Treatment Outcomes for Primary Hepatic Angiosarcoma: National Cancer Database Analysis 2004–2014

SIMPLE SUMMARY: Primary hepatic angiosarcoma is a rare tumor of the liver. The prognosis and treatment of this rare tumor remains an enigma. Despite being the most common liver tumor of mesenchymal origin, the prognosis of patients diagnosed with primary hepatic angiosarcoma has never been compared with that of the most common liver tumor which is hepatocellular carcinoma. In this manuscript, we have analyzed all the recorded cases in the National Cancer Center Database and determine the best approach to treat this rare tumor. We have also conducted a brief review of the literature to guide the reader toward the finer nuances of managing patients diagnosed with primary hepatic angiosarcoma, especially in the context of a liver transplant. ABSTRACT: Background: To determine the risk of mortality and factors associated with survival amongst patients diagnosed with primary hepatic angiosarcoma (PHA). Methods: All patients diagnosed with hepatocellular carcinoma (HCC) or PHA from 2004 to 2014 were identified from the National Cancer Database (NCDB). Further analysis was performed within the cohort of patients with PHA to assess the impact of surgery, chemotherapy, radiation, and facility type on overall survival (OS). A multivariable analysis using the Cox proportional methods and a survival analysis using the Kaplan–Meier method were used. Results: A total of 117,633 patients with HCC were identified, out of whom 346 patients had PHA. Patients with PHA had a mean age of 62.9 years (SD 13.7), the majority were men (64.7%), white (85.8%), and had a Charlson comorbidity index (CCI) of zero (66.2%). A third of the patients with PHA (35.7%) received chemotherapy, and 14.6% underwent a surgical resection. The median survival was 1.9 months (1.8–2.4 months) compared to patients with HCC (10.4 months, 10.2–10.5) (aHR-2.41, 95% CI: 2.10–2.77, p < 0.0001). Surgical resection was associated with a higher median survival (7.7 versus 1.8 months, aHR-0.23, 95% CI: 0.15–0.37, p < 0.0001). A receipt of chemotherapy was associated with a higher median survival than no chemotherapy (5.1 versus 1.2 months, aHR-0.44, 95% CI: 0.32–0.60, p < 0.0001), although the survival benefit did not persist long term. Conclusion: PHA is associated with poor outcomes. A surgical resection and chemotherapy are associated with improved survival outcomes; however, the long-term benefits of chemotherapy are limited.