"Emergency hepatectomy" in a 16-month-old boy with a hepatoblastoma, severe cytomegalovirus-induced pancytopenia, and intractable diarrhea

A 16-month-old boy presented with massive abdominal enlargement. Magnetic r esonance imaging (MRI) revealed a liver tumor measuring 11 x ii x IO cm. Pu lmonary metastases were detected by chest computed tomography. The diagnosi s of a hepatoblastoma was made from the patient's age, the location of the tumor, and marked elevation of alpha(1)-fetoprotein (AFP). Chemotherapy acc ording to the German Cooperative Pediatric Liver Tumor Study HB-94 was star ted, leading to a rapid and dramatic decrease in AFP levels. Despite an exc ellent response to chemotherapy, however, the abdominal distension increase d. On a second MRI scan the tumor was unchanged in size, showing large area s of necrosis and cystic transformation. In addition, the patient acquired a cytomegalovirus infection with intractable diarrhea and severe pancytopen ia. Since the patient's clinical course had worsened, the decision was made to alleviate the life-threatening tumor compression by an extended right h epatic lobectomy despite platelet-transfusion dependence and a white blood cell (WBC) count of only 300/mu l. Postoperative necrosis of the common bil e duct necessitated a secondary resection with Roux-en-Y hepaticojejunostom y. The boy recovered quickly, and the while blood and platelet counts incre ased to normal values immediately after the first surgery so that chemother apy could be finished after the postoperative recovery period. Ile is now i n good condition without evidence of local or metastatic disease 1 year aft er the primary diagnosis.