Venoocclusive liver disease (VOD) as a complication of Wilms' tumour management in the series of consecutive 206 patients

In 4 years (1993-1996) 206 pts. with nephroblastoma were treated. All child ren were treated according to SIOP 93-01 protocol. Overall survival was 92% . In 27 cases hepatotoxic events occurred. In 10 cases, venoocclusive liver disease (VOD) was diagnosed. VOD is a syndrome associated with hepatomegal y, sudden weight gain or ascites and jaundice. It results from damage to th e endothelium of hepatic venules and necrosis of central hepatocytes with s ubsequent proliferation of fibrous tissue and occlusion of the central hepa tic veins. Dactinomycin is one of the drugs considered responsible for its development. Mean age of VOD patients was 4 yrs, however 3 of them were bel ow 1 yr. In all cases, VOD occurred during postoperative chemotherapy (mean 16th week of treatment). All patients received dactinomycin and vincristin e. Five children with right kidney tumors underwent post-operative abdomina l irradiation. Main VOD symptoms were hepatomegaly and ascites (80%). Hyper transaminasaemia, as well as, on ultrasound, gallbladder wall thickening an d/or free abdominal fluid were observed. Median VOD duration was 27 days an d its course was usually temporary and self-limiting. However, in 2 cases r ecurrent VOD episodes were noted. All children received supportive treatmen t only. In 6 cases, VOD resulted in chemotherapy delay or drug reductions, while in 4 others chemotherapy was completed preliminarily. Nevertheless it did not affect patients' outcome overall survival in VOD group was 90%. Conclusions: Total 5% VOD frequency is similar to other reports. Infants an d children receiving abdominal irradiation seem to be at special risk of VO D development.