Primary hepatic angiosarcoma with spleen metastases in an adult woman: a case report and literature review

Primary hepatic angiosarcoma (PHA) is a rare malignancy that carries a poor prognosis, accounting for less than 2% of all primary hepatic tumors. It is reported to be associated with chronic exposure to environmental carcinogens, but the majority of patients were still with unknown etiology. For patients with PHA often present with nonspecific symptoms and its rapid progression, accurate and early diagnosis is difficult and necessary. We described a 41-year old woman with no history of exposure to toxic chemicals having intermittent abdominal distention for 1 month. Imaging examinations showed multiple nodules with different sizes throughout markedly enlarged liver and spleen. Liver histology showed majority of necrotic lesions with foci of atypical cells, which displayed immunoreactivity for endothelial markers CD31, CD34 and FLi-1, supporting the diagnosis of angiosarcoma. She was finally diagnosed as PHA concomitant with spleen metastases through imaging technology combined with the histopathologically results. Then the patient showed a rapidly worsening clinical course. Finally the patient received liver transplantation and splenectomy. Unfortunately, the patient died of infection in 35 days after liver transplantation.