Liver angiosarcoma

Other names| Hepatic angiosarcoma, angiosarcoma of the liver
Specimen of a surgically removed and dissected liver angiosarcoma with visible necrosis and bleeding
Specialty| Oncology
Symptoms| Asymptomatic, abdominal pain, weight loss, anorexia, fatigue
Complications| Disseminated intravascular coagulation, tumor rupture
Usual onset| Age 60-70
Causes| Unknown, exposure to arsenic, thorotrast, vinyl chloride
Diagnostic method| Biopsy, imaging
Differential diagnosis| Hemangioma, hepatocellular carcinoma[1]
Treatment| Surgery, chemotherapy
Prognosis| Poor, most die within 6 months[2][3]
Frequency| 200 new diagnoses per year worldwide

Liver angiosarcoma also known as angiosarcoma of the liver or hepatic angiosarcoma is a rare and rapidly fatal cancer arising from endothelial that line the blood vessels of the liver. It is a type of angiosarcoma. Although very rare with around 200 cases diagnosed each year, it is still considered the third most common primary liver cancer, making up around 2% of all primary liver cancers. Liver angiosarcoma can be primary (referred to in literature as PHA or primary hepatic angiosarcoma), meaning it arose in the liver, or secondary, meaning the angiosarcoma arose elsewhere and metastasized to the liver.[2] This article covers PHA, however much is also applicable to secondary tumors.

## Contents

* 1 Signs and symptoms
* 1.1 Complications
* 2 Causes
* 3 Diagnosis
* 3.1 Differential diagnosis
* 4 Treatment
* 5 Prognosis
* 6 Epidemiology
* 7 References

## Signs and symptoms

Liver angiosarcoma usually presents with vague and non-specific symptoms such as abdominal pain, abdominal distension (which are the two most common symptoms, occurring in around 60% of individuals[3]), weight loss, fatigue or abdominal masses and liver disease like symptoms such as fever, malaise, anorexia and vomiting.[2]

Paradoxically, liver function is generally maintained until the final stages of the disease, further complicating diagnosis.[4]

### Complications

Spontaneous tumor rupture resulting in severe intra-abdominal bleeding and hemoperitoneum is a possibly fatal complication of liver angiosarcoma and is reported in 15–27% of patients. Thrombocytopenia, anemia and the vascular nature of the tumor may contribute to this. Tumor rupture generally carries a very poor prognosis even when bleeding is stopped by means of emergency transcathether arterial embolization (TAE), an analysis of four patients showed a median survival of 23 days following tumor rupture.[5][6][2][7] Due to the close and similarly vascular nature of the spleen, metastasis to it is common and therefore intra-abdominal bleeding may also be caused by splenic rupture.[4]

Liver angiosarcoma may also result in liver failure, a potentially fatal complication.[8]

Like many cancers, liver angiosarcoma can also cause disseminated intravascular coagulation (DIC).[2][9][10][11][12]

## Causes

Most liver angiosarcoma cases are of unknown etiology, making up around 75% of cases.[2]

Despite this, several things are associated with liver angiosarcoma. Exposure to vinyl chloride, arsenic, thorotrast, radium, phenylhydrazine and use of androgens is known to contribute to the pathogenesis of liver angiosarcoma.[2] Cyclophosphamide,[4] diethylstilbestrol and oral contraceptives use are also associated with liver angiosarcoma.[13][2]

In addition liver angiosarcoma is associated with hemochromatosis (iron overload)[14] and neurofibromatosis.[2][15]

A 2007 case report suggests a possible link between Schistosoma japonicum liver fibrosis and liver angiosarcoma.[4][16]

A study in Taiwan found no evidence to suggest that viral hepatitis a significant role in the pathogenesis of liver angiosarcoma.[2][17]

## Diagnosis

CD31 is considered the most reliable tumor marker for liver angiosarcoma.[2]

### Differential diagnosis

Differential diagnosis includes hemangioma,[1] Kaposi's sarcoma, metastatic angiosarcoma of non liver origin, secondary liver cancer from any origin and hepatocellular carcinoma.[2]

## Treatment

Complete surgical resection combined with adjuvant chemotherapy is considered to be the most effective treatment of liver angiosarcoma.[13]

Transcathether arterial embolization (TAE), blocking an artery with the help of a catheter to prevent further bleeding or limit blood supply to the tumor, resulting in suppressed growth, is the most effective treatment for spontaneous rupture of the tumor resulting in intra-abdominal bleeding. Transcatheter arterial chemoembolization (TACE), which is the same as TAE, but also involves the regional injection of chemotherapy drugs, has shown effectiveness at increasing survival, particularly in individuals with few dominant masses rather than several smaller.[2][4][5] TACE allows for simultaneously increasing regional (and therefore also tumor) exposure to chemotherapy while reducing systemic exposure, which both allows for an increased dose, as systemic exposure is generally the limiting factor, and also reduced side effects of chemotherapy. TACE and TAE are both generally performed on the hepatic artery.[18]

Liver angiosarcomas are generally reported to be radioresistant and therefore radiation therapy is not considered an effective treatment.[5][2][7]

Although previously considered a viable treatment option, liver transplantation is no longer considered for liver angiosarcoma, due to its high reoccurrence rate and poor post transplantation survival.[14] The European Liver Transplant Registry considers liver angiosarcoma an absolute contraindication to liver transplantation,[15] reporting that the median survival following liver transplantation is less than 7 months with no one surviving more than 23 months, showing very little difference from no treatment at all.[2][6]

## Prognosis

Due to the aggressive nature and high recurrence rate, the prognosis for liver angiosarcoma is generally very poor. Most patients die within six months and only 3% live more than two years.[2]

A recent case report suggest that the prognosis of liver angiosarcoma may be improving.[19]

## Epidemiology

Although liver angiosarcoma can affect anyone, most people affected are 60–70 years old. Males are affected more often than women at a ratio of 3-4:1,[14][20] although in children, girls are affected more commonly than boys.[2]

## References

1. ↑ 1.0 1.1 Yasir, Saba; Torbenson, Michael S. (May 2019). "Angiosarcoma of the Liver: Clinicopathologic Features and Morphologic Patterns". The American Journal of Surgical Pathology 43 (5): 581–590. doi:10.1097/PAS.0000000000001228. ISSN 1532-0979. PMID 30986799. https://pubmed.ncbi.nlm.nih.gov/30986799/. Retrieved 2021-08-11.
2. ↑ 2.00 2.01 2.02 2.03 2.04 2.05 2.06 2.07 2.08 2.09 2.10 2.11 2.12 2.13 2.14 2.15 2.16 Chaudhary, P.; Bhadana, U.; Singh, R. a. K.; Ahuja, A. (September 2015). "Primary hepatic angiosarcoma". European Journal of Surgical Oncology: The Journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology 41 (9): 1137–1143. doi:10.1016/j.ejso.2015.04.022. ISSN 1532-2157. PMID 26008857. https://pubmed.ncbi.nlm.nih.gov/26008857/. Retrieved 2021-08-11.
3. ↑ 3.0 3.1 Zeng, Dong; Cheng, Jianan; Gong, Zhihua; Chen, Jianghong; Long, Haixia; Zhu, Bo (2020-05-05). "A pooled analysis of primary hepatic angiosarcoma". Japanese Journal of Clinical Oncology 50 (5): 556–567. doi:10.1093/jjco/hyaa017. ISSN 1465-3621. PMID 32083280.
4. ↑ 4.0 4.1 4.2 4.3 4.4 Zheng, Ya-Wen; Zhang, Xin-Wei; Zhang, Jia-Li; Hui, Zhen-Zhen; Du, Wei-Jiao; Li, Run-Mei; Ren, Xiu-Bao (May 2014). "Primary hepatic angiosarcoma and potential treatment options". Journal of Gastroenterology and Hepatology 29 (5): 906–911. doi:10.1111/jgh.12506. ISSN 1440-1746. PMID 24372769.
5. ↑ 5.0 5.1 5.2 Zhu, Yi-Ping; Chen, Yan-Min; Matro, Erik; Chen, Ren-Biao; Jiang, Zhi-Nong; Mou, Yi-Ping; Hu, Hong-Jie; Huang, Chao-Jie et al. (2015-05-21). "Primary hepatic angiosarcoma: A report of two cases and literature review". World Journal of Gastroenterology 21 (19): 6088–6096. doi:10.3748/wjg.v21.i19.6088. ISSN 1007-9327. PMID 26019478.
6. ↑ 6.0 6.1 Schueler, Samuel A.; Hwang, Daniel G.; Twaddell, William; Raufman, Jean-Pierre; Urrunaga, Nathalie H. (February 2020). "Angiosarcoma: A rare cause of acute liver failure". Clinics and Research in Hepatology and Gastroenterology 44 (1): e14–e16. doi:10.1016/j.clinre.2019.06.014. ISSN 2210-741X. PMID 31296397. https://pubmed.ncbi.nlm.nih.gov/31296397/. Retrieved 2021-10-10.
7. ↑ 7.0 7.1 Chien, Chin-Ying; Hwang, Cheng-Cheng; Yeh, Chun-Nan; Chen, Huang-Yang; Wu, Jui-Teng; Cheung, Chan-Siu; Lin, Chih-Lang; Yen, Cho-Li et al. (2012-01-26). "Liver angiosarcoma, a rare liver malignancy, presented with intraabdominal bleeding due to rupture--a case report". World Journal of Surgical Oncology 10: 23. doi:10.1186/1477-7819-10-23. ISSN 1477-7819. PMID 22280556.
8. ↑ Abegunde, Ayokunle T.; Aisien, Efe; Mba, Benjamin; Chennuri, Rohini; Sekosan, Marin (2015). "Fulminant Hepatic Failure Secondary to Primary Hepatic Angiosarcoma". Case Reports in Gastrointestinal Medicine 2015: 869746. doi:10.1155/2015/869746. ISSN 2090-6528. PMID 25815217.
9. ↑ Wadhwa, Sanya; Kim, Tae Hun; Lin, Leah; Kanel, Gary; Saito, Takeshi (2017-04-07). "Hepatic angiosarcoma with clinical and histological features of Kasabach-Merritt syndrome". World Journal of Gastroenterology 23 (13): 2443–2447. doi:10.3748/wjg.v23.i13.2443. ISSN 2219-2840. PMID 28428724.
10. ↑ Fernando, Nadeeshani; Butcherine, Kylie; Harle, Robin; Ritchey, Donald (August 2019). "Mysterious abdominal pain and disseminated intravascular coagulation due to hepatic angiosarcoma". Internal Medicine Journal 49 (8): 1053–1054. doi:10.1111/imj.14382. ISSN 1445-5994. PMID 31387140. https://pubmed.ncbi.nlm.nih.gov/31387140/. Retrieved 2021-10-11.
11. ↑ Mazharuddin, Samir; Podduturi, Varsha; Guileyardo, Joseph M.; Cooper, Barry (January 2015). "Hepatic angiosarcoma associated with disseminated intravascular coagulation". Proceedings (Baylor University. Medical Center) 28 (1): 54–56. doi:10.1080/08998280.2015.11929186. ISSN 0899-8280. PMID 25552799.
12. ↑ Rosen, Emily A.; Vallurupalli, Mounica; Choy, Edwin; Lennerz, Jochen K.; Kuter, David J. (2018-11-21). "Management of disseminated intravascular coagulation in a patient with hepatic angiosarcoma". Medicine 97 (47): e13321. doi:10.1097/MD.0000000000013321. ISSN 0025-7974. PMID 30461646.
13. ↑ 13.0 13.1 Millan, Mauricio; Delgado, Alejandro; Caicedo, Luis A.; Arrunategui, Ana M.; Meneses, Carlos A.; Villegas, Jorge I.; Serrano, Oscar; Caicedo, Liliana et al. (2016-09-29). "Liver Angiosarcoma: Rare tumour associated with a poor prognosis, literature review and case report". International Journal of Surgery Case Reports 28: 165–168. doi:10.1016/j.ijscr.2016.09.044. ISSN 2210-2612. PMID 27718433.
14. ↑ 14.0 14.1 14.2 Maluf, D.; Cotterell, A.; Clark, B.; Stravitz, T.; Kauffman, H. M.; Fisher, R. A. (June 2005). "Hepatic angiosarcoma and liver transplantation: case report and literature review". Transplantation Proceedings 37 (5): 2195–2199. doi:10.1016/j.transproceed.2005.03.060. ISSN 0041-1345. PMID 15964377. https://pubmed.ncbi.nlm.nih.gov/15964377/. Retrieved 2021-10-10.
15. ↑ 15.0 15.1 Orlando, Giuseppe; Adam, Rene; Mirza, Darius; Soderdahl, Goran; Porte, Robert J.; Paul, Andreas; Burroughs, Andrew K.; Seiler, Christian A. et al. (2013-03-27). "Hepatic hemangiosarcoma: an absolute contraindication to liver transplantation--the European Liver Transplant Registry experience". Transplantation 95 (6): 872–877. doi:10.1097/TP.0b013e318281b902. ISSN 1534-6080. PMID 23354302.
16. ↑ Wang, Ze-Sheng; Wang, Wei-Xing; Xiong, Cheng-Long; Zhan, Na; Li, Hui (July 2007). "Spontaneous ruptured primary hepatic angiosarcoma coincident with Schistosoma Japonica liver fibrosis". Hepatology Research 37 (7): 572–576. doi:10.1111/j.1872-034X.2007.00067.x. ISSN 1386-6346. PMID 17540001. https://pubmed.ncbi.nlm.nih.gov/17540001/. Retrieved 2021-10-10.
17. ↑ Huang, Neng-Chyan; Wann, Shue-Ren; Chang, Hong-Tai; Lin, Shoa-Lin; Wang, Jyh-Seng; Guo, How-Ran (2011-12-26). "Arsenic, vinyl chloride, viral hepatitis, and hepatic angiosarcoma: a hospital-based study and review of literature in Taiwan". BMC Gastroenterology 11: 142. doi:10.1186/1471-230X-11-142. ISSN 1471-230X. PMID 22200164.
18. ↑ Guan, Yong-Song; He, Qing; Wang, Ming-Quan (2012-08-26). "Transcatheter Arterial Chemoembolization: History for More than 30 Years". ISRN Gastroenterology 2012: 480650. doi:10.5402/2012/480650. ISSN 2090-4398. PMID 22966466.
19. ↑ Huang, Neng-Chyan; Kuo, Yau-Chang; Chiang, Jui-Chin; Hung, Shih-Yuan; Wang, Huay-Min; Hung, Yao-Min; Chang, Yun-Te; Wann, Shue-Ren et al. (May 2015). "Hepatic angiosarcoma may have fair survival nowadays". Medicine 94 (19): e816. doi:10.1097/MD.0000000000000816. ISSN 1536-5964. PMID 25984668.
20. ↑ Averbukh, Leon D.; Mavilia, Marianna G.; Einstein, Michael M. (2018-09-11). "Hepatic Angiosarcoma: A Challenging Diagnosis". Cureus 10 (9): e3283. doi:10.7759/cureus.3283. ISSN 2168-8184. PMID 30443453.

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