The Clinical Features of Hepatic Angiosarcoma: A Report of Four Cases and A Review of the English Literature

Locker, Gershon Y.; Doroshow, James H.; Zwelling, Leonard A.; Chabner, Bruce A.

doi:10.1097/00005792-197901000-00003

Cited by 229 publications

(241 citation statements)

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“…Other chemical carcinogens include arsenic, radium and anabolic steroids [85]. There are reports of angiosarcoma in association with foreign bodies, including accidentally retained surgical gauzes [86,87], vascular [88,89], and orthopaedic prostheses [90,91] and even in association with a gouty tophus [92].…”

Section: Aetiologymentioning

confidence: 99%

Vascular-targeted agents for the treatment of angiosarcoma

Young

Woll

Staton

et al. 2013

Cancer Chemother Pharmacol

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Section: Aetiologymentioning

confidence: 99%

Vascular-targeted agents for the treatment of angiosarcoma

Young

Woll

Staton

et al. 2013

Cancer Chemother Pharmacol

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“…1 Hepatic AS is a rare (2% of primary liver tumors), but aggressive cancer. 2,3 Previous studies established an association between hepatic AS and exposure to environmental toxins such as thorotrast, vinyl chloride, inorganic arsenic, and others. 2,4 Patient survival is very poor with a median life expectancy of 6 months; only 3% of affected patients live longer than 2 years.…”

mentioning

confidence: 99%

“…2,3 Previous studies established an association between hepatic AS and exposure to environmental toxins such as thorotrast, vinyl chloride, inorganic arsenic, and others. 2,4 Patient survival is very poor with a median life expectancy of 6 months; only 3% of affected patients live longer than 2 years. 2 Currently, the conventional therapy of localized AS is surgical excision with the goal of complete resection.…”

mentioning

confidence: 99%

“…2,4 Patient survival is very poor with a median life expectancy of 6 months; only 3% of affected patients live longer than 2 years. 2 Currently, the conventional therapy of localized AS is surgical excision with the goal of complete resection. [5][6][7] However, in the case of hepatic AS, this therapeutical option is applicable only in few cases, and liver transplantation is contraindicated.…”

mentioning

confidence: 99%

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Generation of a murine hepatic angiosarcoma cell line and reproducible mouse tumor model

Rothweiler¹,

Dill²,

Terracciano

et al. 2015

Laboratory Investigation

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Hepatic angiosarcoma (AS) is a rare and highly aggressive tumor of endothelial origin with dismal prognosis. Studies of the molecular biology of AS and treatment options are limited as animal models are rare. We have previously shown that inducible knockout of Notch1 in mice leads to spontaneous formation of hepatic AS. The aims of this study were to: (1) establish and characterize a cell line derived from this murine AS, (2) identify molecular pathways involved in the pathogenesis and potential therapeutic targets, and (3) generate a tumor transplantation model. AS cells retained specific endothelial properties such as tube formation activity, as well as expression of CD31 and Von Willebrand factor. However, electron microscopy analysis revealed signs of dedifferentiation with loss of fenestrae and loss of contact inhibition. Microarray and pathway analysis showed substantial changes in gene expression and revealed activation of the Myc pathway. Exposing the AS cells to sorafenib reduced migration, filopodia dynamics, and cell proliferation but did not induce apoptosis. In addition, sorafenib suppressed ERK phosphorylation and expression of cyclin D2. Injection of AS cells into NOD/SCID mice resulted in formation of undifferentiated tumors, confirming the tumorigenic potential of these cells. In summary, we established and characterized a murine model of spontaneous AS formation and hepatic AS cell lines as a useful in vitro tool. Our data demonstrate antitumor activity of sorafenib in AS cells with potent inhibition of migration, filopodia formation, and cell proliferation, supporting further evaluation of sorafenib as a novel treatment strategy. In addition, AS cell transplantation provides a subcutaneous tumor model useful for in vivo preclinical drug testing.

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“…2 It is a highly vascular tumor classically associated with polyvinylchloride exposure. 3 Orthotopic liver transplantation has been abandoned as treatment for hepatic angiosarcoma because the recurrence rate is 64%, and patients have a very low survival rate. 4 We describe a 70-year-old woman who underwent orthotopic liver transplantation for decompensated cirrhosis in whom an incidental hepatic angiosarcoma was diagnosed in the explant.…”

mentioning

confidence: 99%