Primary hepatic angiosarcoma: A report of two cases and literature review

Abstract

Primary hepatic angiosarcoma (PHA) is a rare malignancy that carries a poor prognosis. Of 1500 patients who underwent hepatectomy for primary hepatic tumors between 1994 and 2013 at our center, two patients were pathologically diagnosed with PHA. Clinical characteristics, treatment modalities, and outcomes of the two patients were collected and analyzed. Both patients underwent hepatectomy and had a postoperative survival time of 8 and 16 mo, respectively. A search of PubMed yielded eight references reporting 35 cases of PHA published between 2004 and 2013. On the basis of the presented cases and review of the literature, we endorse complete surgical resection as the mainstay definitive treatment of PHA, with adjuvant postoperative chemotherapy potentially improving survival. Palliative chemotherapy is an option in advanced hepatic angiosarcoma.

Hepatic Epithelioid Hemangioendothelioma: Difficult Differential Diagnosis from Angiosarcoma

“…Hepatic EHE has a predilection for age 40-50 years, while angiosarcoma has a predilection for age 60-80 years. Symptoms of hepatic EHE and angiosarcoma are very similar: abdominal pain, weight loss, weakness, anorexia, and asymptomatic [3,6]. Imaging studies sometimes reveal similar findings between hepatic EHE and angiosarcoma.…”

“…Moreover, hepatic EHE tends to be multiple and located in the periphery [3]. Angiosarcoma presents differently on CT imaging according to its various morphological patterns which depend on its necrosis and hemorrhage [6]. Pathologically, hepatic EHE tends to show preserved hepatic acinar structure, severe sclerosis, moderate cell density, and mild dysplasia.…”

Primary Hepatic Angiosarcoma: A Rare Liver Malignancy – Varying Manifestations but Grave Prognosis

“…PHA is a liver tumor originating from endothelial and fibroblastic tissue, accounting for 0.1-2% of all primary liver malignancies [1]. Despite its rarity, PHA remains the most common malignant mesenchymal cell tumor of the liver [2]. PHA generally develops in the sixth to seventh decade of life and is predominantly found in males with a male-to-female ratio of 2-4:1 [11].…”

“…Vinyl chloride, in plastic fabrication, has also been shown to have an established association with PHA with the hazard ratio approximately 10-to 15-fold. Besides, other agents including arsenic androgen, anabolic steroids, cyclophosphamide, phenelzine, and copper are proposed as possible carcinogens [2,9]. However, these cases are rare and lack explicit associations.…”

“…These symptoms include abdominal pain, fatigue, weight loss, jaundice, and anorexia. Physical examination can reveal hepatomegaly, ascites, or jaundice [2]. Few patients present with hemoperitoneum secondary to tumor rupture, which was reported in 17-27% of cases [3,4].…”