A PowerPoint review focusing on hepatic angiosarcomas. Designed for surgical residents but likely useful for any medical profession, student or patient. Specifically, we discuss epidemiology, clinical presentation, pathology, etiology and treatment.

1. Hepatic Angiosarcoma
Dr. Joseph A. Di Como

2. Hepatic Angiosarcoma
● Rare malignancy, still third most common primary liver tumor. Previously
known as Kupffer cell sarcoma.
● May manifest as a single mass with satellite nodules or as a diffuse
infiltrative mass.
● Variable appearance on both CT and MRI reflecting pleomorphic histological
nature.
● Prognosis poor, survival uncommon beyond one year from diagnosis.

3. Epidemiology
● Account for .01-2% of primary liver tumors.
● Most commonly occurs in patients in 7th-8th decade of live with a male
predominance (M:F =4:1)

4. Clinical Presentation
● Patient typically have non-specific abdominal symptoms and may present
with an abdominal mass or hepatomegaly.
● Also can present with spontaneous rupture and hemoperitoneum.
● Many found incidentally.
● Thrombocytopenia and anemia common, may be related to consuption of
clotting factors.
● No good tumor markers

5. Pathology
● Arise from malignant spindle cells from endothelial origin.
● Metastasis is common at presentation, mostly affecting lung and spleen.

6. Etiology
● Most arise spontaneously, although hemochromatosis and
neurofibromatosis type 1 have been associated.
● Environmental exposure to Thorotrast, arsenic, radiation and vinyl chloride
has been implicated as risk factor.

7. Treatment
Initial treatment consists of performing a resection at the anatomic site of the
affected liver. However due to the infiltrative nature of the tumor and early
metastasis this is not always feasible.
Liver transplant has suggested as a better solution but….

8. Treatment - Liver Transplant
● In 1990 Starzl et al. published the first retrospective study involving liver
transplant and malignant tumors. Series involved 1469 patients who
received a liver transplant between 1980 and 1988, of these 115 were due to
a hepatic malignancy, of these 2 were angiosarcomas. 100% mortality in
first year.
● In 2006 another retrospective study of 19 patients with liver tumors that
underwent liver transplant (6 angiosarcomas, 13 metastatic sarcomas)
showed survival at 12 months was 20% and a 100% mortality within 15
months after liver transplant.

9. Treatment - Liver Transplant
● European Register of Liver Transplants published a retrospective study of
22 cases of liver angiosarcoma who received liver transplants between
1983 and 2004, survival at 12 months was 24% and a 100% mortality at 24
months.

10. Treatment - Chemotherapy
● Traditionally has a very poor response to chemotherapy.
● One study found two out of four patients with metastatic hepatic
angiosarcoma treated with chemotherapy died within three months after
diagnosis, other two survived for more than 6 months.

11. Treatment - Resection
● Less than 20% of patient are candidates for surgery at presentation.
● Surgery has proven to be beneficial and occasional long-term survivors after
resections have been reported.
● In 2015 retrospective series was performed in Taiwan consisting of 3503
patients with primary liver tumors. Of these 9 had hepatic angiosarcoma
and 3 (33.3%) survived for 2 years or more.

12. In conclusion...
Hepatic angiosarcomas may mimic benign vascular tumors.
On CT will often be multifocal with varied enhancement patterns on multiphasic
contrast-enhanced CT (likely related to pleomorphic tumor histology).
Early detection and resection are currently the only way to achieve a favorable
prognosis.

13. References
1. Chien CY, Hwang CC, Yeh CN et-al. Liver angiosarcoma, a rare liver malignancy, presented with intraabdominal bleeding due to
rupture-a case report. World J Surg Oncol. 2012;10 (1): 23. doi:10.1186/1477-7819-10-23 - Free text at pubmed - Pubmed citation
2. Koyama T, Fletcher JG, Johnson CD et-al. Primary hepatic angiosarcoma: findings at CT and MR imaging. Radiology. 2002;222
(3): 667-73. doi:10.1148/radiol.2223010877 - Pubmed citation
3. Kim HR, Rha SY, Cheon SH et-al. Clinical features and treatment outcomes of advanced stage primary hepatic angiosarcoma.
Ann. Oncol. 2009;20 (4): 780-7. doi:10.1093/annonc/mdn702 - Pubmed citation
4. Peterson MS, Baron RL, Rankin SC. Hepatic angiosarcoma: findings on multiphasic contrast-enhanced helical CT do not mimic
hepatic hemangioma. AJR Am J Roentgenol. 2000;175 (1): 165-70. doi:10.2214/ajr.175.1.1750165 - Pubmed citation
5. Thapar S, Rastogi A, Ahuja A et-al. Angiosarcoma of the liver: imaging of a rare salient entity. J Radiol Case Rep. 2014;8 (8): 24-32.
doi:10.3941/jrcr.v8i8.1693 - Free text at pubmed - Pubmed citation