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

Abstract

Primary hepatic angiosarcoma (PHA) is a rare mesenchymal liver tumor, accounting for 0.1-2% of primary liver malignancies. The clinical presentations of PHA are variable, from asymptomatic to liver failure or complicated with tumor rupture. The diagnosis of PHA is difficult due to the lack of specific clinical manifestation and investigation results, which can be confused with other liver tumors resulting in late diagnosis. However, there is currently a paucity of effective therapeutic approaches. We advocate early diagnosis with radiological imaging and histopathology because most of them are diagnosed in late-stage and carry a grave prognosis. Surgical resection remains the mainstay of treatment, which can significantly prolong survival. Chemotherapy, including transarterial chemoembolization, is an option for palliative treatment. Unfortunately, molecular treatment has limited efficacy and liver transplantation is also not recommended due to high rate of recurrence. We present a case series of four patients with biopsy-proven PHA which had distinct presentations and clinical courses.

Keywords: Hepatic angiosarcoma; Intra-tumoral hemorrhage; Primary hepatic angiosarcoma.

Fig. 1

Histopathological examination of tumor tissue in case 2. a Histopathological study (hematoxylin-eosin stain, ×40) showed the tumor cells (T) grow along sinusoids adjacent to hepatic cords (L). b The tumor cells appearabundant, poorly defined cell borders, pleomorphic with hyperchromatic nuclei. The abnormal vessels also showed enlarged endothelial cells with nuclear atypia (×400). The Immunohistochemical study showed positive staining for CD 34 (c) and factor VIII (d).

Fig. 2

Multiphase CT scan of liver in case 3 demonstrates a large hemorrhagic hepatic mass 15 × 20 × 23 cm in size with an enhanced solid part at the anterosuperior part on plain phase.

Fig. 3

Histopathological finding of primary hepatic angiosarcoma in case 3. a Histological examination of tumor tissue (hematoxylin-eosin stain, ×40) showed an area of the tumor tissue (T) infiltrated in the liver tissue (L). b The tumor cells present diffuse proliferation of abnormal small vessels and enlargement of endothelial cells with nuclear atypia (×400). The immunohistochemical study of the same tissue showed positive staining for CD 34 (c) and factor VIII marker (d), which indicated hepatic angiosarcoma.

Fig. 4

Multiphase CT scan of the liver in case 4 illustrates multiple scattered hypervascular infiltrative masses and nodules, about 1–9 cm in size in an almost entire enlarged liver. The masses have progressive enhancement on portal venous phase, and most of them have surrounding ground-glass opacity which is a typical finding for hemorrhage in angiosarcoma.