A 47-year-old woman without past medical history was admitted due to acute right upper abdominal pain for 8 h and syncope prior to admission. She had a history of taking oral contraceptive pills 4-6 months per year, for 7 years. Physical examination revealed vital signs: BP 100/60 mm Hg, pulse rate 115/min. Her body mass index was 33.6 kg/m2. Abdomen examination showed marked tenderness at the right upper quadrant; the liver was palpated 8 cm below the costal margin with a liver span of 20 cm with mild tenderness, no splenomegaly, and no shifting dullness.
Laboratory investigations showed: WBC count 8,200/dL, Hb 7.4 g/dL, and platelet count 592 x 103/dL, AST 34 U/L, ALT 23 U/L, ALP 335 U/L, TB 1.22 mg%, DB 0.73 mg%; coagulogram was in normal limit, AFP was 2.72 ng/mL.
The emergency CT scan revealed acute rupture of liver tumor by evidence of acute clot and acute hematoma. She was provisionally diagnosed with ruptured hepatic adenoma and underwent emergency hepatic embolization. The angiography finding revealed a large hepatic mass at the anterior aspect of the right hepatic lobe, receiving arterial supply from the anterior branch of the right branch of the hepatic artery. Eventually, embolization was performed to stop bleeding. The scheduled CT scan 2 weeks after embolization showed a larger diameter of hemorrhagic mass from 20.7 to 23.0 cm, lysed perihepatic hematoma, without distance metastasis (Fig. 2).
Four weeks after embolization, she underwent extended right hepatectomy with wedge diaphragm and primary repair. The operation demonstrated a huge liver mass with intratumoral hemorrhage, displaced IVC, and mediastinum. The specimen consisted of a liver tumor size 28 x 20 x 14 cm and weighing 2,300 g. The tumor appeared to be a light-brownish rubbery mass with a massive hemorrhage. The histopathological finding was compatible with angiosarcoma (French system grade III) and free tumor margin. The immunochemical staining was positive for CD34 and factor VIII, consistent with PHA (Fig. 3).
The follow-up CT scan was performed after surgery, and it showed multiple new nodules about 0.5-2 cm in the left lobe liver, a heterogenous enhancing mass size 3.7 cm in the right subdiaphragmatic space; peritoneal thickening was also noted. Thus, the physicians planned to treat her with chemotherapy, doxorubicin. However, she refused chemotherapy treatment because of her poor clinical condition, and she passed away 4 months later.