Veno-occlusive disease of the liver in a patient with infantile fibrosarcoma on VAC regimen of the intergroup rhabdomyosarcoma study (IRS) IV

Abstract

In this communication, we report a case of veno-occlusive disease (VOD) of the liver occurring in a three-monht-old girl with infantile fibrosarcoma who was receiving vincristine (VCR) (0,025 mg/ kg, in a week), actinomycin D (Act-D) (7,5 m g/kg/day, 5 days), and cyclophosphamide (CTX) (36mg/kg) according to the Intergroup Rhabdomyosarcoma Study (IRS) IV. Five weeks into the chemotherapy, her liver rapidly enlarged and ascites, pleural effusion, hepatic encephalopathy, rapid weight gain and a refractory thrombocytopenia developed. An ultrasonographic scan of the liver revealed massive hepatomegaly and ascites. Despite aggressive treatment, her fulminant clinical course resulted in death. To the best of our knowledge, this patient is the eleventh case of VOD occuring during a VAC regimen of IRS-IV. Increased CTX dose in the VAC regimen of IRS-IV is likely to account for high prevalance of VOD of the liver.