of long term survival for patients with Wilms' tumours.