Abstract
Hemophilia A is a congenital bleeding disorder due to factor VIII deficiency. Particular attention must be paid when the patient with the disorder has surgery. This time we safely performed hepatic resection for a liver tumor in a patient with hemophilia A and diagnosed it as angiomyolipoma (AML) postoperatively. It was difficult to distinguish from hepatocellular carcinoma (HCC) preoperatively. The case involved a 74-year-old man who had been treated for hepatitis C, hemophilia A and diabetes. Abdominal CT scan showed a mass 1.2cm in diameter in the lateral segment of the liver and a mass 1.6cm in diameter in the right kidney. The liver tumor was likely to be hepatocellular carcinoma and the kidney tumor to be renal cell carcinoma. Laparoscopic assisted partial hepatectomy and right nephrectomy were performed. Microscopic findings demonstrated that the tumor was composed of abnormal blood vessels, smooth muscle cells and adipose cells. Immunohistochemical study showed positive staining for homatropine methylbromide 45 (HMB-45). We diagnosed the liver tumor as hepatic AML. The kidney tumor was an eosinophilic granular cell tumor and showed different histology from that of the hepatic tumor. The patient's postoperative course was uneventful and he was discharged from the hospital on the 17th postoperative day. He has been followed in our clinic. Hepatic AML was difficult to diagnose before surgery and required surgical treatment for pathological diagnosis.
