Management of Primary Hepatic Angiosarcoma: A Comprehensive Review

Area of Science:

• Hepatobiliary Oncology
• Surgical Pathology
• Medical Oncology

Background:

• Primary hepatic angiosarcoma (PHA) is a rare but aggressive liver cancer, comprising 0.1-2% of primary liver malignancies.
• It has a poor prognosis with a median survival of 6-9 months due to late diagnosis and aggressive biology.
• Currently, no specific tumor markers or diagnostic imaging features exist for PHA.

Purpose of the Study:

• To provide a comprehensive overview of the current understanding and management strategies for primary hepatic angiosarcoma.
• To synthesize recent advancements in diagnosis, treatment, and molecular profiling of PHA.
• To highlight future directions for improving patient outcomes.

Main Methods:

• A comprehensive narrative review of studies on PHA epidemiology, pathology, molecular biology, imaging, and management.
• Searches were conducted in PubMed, Embase, Cochrane Library, and Scopus up to June 2026.
• Preference was given to systematic reviews, multicenter cohorts, and prospective trials.

Main Results:

• ERG is the most sensitive immunohistochemical marker for PHA.
• Surgical R0 resection offers the only potential cure, significantly improving survival (17.2 months vs. 3.7 months without surgery).
• For unresectable disease, cabozantinib plus nivolumab showed a 59% response rate, and gemcitabine demonstrated 38% response in second-line treatment. Recurrent genetic alterations (TP53, KDR, PIK3CA, PTPRB, PLCG1) suggest potential for molecular profiling.

Conclusions:

• Current PHA management relies on surgical resection when feasible, combination immunotherapy-tyrosine kinase inhibitor (ICI-TKI) therapy for unresectable cases, and molecular profiling.
• International collaboration, multi-institutional trials, and specialized multidisciplinary care are crucial for advancing PHA management.
• Liver transplantation is contraindicated for PHA.