Key result
Four patients with biopsy-proven primary hepatic angiosarcoma demonstrated distinct clinical presentations and courses, highlighting the variable manifestations and grave prognosis of this rare tumor.
Why the study?
Primary hepatic angiosarcoma is a rare malignancy with variable presentations, challenging diagnosis often resulting in late-stage detection, and a paucity of effective therapeutic approaches.
Case Report (n=4)
Primary hepatic angiosarcoma is a rare liver malignancy with variable presentations and a grave prognosis, for which surgical resection remains the mainstay of treatment.
Highlights diagnostic challenges in rare liver tumors; leaves open effective therapies for PHA.
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.
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Rujeerapaiboon et al. (2020) conducted a case report in Primary hepatic angiosarcoma (n=4). Four patients with biopsy-proven primary hepatic angiosarcoma demonstrated distinct clinical presentations and courses, highlighting the variable manifestations and grave prognosis of this rare tumor.
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