A 55-year-old woman with a medical history of type 2 diabetes mellitus, presented with right upper abdominal pain and significant weight loss for 5 months, and progressive jaundice for 2 months. She denied a history of carcinogen exposure. Physical examination showed hepatomegaly 4 cm below the right costal margin with a liver span of 15 cm. Liver examination described a rubbery to firm consistency, blunt edge, and smooth surface. Other findings were unremarkable except mild icteric sclerae. The complete blood count revealed: WBC 9,820/dL, Hb 12.0 g/dL, and platelet count 98 x 103/dL. The liver function test showed: AST 44 U/L, ALT 20 U/L, ALP 449 U/L, TB 3.81 mg%, DB 2.57 mg%. The serum AFP was 2.16 ng/mL (normal <7 mg/mL).
Multiphase CT scan demonstrated diffused multiple arterial enhancing infiltrating nodules varying in size, scattered throughout the entire liver, with ascites and mesenteric fat reticulation suspicious of carcinomatosis peritonei.
Laparoscopic liver biopsy was performed for diagnosis, and the specimen consisted of a piece of light brown irregular soft tissue. Histopathological findings showed the presence of diffused proliferation of abnormal small vessels with diffused hemorrhage. The abnormal vessels showed enlarged endothelial cells with nuclear atypia. Immunohistochemical study showed positive staining for CD34 and factor VIII in the endothelial cells. Histopathological study was consistent with PHA (Fig. 1).
She received a total of five cycles of paclitaxel with a 20% dose reduction. The following posttreatment CT scans showed progression of the disease with peritoneal metastasis, and she later decided to stop treatment. She survived for 19 months and 14 months after symptom onset and treatment initiation, respectively.